Revised American thyroid association guidelines for the management of medullary thyroid carcinoma

Thyroid

Volumn 25, Issue 6, 2015, Pages 567-610

  Wells, Samuel A ^a   Asa, Sylvia L ^b   Dralle, Henning ^c   Elisei, Rossella ^d   Evans, Douglas B ^e   Gagel, Robert F ^f   Lee, Nancy ^g   MacHens, Andreas ^c   Moley, Jeffrey F ^h   Pacini, Furio ⁱ   Raue, Friedhelm ^j   Frank Raue, Karin ^j   Robinson, Bruce ^k   Rosenthal, M Sara ^l   Santoro, Massimo ^m   Schlumberger, Martin ⁿ   Shah, Manisha ^o   Waguespack, Steven G ^f  

^a NATIONAL CANCER INSTITUTE   (United States)

^b UNIVERSITY HEALTH NETWORK   (Canada)

^c MARTIN LUTHER UNIVERSITÄT HALLE WITTENBERG   (Germany)

^d UNIVERSITY OF PISA   (Italy)

^e MEDICAL COLLEGE OF WISCONSIN   (United States)

^f UNIVERSITY OF TEXAS MD ANDERSON CANCER CENTER   (United States)

^g MEMORIAL SLOAN KETTERING CANCER CENTER   (United States)

^h WASHINGTON UNIVERSITY SCHOOL OF MEDICINE   (United States)

ⁱ UNIVERSITY OF SIENA   (Italy)

^j UNIVERSITY OF HEIDELBERG   (Germany)

^k UNIVERSITY OF SYDNEY   (Australia)

^l UNIVERSITY OF KENTUCKY   (United States)

^m UNIVERSITY OF NAPLES FEDERICO II   (Italy)

ⁿ INSTITUT GUSTAVE ROUSSY   (France)

^o THE OHIO STATE UNIVERSITY WEXNER MEDICAL CENTER   (United States)

more..

Author keywords

[No Author keywords available]

Indexed keywords

CABOZANTINIB; CALCITONIN; CARCINOEMBRYONIC ANTIGEN; PROTEIN TYROSINE KINASE INHIBITOR; VANDETANIB; ANTINEOPLASTIC AGENT; PROTEIN RET; RET PROTEIN, HUMAN;

ARTICLE; BONE METASTASIS; CANCER DIAGNOSIS; CANCER PALLIATIVE THERAPY; CANCER PATIENT; CANCER THERAPY; DNA DETERMINATION; EVIDENCE BASED MEDICINE; FINE NEEDLE ASPIRATION BIOPSY; GENETIC SCREENING; GERMLINE MUTATION; HORMONE SUBSTITUTION; HUMAN; LIVER METASTASIS; LUNG METASTASIS; MEDIASTINUM METASTASIS; MEDICAL PRACTICE; MEDICAL SOCIETY; ONCOGENE RET; PATIENT CARE; PHASE 1 CLINICAL TRIAL (TOPIC); PHASE 2 CLINICAL TRIAL (TOPIC); PHASE 3 CLINICAL TRIAL (TOPIC); PRACTICE GUIDELINE; PRIORITY JOURNAL; RANDOMIZED CONTROLLED TRIAL (TOPIC); SKIN METASTASIS; SYSTEMIC THERAPY; THYROID MEDULLARY CARCINOMA; THYROIDECTOMY; UNITED STATES; CARCINOMA, MEDULLARY; CARCINOMA, NEUROENDOCRINE; DISEASE MANAGEMENT; GENETICS; MULTIPLE ENDOCRINE NEOPLASIA TYPE 2A; MULTIPLE ENDOCRINE NEOPLASIA TYPE 2B; PROCEDURES; RADIOTHERAPY; THYROID NEOPLASMS;

ANTINEOPLASTIC COMBINED CHEMOTHERAPY PROTOCOLS; BIOPSY, FINE-NEEDLE; CARCINOMA, MEDULLARY; CARCINOMA, NEUROENDOCRINE; DISEASE MANAGEMENT; GENETIC TESTING; HORMONE REPLACEMENT THERAPY; HUMANS; MULTIPLE ENDOCRINE NEOPLASIA TYPE 2A; MULTIPLE ENDOCRINE NEOPLASIA TYPE 2B; PROTO-ONCOGENE PROTEINS C-RET; RADIOTHERAPY; SOCIETIES, MEDICAL; THYROID NEOPLASMS; THYROIDECTOMY;

EID: 84930511149     PISSN: 10507256     EISSN: 15579077     Source Type: Journal    
DOI: 10.1089/thy.2014.0335     Document Type: Article

References (366)

1. Cancer of the Thyroid Invasive: Trends in SEER Incidence and U.S. Mortality Using the Joinpoint Regression Program, 1975-2011(SEER) Stat version 8.1.2 Rate Session. (Access the SEER 18 database at www.seer.cancer.gov).
2. Incidence-SEER 18 Regs Research Data + Hurricane Katrina Impacted Louisiana Cases, Nov 2012 Sub (2000-2010)-Linked To County Attributes-Total U.S., 1969-2011 Counties, National Cancer Institute, DCCPS, Surveillance Research Program, Surveillance Systems Branch, released April 2013, based on the November 2012 submission.
3. Brandi ML, Gagel RF, Angeli A, Bilezikian JP, Beck-Peccoz P, Bordi C, Conte-Devolx B, Falchetti A, Gheri RG, Libroia A, Lips CJ, Lombardi G, Mannelli M, Pacini F, Ponder BA, Raue F, Skogseid B, Tamburrano G, Thakker RV, Thompson NW, Tomassetti P, Tonelli F, Wells SA Jr, Marx SJ 2001 Guidelines for diagnosis and therapy of MEN type 1 and type 2. J Clin Endocrinol Metab 86:5658-5671.
4. Tuttle RM, Ball DW, Byrd D, Daniels GH, Dilawari RA, Doherty GM, Duh QY, Ehya H, Farrar WB, Haddad RI, Kandeel F, Kloos RT, Kopp P, Lamonica DM, Loree TR, Lydiatt WM, McCaffrey J, Olson JA Jr, Parks L, Ridge JA, Shah JP, Sherman SI, Sturgeon C, Waguespack SG, Wang TN, Wirth LJ 2010 Medullary carcinoma. J Natl Compr Canc Netw 8:512-530.
5. Chen H, Sippel RS, O'Dorisio MS, Vinik AI, Lloyd RV, Pacak K 2010 The North American Neuroendocrine Tumor Society consensus guideline for the diagnosis and management of neuroendocrine tumors: pheochromocytoma, paraganglioma, and medullary thyroid cancer. Pancreas 39:775-783.
6. Kloos RT, Eng C, Evans DB, Francis GL, Gagel RF, Gharib H, Moley JF, Pacini F, Ringel MD, Schlumberger M, Wells SA Jr 2009 Medullary thyroid cancer: management guidelines ofthe American Thyroid Association. Thyroid 19:565-612.
7. Jaquet J 1906 Ein Fall von metastasierenden Amyloidtumoren (Lymphosarkom). Virchows Arch Pathol Anat Physiol 185:251-268.
8. Hazard JB, Hawk WA, Crile G Jr 1959 Medullary (solid) carcinoma of the thyroid; a clinicopathologic entity. J Clin Endocrinol Metab 19:152-161.
9. Williams ED 1966 Histogenesis of medullary carcinoma of the thyroid. J Clin Pathol 19:114-118.
10. Tashjian AH Jr, Melvin EW 1968 Medullary carcinoma of the thyroid gland. Studies of thyrocalcitonin in plasma and tumor extracts. N Engl J Med 279:279-283.
11. Wells SA Jr, Baylin SB, Linehan WM, Farrell RE, Cox EB, Cooper CW 1978 Provocative agents and the diagnosis of medullary carcinoma of the thyroid gland. Ann Surg 188:139-141.
12. Pachnis V, Mankoo B, Costantini F 1993 Expression of the c-ret proto-oncogene during mouse embryogenesis. Development 119:1005-1017.
13. Zordan P, Tavella S, Brizzolara A, Biticchi R, Ceccherini I, Garofalo S, Ravazzolo R, Bocciardi R 2006 The immediate upstream sequence of the mouse Ret gene controls tissue-specific expression in transgenic mice. Int J Mol Med 18:601-608.
14. Takahashi M, Ritz J, Cooper GM 1985 Activation of a novel human transforming gene, ret, by DNA rearrangement. Cell 42:581-588.
15. Donis-Keller H, Dou S, Chi D, Carlson KM, Toshima K, Lairmore TC, Howe JR, Moley JF, Goodfellow P, Wells SA Jr 1993 Mutations in the RET proto-oncogene are associated with MEN 2A and FMTC. Hum Mol Genet 2:851-856.
16. Mulligan LM, Kwok JB, Healey CS, Elsdon MJ, Eng C, Gardner E, Love DR, Mole SE, Moore JK, Papi L, et al. 1993 Germ-line mutations of the RET proto-oncogene in multiple endocrine neoplasia type 2A. Nature 363:458-460.
17. Carlson KM, Dou S, Chi D, Scavarda N, Toshima K, Jackson CE, Wells SA Jr, Goodfellow PJ, Donis-Keller H 1994 Single missense mutation in the tyrosine kinase catalytic domain of the RET protooncogene is associated with multiple endocrine neoplasia type 2B. Proc Natl Acad Sci USA 91:1579-1583.
18. Hofstra RM, Landsvater RM, Ceccherini I, Stulp RP, Stelwagen T, Luo Y, Pasini B, Hoppener JW, van Amstel HK, Romeo G, et al. 1994 A mutation in the RET protooncogene associated with multiple endocrine neoplasia type 2B and sporadic medullary thyroid carcinoma. Nature 367:375-376.
19. Eng C, Smith DP, Mulligan LM, Nagai MA, Healey CS, Ponder MA, Gardner E, Scheumann GF, Jackson CE, Tunnacliffe A, et al. 1994 Point mutation within the tyrosine kinase domain of the RET proto-oncogene in multiple endocrine neoplasia type 2B and related sporadic tumours. Hum Mol Genet 3:237-241.
20. Marsh DJ, Learoyd DL, Andrew SD, Krishnan L, Pojer R, Richardson AL, Delbridge L, Eng C, Robinson BG 1996 Somatic mutations in the RET proto-oncogene in sporadic medullary thyroid carcinoma. Clin Endocrinol (Oxf) 44:249-257.
21. Moura MM, Cavaco BM, Pinto AE, Leite V 2011 High prevalence of RAS mutations in RET-negative sporadic medullary thyroid carcinomas. J Clin Endocrinol Metab 96:E863-E868.
22. Boichard A, Croux L, Al Ghuzlan A, Broutin S, Dupuy C, Leboulleux S, Schlumberger M, Bidart JM, Lacroix L 2012 Somatic RAS mutations occur in a large proportion of sporadic RET-negative medullary thyroid carcinomas and extend to a previously unidentified exon. J Clin Endocrinol Metab 97:E2031-E2035.
23. Ciampi R, Mian C, Fugazzola L, Cosci B, Romei C, Barollo S, Cirello V, Bottici V, Marconcini G, Rosa PM, Borrello MG, Basolo F, Ugolini C, Materazzi G, Pinchera A, Elisei R 2013 Evidence of a low prevalence of RAS mutations in a large medullary thyroid cancer series. Thyroid 23:50-57.
24. Agrawal N, Jiao Y, Sausen M, Leary R, Bettegowda C, Roberts NJ, Bhan S, Ho AS, Khan Z, Bishop J, Westra WH, Wood LD, Hruban RH, Tufano RP, Robinson B, Dralle H, Toledo SP, Toledo RA, Morris LG, Ghossein RA, Fagin JA, Chan TA, Velculescu VE, Vogelstein B, Kinzler KW, Papadopoulos N, Nelkin BD, Ball DW 2013 Exomic sequencing of medullary thyroid cancer reveals dominant and mutually exclusive oncogenic mutations in RET and RAS. J Clin Endocrinol Metab 98:E364-E369.
25. Schilling T, Burck J, Sinn HP, Clemens A, Otto HF, Hoppner W, Herfarth C, Ziegler R, Schwab M, Raue F 2001 Prognostic value of codon 918 (ATG/ACG) RET proto-oncogene mutations in sporadic medullary thyroid carcinoma. Int J Cancer 95:62-66.
26. Elisei R, Cosci B, Romei C, Bottici V, Renzini G, Molinaro E, Agate L, Vivaldi A, Faviana P, Basolo F, Miccoli P, Berti P, Pacini F, Pinchera A 2008 Prognostic significance of somatic RET oncogene mutations in sporadic medullary thyroid cancer: a 10-year follow-up study. J Clin Endocrinol Metab 93:682-687.
27. Romei C, Ugolini C, Cosci B, Torregrossa L, Vivaldi A, Ciampi R, Tacito A, Basolo F, Materazzi G, Miccoli P, Vitti P, Pinchera A, Elisei R 2012 Low prevalence of the somatic M918T RET mutation in micro-medullary thyroid cancer. Thyroid 22:476-481.
28. Santoro M, Melillo RM, Fusco A 2006 RET/PTC activation in papillary thyroid carcinoma: European Journal of Endocrinology Prize Lecture. Eur J Endocrinol 155: 645-653.
29. Kohno T, Ichikawa H, Totoki Y, Yasuda K, Hiramoto M, Nammo T, Sakamoto H, Tsuta K, Furuta K, Shimada Y, Iwakawa R, Ogiwara H, Oike T, Enari M, Schetter AJ, Okayama H, Haugen A, Skaug V, Chiku S, Yamanaka I, Arai Y, Watanabe S, Sekine I, Ogawa S, Harris CC, Tsuda H, Yoshida T, Yokota J, Shibata T 2012 KIF5B-RET fusions in lung adenocarcinoma. Nat Med 18:375-377.
30. Ballerini P, Struski S, Cresson C, Prade N, Toujani S, Deswarte C, Dobbelstein S, Petit A, Lapillonne H, Gautier EF, Demur C, Lippert E, Pages P, Mansat-De Mas V, Donadieu J, Huguet F, Dastugue N, Broccardo C, Perot C, Delabesse E 2012 RET fusion genes are associated with chronic myelomonocytic leukemia and enhance monocytic differentiation. Leukemia 26:2384-2389.
31. Attie T, Pelet A, Edery P, Eng C, Mulligan LM, Amiel J, Boutrand L, Beldjord C, Nihoul-Fekete C, Munnich A, et al. 1995 Diversity of RET proto-oncogene mutations in familial and sporadic Hirschsprung disease. Hum Mol Genet 4:1381-1386.
32. Amiel J, Sproat-Emison E, Garcia-Barcelo M, Lantieri F, Burzynski G, Borrego S, Pelet A, Arnold S, Miao X, Griseri P, Brooks AS, Antinolo G, de Pontual L, Clement-Ziza M, Munnich A, Kashuk C, West K, Wong KK, Lyonnet S, Chakravarti A, Tam PK, Ceccherini I, Hofstra RM, Fernandez R; Hirschsprung Disease Consortium 2008 Hirschsprung disease, associated syndromes and genetics: a review. J Med Genet 45:1-14.
33. Margraf RL, Crockett DK, Krautscheid PM, Seamons R, Calderon FR, Wittwer CT, Mao R 2009 Multiple endocrine neoplasia type 2 RET protooncogene database: repository of MEN2-associated RET sequence variation and reference for genotype/phenotype correlations. Hum Mutat 30:548-556.
34. Leboulleux S, Baudin E, Travagli JP, Schlumberger M 2004 Medullary thyroid carcinoma. Clin Endocrinol 61: 299-310.
35. Machens A, Hinze R, Thomusch O, Dralle H 2002 Pattern of nodal metastasis for primary and reoperative thyroid cancer. World J Surg 26:22-28.
36. Moley JF 2010 Medullary thyroid carcinoma: management of lymph node metastases. J Natl Compr Canc Netw 8:549-556.
37. Kebebew E, Ituarte PH, Siperstein AE, Duh QY, Clark OH 2000 Medullary thyroid carcinoma: clinical characteristics, treatment, prognostic factors, and a comparison of staging systems. Cancer 88:1139-1148.
38. Modigliani E, Cohen R, Campos JM, Conte-Devolx B, Maes B, Boneu A, Schlumberger M, Bigorgne JC, Dumontier P, Leclerc L, Corcuff B, Guilhem I 1998 Prognostic factors for survival and for biochemical cure in medullary thyroid carcinoma: results in 899 patients. The GETC Study Group. Groupe d'etude des tumeurs a calcitonine. Clin Endocrinol (Oxf) 48:265-273.
39. Kebebew E, Greenspan FS, Clark OH, Woeber KA, Grunwell J 2005 Extent of disease and practice patterns for medullary thyroid cancer. J Am Coll Surg 200:890-896.
40. Roman S, Lin R, Sosa JA 2006 Prognosis of medullary thyroid carcinoma: demographic, clinical, and pathologic predictors of survival in 1252 cases. Cancer 107:2134-2142.
41. Steiner AL, Goodman AD, Powers SR 1968 Study of a kindred with pheochromocytoma, medullary thyroid carcinoma, hyperparathyroidism and Cushing's disease: multiple endocrine neoplasia, type 2. Medicine (Baltimore) 47:371-409.
42. Wermer P 1954 Genetic aspects of adenomatosis of endocrine glands. Am J Med 16:363-371.
43. Telenius-Berg M, Berg B, Hamberger B, Tibblin S, Tisell LE, Ysander L, Welander G 1984 Impact of screening on prognosis in the multiple endocrine neoplasia type 2 syndromes: natural history and treatment results in 105 patients. Henry Ford Hosp Med J 32:225-231.
44. Gagel RF, Levy ML, Donovan DT, Alford BR, Wheeler T, Tschen JA 1989 Multiple endocrine neoplasia type 2a associated with cutaneous lichen amyloidosis. Ann Intern Med 111:802-806.
45. Borst MJ, VanCamp JM, Peacock ML, Decker RA 1995 Mutational analysis of multiple endocrine neoplasia type 2A associated with Hirschsprung's disease. Surgery 117: 386-391.
46. Williams ED, Pollock DJ 1966 Multiple mucosal neuromata with endocrine tumours: a syndrome allied to von Recklinghausen's disease. J Pathol Bacteriol 91:71-80.
47. Schimke RN, Hartmann WH, Prout TE, Rimoin DL 1968 Syndrome of bilateral pheochromocytoma, medullary thyroid carcinoma and multiple neuromas. A possible regulatory defect in the differentiation of chromaffin tissue. N Engl J Med 279:1-7.
48. Gorlin RJ, Sedano HO, Vickers RA, Cervenka J 1968 Multiple mucosal neuromas, pheochromocytoma and medullary carcinoma of the thyroid-a syndrome. Cancer 22:293-299.
49. Farndon JR, Leight GS, Dilley WG, Baylin SB, Smallridge RC, Harrison TS, Wells SA Jr 1986 Familial medullary thyroid carcinoma without associated endocrinopathies: a distinct clinical entity. Br J Surg 73: 278-281.
50. Eng C, Clayton D, Schuffenecker I, Lenoir G, Cote G, Gagel RF, van Amstel HK, Lips CJ, Nishisho I, Takai SI, Marsh DJ, Robinson BG, Frank-Raue K, Raue F, Xue F, Noll WW, Romei C, Pacini F, Fink M, Niederle B, Zedenius J, Nordenskjold M, Komminoth P, Hendy GN, Mulligan LM, et al. 1996 The relationship between specific RET proto-oncogene mutations and disease phenotype in multiple endocrine neoplasia type 2. International RET mutation consortium analysis. JAMA 276:1575-1579.
51. Moers AM, Landsvater RM, Schaap C, Jansen-Schillhorn van Veen JM, de Valk IA, Blijham GH, Hoppener JW, Vroom TM, van Amstel HK, Lips CJ 1996 Familial medullary thyroid carcinoma: not a distinct entity? Genotype-phenotype correlation in a large family. Am J Med 101:635-641.
52. Da Silva AM, Maciel RM, Da Silva MR, Toledo SR, De Carvalho MB, Cerutti JM 2003 A novel germ-line point mutation in RET exon 8 (Gly(533)Cys) in a large kindred with familial medullary thyroid carcinoma. J Clin Endocrinol Metab 88:5438-5443.
53. Kaldrymides P, Mytakidis N, Anagnostopoulos T, Vassiliou M, Tertipi A, Zahariou M, Rampias T, Koutsodontis G, Konstantopoulou I, Ladopoulou A, Bei T, Yannoukakos D 2006 A rare RET gene exon 8 mutation is found in two Greek kindreds with familial medullary thyroid carcinoma: implications for screening. Clin Endocrinol (Oxf) 64:561-566.
54. Oliveira MN, Hemerly JP, Bastos AU, Tamanaha R, Latini FR, Camacho CP, Impellizzeri A, Maciel RM, Cerutti JM 2011 The RET p.G533C mutation confers predisposition to multiple endocrine neoplasia type 2A in a Brazilian kindred and is able to induce a malignant phenotype in vitro and in vivo. Thyroid 21:975-985.
55. Peppa M, Boutati E, Kamakari S, Pikounis V, Peros G, Panayiotides IG, Economopoulos T, Raptis SA, Hadjidakis D 2008 Multiple endocrine neoplasia type 2A in two families with the familial medullary thyroid carcinoma associated G533C mutation of the RET proto-oncogene. Eur J Endocrinol 159:767-771.
56. Bethanis S, Koutsodontis G, Palouka T, Avgoustis C, Yannoukakos D, Bei T, Papadopoulos S, Linos D, Tsagarakis S 2007 A newly detected mutation of the RET protooncogene in exon 8 as a cause of multiple endocrine neoplasia type 2A. Hormones (Athens) 6:152-156.
57. Castro MR, Thomas BC, Richards ML, Zhang J, Morris JC 2013 Multiple endocrine neoplasia type 2A due to an exon 8 (G533C) mutation in a large North American kindred. Thyroid 23:1547-1552.
58. Siggelkow H, Melzer A, Nolte W, Karsten K, Hoppner W, Hufner M 2001 Presentation of a kindred with familial medullary thyroid carcinoma and Cys611Phe mutation of the RET proto-oncogene demonstrating low grade malignancy. Eur J Endocrinol 144:467-473.
59. Boccia LM, Green JS, Joyce C, Eng C, Taylor SA, Mulligan LM 1997 Mutation of RET codon 768 is associated with the FMTC phenotype. Clin Genet 51:81-85.
60. Jimenez C, Dang GT, Schultz PN, El-Naggar A, Shapiro S, Barnes EA, Evans DB, Vassilopoulou-Sellin R, Gagel RF, Cote GJ, Hoff AO 2004 A novel point mutation of the RET protooncogene involving the second intracellular tyrosine kinase domain in a family with medullary thyroid carcinoma. J Clin Endocrinol Metab 89:3521-3526.
61. Raue F, Frank-Raue K 2012 Genotype-phenotype correlation in multiple endocrine neoplasia type 2. Clinics (Sao Paulo) 67(Suppl 1):69-75.
62. Imai T, Uchino S, Okamoto T, Suzuki S, Kosugi S, Kikumori T, Sakurai A; MEN Consortium of Japan 2013 High penetrance of pheochromocytoma in multiple endocrine neoplasia 2 caused by germ line RET codon 634 mutation in Japanese patients. Eur J Endocrinol 168:683-687.
63. Frank-Raue K, Rybicki LA, Erlic Z, Schweizer H, Winter A, Milos I, Toledo SP, Toledo RA, Tavares MR, Alevizaki M, Mian C, Siggelkow H, Hufner M, Wohllk N, Opocher G, Dvorakova S, Bendlova B, Czetwertynska M, Skasko E, Barontini M, Sanso G, Vorlander C, Maia AL, Patocs A, Links TP, de Groot JW, Kerstens MN, Valk GD, Miehle K, Musholt TJ, Biarnes J, Damjanovic S, Muresan M, Wuster C, Fassnacht M, Peczkowska M, Fauth C, Golcher H, Walter MA, Pichl J, Raue F, Eng C, Neumann HP; International RET Exon 10 Consortium 2011 Risk profiles and penetrance estimations in multiple endocrine neoplasia type 2A caused by germline RET mutations located in exon 10. Hum Mutat 32:51-58.
64. Lairmore TC, Ball DW, Baylin SB, Wells SA Jr 1993 Management of pheochromocytomas in patients with multiple endocrine neoplasia type 2 syndromes. Ann Surg 217:595-601; discussion 601-603.
65. Lips CJ, Landsvater RM, Hoppener JW, Geerdink RA, Blijham G, van Veen JM, van Gils AP, de Wit MJ, Zewald RA, Berends MJ, et al. 1994 Clinical screening as compared with DNA analysis in families with multiple endocrine neoplasia type 2A. N Engl J Med 331:828-835.
66. Herfarth KK, Bartsch D, Doherty GM, Wells SA Jr, Lairmore TC 1996 Surgical management of hyperparathyroidism in patients with multiple endocrine neoplasia type 2A. Surgery 120:966-973; discussion 973-974.
67. Tanaka A, Arita K, Lai-Cheong JE, Palisson F, Hide M, McGrath JA 2009 New insight into mechanisms of pruritus from molecular studies on familial primary localized cutaneous amyloidosis. Brit J Derm 161:1217-1224.
68. Verga U, Fugazzola L, Cambiaghi S, Pritelli C, Alessi E, Cortelazzi D, Gangi E, Beck-Peccoz P 2003 Frequent association between MEN 2A and CLA. Clin Endocrinol (Oxf) 59:156-161.
69. Ceccherini I, Romei C, Barone V, Pacini F, Martino E, Loviselli A, Pinchera A, Romeo G 1994 Identification of the Cys634/Tyr mutation of the RET proto-oncogene in a pedigree with multiple endocrine neoplasia type 2A and localized CLA. J Endocrinol Invest 17:201-204.
70. Rothberg AE, Raymond VM, Gruber SB, Sisson J 2009 Familial medullary thyroid carcinoma associated with cutaneous lichen amyloidosis. Thyroid 19:651-655.
71. Mulligan LM, Eng C, Attie T, Lyonnet S, Marsh DJ, Hyland VJ, Robinson BG, Frilling A, Verellen-Dumoulin C, Safar A, et al. 1994 Diverse phenotypes associated with exon 10 mutations of the RET proto-oncogene. Hum Mol Genet 3:2163-2167.
72. Mulligan LM, Ponder BA 1995 Genetic basis of endocrine disease: multiple endocrine neoplasia type 2. J Clin Endocrinol Metab 80:1989-1995.
73. Verdy M, Weber AM, Roy CC, Morin CL, Cadotte M, Brochu P 1982 Hirschsprung's disease in a family with multiple endocrine neoplasia type 2. J Pediatr Gastroenterol Nutr 1:603-607.
74. Decker RA, Peacock ML 1998 Occurrence of MEN 2a in familial Hirschsprung's disease: a new indication for genetic testing of the RET proto-oncogene. J Pediatr Surg 33:207-214.
75. Sijmons RH, Hofstra RM, Wijburg FA, Links TP, Zwierstra RP, Vermey A, Aronson DC, Tan-Sindhunata G, Brouwers-Smalbraak GJ, Maas SM, Buys CH 1998 Oncological implications of RET gene mutations in Hirschsprung's disease. Gut 43:542-547.
76. Asai N, Jijiwa M, Enomoto A, Kawai K, Maeda K, Ichiahara M, Murakumo Y, Takahashi M 2006 RET receptor signaling: dysfunction in thyroid cancer and Hirschsprung's disease. Pathol Int 56:164-172.
77. Smith DP, Houghton C, Ponder BA 1997 Germline mutation of RET codon 883 in two cases of de novo MEN 2B. Oncogene 15:1213-1217.
78. Gimm O, Marsh DJ, Andrew SD, Frilling A, Dahia PL, Mulligan LM, Zajac JD, Robinson BG, Eng C 1997 Germline dinucleotide mutation in codon 883 of the RET proto-oncogene in multiple endocrine neoplasia type 2B without codon 918 mutation. J Clin Endocrinol Metab 82:3902-3904.
79. Jasim S, Ying AK, Waguespack SG, Rich TA, Grubbs EG, Jimenez C, Hu MI, Cote G, Habra MA 2011 Multiple endocrine neoplasia type 2B with a RET proto-oncogene A883F mutation displays a more indolent form of medullary thyroid carcinoma compared with a RET M918T mutation. Thyroid 21:189-192.
80. Smith VV, Eng C, Milla PJ 1999 Intestinal ganglioneuromatosis and multiple endocrine neoplasia type 2B: implications for treatment. Gut 45:143-146.
81. Cohen MS, Phay JE, Albinson C, DeBenedetti MK, Skinner MA, Lairmore TC, Doherty GM, Balfe DM, Wells SA Jr, Moley JF 2002 Gastrointestinal manifestations of multiple endocrine neoplasia type 2. Ann Surg 235:648-654; discussion 654-655.
82. Waguespack SG, Rich TA, Perrier ND, Jimenez C, Cote GJ 2011 Management of medullary thyroid carcinoma and MEN2 syndromes in childhood. Nat Rev Endocrinol 7: 596-607.
83. Miyauchi A, Futami H, Hai N, Yokozawa T, Kuma K, Aoki N, Kosugi S, Sugano K, Yamaguchi K 1999 Two germline missense mutations at codons 804 and 806 of the RET proto-oncogene in the same allele in a patient with multiple endocrine neoplasia type 2B without codon 918 mutation. Jpn J Cancer Res 90:1-5.
84. Iwashita T, Murakami H, Kurokawa K, Kawai K, Miyauchi A, Futami H, Qiao S, Ichihara M, Takahashi M 2000 A two-hit model for development of multiple endocrine neoplasia type 2B by RET mutations. Biochem Biophys Res Commun 268:804-808.
85. Kameyama K, Okinaga H, Takami H 2004 RET oncogene mutations in 75 cases of familial medullary thyroid carcinoma in Japan. Biomed Pharmacother 58:345-347.
86. Nakao KT, Usui T, Ikeda M, Mori Y, Yamamoto T, Kawashima ST, Nanba K, Yuno A, Tamanaha T, Tagami T, Naruse M, Asato R, Shimatsu A 2013 Novel tandem germline RET proto-oncogene mutations in a patient with multiple endocrine neoplasia type 2B: report of a case and a literature review of tandem RET mutations with in silico analysis. Head Neck 35:E363-E368.
87. Bartsch DK, Hasse C, Schug C, Barth P, Rothmund M, Hoppner W 2000 A RET double mutation in the germline of a kindred with FMTC. Exp Clin Endocrinol Diabetes 108:128-132.
88. Kasprzak L, Nolet S, Gaboury L, Pavia C, Villabona C, Rivera-Fillat F, Oriola J, Foulkes WD 2001 Familial medullary thyroid carcinoma and prominent corneal nerves associated with the germline V804M and V778I mutations on the same allele of RET. J Med Genet 38: 784-787.
89. 2012 Gene Tests: Medical Genetics Information Resource [database online]. www.genetest.org
90. Conzo G, Circelli L, Pasquali D, Sinisi A, Sabatino L, Accardo G, Renzullo A, Santini L, Salvatore F, Colantuoni V 2012 Lessons to be learned from the clinical management of a MEN 2A patient bearing a novel 634/640/700 mutation of the RET proto-oncogene. Clin Endocrinol (Oxf) 77:934-936.
91. Qi XP, Ma JM, Du ZF, Ying RB, Fei J, Jin HY, Han JS, Wang JQ, Chen XL, Chen CY, Liu WT, Lu JJ, Zhang JG, Zhang XN 2011 RET germline mutations identified by exome sequencing in a Chinese multiple endocrine neoplasia type 2A/familial medullary thyroid carcinoma family. PLoS One 6:e20353.
92. Dvorakova S, Vaclavikova E, Ryska A, Cap J, Vlcek P, Duskova J, Kodetova D, Holub V, Novak Z, Bendlova B 2006 Double germline mutations in the RET Protooncogene in MEN 2A and MEN 2B kindreds. Exp Clin Endocrinol Diabetes 114:192-196.
93. Poturnajova M, Altanerova V, Kostalova L, Breza J, Altaner C 2005 Novel germline mutation in the transmembrane region of RET gene close to Cys634Ser mutation associated with MEN 2A syndrome. J Mol Med (Berl) 83:287-295.
94. Nunes AB, Ezabella MC, Pereira AC, Krieger JE, Toledo SP 2002 A novel Val648Ile substitution in RET protooncogene observed in a Cys634Arg multiple endocrine neoplasia type 2A kindred presenting with an adrenocorticotropin-producing pheochromocytoma. J Clin Endocrinol Metab 87:5658-5661.
95. Cerutti JM, Maciel RM 2013 An unusual genotypephenotype correlation in MEN 2 patients: should screening for RET double germline mutations be performed to avoid misleading diagnosis and treatment? Clin Endocrinol (Oxf) 79:591-592.
96. Valente FO, Dias da Silva MR, Camacho CP, Kunii IS, Bastos AU, da Fonseca CC, Simiao HP, Tamanaha R, Maciel RM, Cerutti JM 2013 Comprehensive analysis of RET gene should be performed in patients with multiple endocrine neoplasia type 2 (MEN 2) syndrome and no apparent genotype-phenotype correlation: an appraisal of p.Y791F and p.C634Y RET mutations in five unrelated Brazilian families. J Endocrinol Invest 36:975-981.
97. Elisei R, Romei C, Cosci B, Agate L, Bottici V, Molinaro E, Sculli M, Miccoli P, Basolo F, Grasso L, Pacini F, Pinchera A 2007 RET genetic screening in patients with medullary thyroid cancer and their relatives: experience with 807 individuals at one center. J Clin Endocrinol Metab 92:4725-4729.
98. Eng C, Mulligan LM, Smith DP, Healey CS, Frilling A, Raue F, Neumann HP, Ponder MA, Ponder BA 1995 Low frequency of germline mutations in the RET protooncogene in patients with apparently sporadic medullary thyroid carcinoma. Clin Endocrinol (Oxf) 43:123-127.
99. Carlson KM, Bracamontes J, Jackson CE, Clark R, Lacroix A, Wells SA Jr, Goodfellow PJ 1994 Parent-oforigin effects in multiple endocrine neoplasia type 2B. Am J Hum Genet 55:1076-1082.
100. Schuffenecker I, Ginet N, Goldgar D, Eng C, Chambe B, Boneu A, Houdent C, Pallo D, Schlumberger M, Thivolet C, Lenoir GM 1997 Prevalence and parental origin of de novo RET mutations in multiple endocrine neoplasia type 2A and familial medullary thyroid carcinoma. Le Groupe d'Etude des Tumeurs a Calcitonine. Am J Hum Genet 60:233-237.
101. Tarasoff v. Regents of the University of California, 551 P.2d, 334 (CA 1976).
102. Pate v Threlkel, 661 So.2d 278 (Fla 1995).
103. Rosenthal MS, Pierce HH 2005 Inherited medullary thyroid cancer and the duty to warn: revisiting Pate v. Threlkel in light of HIPAA. Thyroid 15:140-145.
104. Laberge AM, Burke W 2009 Duty to warn at-risk family members of genetic disease. Virtual Mentor 11:656-660.
105. Sulmasy DP 2000 On warning families about genetic risk: the ghost of Tarasoff. Am J Med 109:738-739.
106. American Medical Association 2003 Opinion 2.131: Disclosure of familial risk in genetic testing. Code of medical ethics. Available at www.ama-assn.org/ama/pub/physicianresources/medical-ethics/code-medical-ethics/opinion2131.page?
107. Robson ME, Storm CD, Weitzel J, Wollins DS, Offit K 2010 American Society of Clinical Oncology policy statement update: genetic and genomic testing for cancer susceptibility. J Clin Oncol 28:893-901.
108. 1998 ASHG statement. Professional disclosure of familial genetic information. The American Society of Human Genetics Social Issues Subcommittee on Familial Disclosure. Am J Hum Genet 62:474-483.
109. Green RC, Berg JS, Grody WW, Kalia SS, Korf BR, Martin CL, McGuire AL, Nussbaum RL, O'Daniel JM, Ormond KE, Rehm HL, Watson MS, Williams MS, Biesecker LG; American College of Medical Genetics and Genomics 2013 ACMG recommendations for reporting of incidental findings in clinical exome and genome sequencing. Genet Med 15:565-574.
110. Gostin LO 2001 National health information privacy: regulations under the Health Insurance Portability and Accountability Act. JAMA 285:3015-3021.
111. Fleisher LD, Cole LJ 2001 Health Insurance Portability and Accountability Act is here: what price privacy? Genet Med 3:286-289.
112. Rosenthal MS, Diekema DS 2011 Pediatric ethics guidelines for hereditary medullary thyroid cancer. Int J Pediatr Endocrinol 2011:847603.
113. 1995 Informed consent, parental permission, and assent in pediatric practice. Committee on Bioethics, American Academy of Pediatrics. Pediatrics 95:314-317.
114. Huang SM, Tao BL, Tzeng CC, Liu HT, Wang WP 1997 Prenatal molecular diagnosis of RET proto-oncogene mutation in multiple endocrine neoplasia type 2A. J Formos Med Assoc 96:542-544.
115. Martinelli P, Maruotti GM, Pasquali D, Paladini D, Agangi A, Rippa E, Colantuoni V, Bellastella A, Sinisi AA 2004 Genetic prenatal RET testing and pregnancy management of multiple endocrine neoplasia Type II A (MEN2A): a case report. J Endocrinol Invest 27:357-360.
116. Offit K, Kohut K, Clagett B, Wadsworth EA, Lafaro KJ, Cummings S, White M, Sagi M, Bernstein D, Davis JG 2006 Cancer genetic testing and assisted reproduction. J Clin Oncol 24:4775-4782.
117. Offit K, Sagi M, Hurley K 2006 Preimplantation genetic diagnosis for cancer syndromes: a new challenge for preventive medicine. JAMA 296:2727-2730.
118. Fan HC, Gu W, Wang J, Blumenfeld YJ, El-Sayed YY, Quake SR 2012 Non-invasive prenatal measurement of the fetal genome. Nature 487:320-324.
119. Macher HC, Martinez-Broca MA, Rubio-Calvo A, Leon-Garcia C, Conde-Sanchez M, Costa A, Navarro E, Guerrero JM 2012 Non-invasive prenatal diagnosis of multiple endocrine neoplasia type 2A using COLD-PCR combined with HRM genotyping analysis from maternal serum. PLoS One 7:e51024.
120. Lietman SA 2011 Preimplantation genetic diagnosis for hereditary endocrine disease. Endocr Pract 17(Suppl 3): 28-32.
121. Martin J, Cervero A, Mir P, Martinez-Conejero JA, Pellicer A, Simón C 2013 The impact of next-generation sequencing technology on preimplantation genetic diagnosis and screening. Fertil Steril 99:1054-1061.e3.
122. Altarescu G, Barenholz O, Renbaum P, Beeri R, Levy-Lahad E, Margalioth EJ, Brooks B, Varshaver I, Eldar-Geva T 2011 Preimplantation genetic diagnosis (PGD)-prevention of the birth of children affected with endocrine diseases. J Pediatr Endocrinol Metab 24:543-548.
123. Chervenak FA, McCullough LB 1996 The fetus as a patient: an essential ethical concept for maternal-fetal medicine. J Matern Fetal Med 5:115-119.
124. Abe K, Adachi I, Miyakawa S, Tanaka M, Yamaguchi K, Tanaka N, Kameya T, Shimosato Y 1977 Production of calcitonin, adrenocorticotropic hormone, and betamelanocyte-stimulating hormone in tumors derived from amine precursor uptake and decarboxylation cells. Cancer Res 37:4190-4194.
125. Baylin SB, Beaven MA, Engelman K, Sjoerdsma A 1970 Elevated histaminase activity in medullary carcinoma of the thyroid gland. N Engl J Med 283:1239-1244.
126. Hirsch PF, Gauthier GF, Munson PL 1963 Thyroid hypocalcemic principle and recurrent laryngeal nerve injury as factors affecting the response to parathyroidectomy in rats. Endocrinology 73:244-252.
127. Ishikawa N, Hamada S 1976 Association of medullary carcinoma of the thyroid with carcinoembryonic antigen. Br J Cancer 34:111-115.
128. Zeytinoglu FN, Gagel RF, Tashjian AH Jr, Hammer RA, Leeman SE 1980 Characterization of neurotensin production by a line of rat medullary thyroid carcinoma cells. Proc Natl Acad Sci USA 77:3741-3745.
129. Mato E, Matias-Guiu X, Chico A, Webb SM, Cabezas R, Berna L, De Leiva A 1998 Somatostatin and somatostatin receptor subtype gene expression in medullary thyroid carcinoma. J Clin Endocrinol Metab 83:2417-2420.
130. Costante G, Durante C, Francis Z, Schlumberger M, Filetti S 2009 Determination of calcitonin levels in C-cell disease: clinical interest and potential pitfalls. Nat Clin Pract Endocrinol Metab 5:35-44.
131. Becker KL, Nylén ES, White JC, Mü ller B, Snider RH Jr 2004 Clinical review 167: Procalcitonin and the calcitonin gene family of peptides in inflammation, infection, and sepsis: a journey from calcitonin back to its precursors. J Clin Endocrinol Metab 89:1512-1525.
132. Whang KT, Steinwald PM, White JC, Nylen ES, Snider RH, Simon GL, Goldberg RL, Becker KL 1998 Serum calcitonin precursors in sepsis and systemic inflammation. J Clin Endocrinol Metab 83:3296-3301.
133. Borchhardt KA, Horl WH, Sunder-Plassmann G 2005 Reversibility of 'secondary hypercalcitoninemia' after kidney transplantation. Am J Transplant 5:1757-1763.
134. Bevilacqua M, Dominguez LJ, Righini V, Valdes V, Vago T, Leopaldi E, Baldi G, Barrella M, Barbagallo M 2006 Dissimilar PTH, gastrin, and calcitonin responses to oral calcium and peptones in hypocalciuric hypercalcemia, primary hyperparathyroidism, and normal subjects: a useful tool for differential diagnosis. J Bone Miner Res 21:406-412.
135. Schuetz M, Duan H, Wahl K, Pirich C, Antoni A, Kommata S, Kletter K, Dudczak R, Karanikas G, Willheim M 2006 T lymphocyte cytokine production patterns in Hashimoto patients with elevated calcitonin levels and their relationship to tumor initiation. Anticancer Res 26:4591-4596.
136. Pratz KW, Ma C, Aubry MC, Vrtiska TJ, Erlichman C 2005 Large cell carcinoma with calcitonin and vasoactive intestinal polypeptide-associated Verner-Morrison syndrome. Mayo Clin Proc 80:116-120.
137. Sim SJ, Glassman AB, Ro JY, Lee JJ, Logothetis CJ, Liu FJ 1996 Serum calcitonin in small cell carcinoma of the prostate. Ann Clin Lab Sci 26:487-495.
138. Machens A, Haedecke J, Holzhausen HJ, Thomusch O, Schneyer U, Dralle H 2000 Differential diagnosis of calcitonin-secreting neuroendocrine carcinoma of the foregut by pentagastrin stimulation. Langenbecks Arch Surg 385: 398-401.
139. Yocum MW, Butterfield JH, Gharib H 1994 Increased plasma calcitonin levels in systemic mast cell disease. Mayo Clin Proc 69:987-990.
140. Toledo SP, Lourenco DM Jr, Santos MA, Tavares MR, Toledo RA, Correia-Deur JE 2009 Hypercalcitoninemia is not pathognomonic of medullary thyroid carcinoma. Clinics (Sao Paulo) 64:699-706.
141. Preissner CM, Dodge LA, O'Kane DJ, Singh RJ, Grebe SK 2005 Prevalence of heterophilic antibody interference in eight automated tumor marker immunoassays. Clin Chem 51:208-210.
142. Leboeuf R, Langlois MF, Martin M, Ahnadi CE, Fink GD 2006 "Hook effect" in calcitonin immunoradiometric assay in patients with metastatic medullary thyroid carcinoma: case report and review of the literature. J Clin Endocrinol Metab 91:361-364.
143. d'Herbomez M, Caron P, Bauters C, Do Cao C, Schlienger JL, Sapin R, Baldet L, Carnaille B, Wemeau JL 2007 Reference range of serum calcitonin levels in humans: influence of calcitonin assays, sex, age, and cigarette smoking. Eur J Endocrinol 157:749-755.
144. Basuyau JP, Mallet E, Leroy M, Brunelle P 2004 Reference intervals for serum calcitonin in men, women, and children. Clin Chem 50:1828-1830.
145. Guyetant S, Rousselet MC, Durigon M, Chappard D, Franc B, Guerin O, Saint-Andre JP 1997 Sex-related Ccell hyperplasia in the normal human thyroid: a quantitative autopsy study. J Clin Endocrinol Metab 82:42-47.
146. Algeciras-Schimnich A, Preissner CM, Theobald JP, Finseth MS, Grebe SK 2009 Procalcitonin: a marker for the diagnosis and follow-up of patients with medullary thyroid carcinoma. J Clin Endocrinol Metab 94:861-868.
147. Walter MA, Meier C, Radimerski T, Iten F, Kranzlin M, Muller-Brand J, de Groot JW, Kema IP, Links TP, Muller B 2010 Procalcitonin levels predict clinical course and progression-free survival in patients with medullary thyroid cancer. Cancer 116:31-40.
148. Lorenz K, Elwerr M, Machens A, Abuazab M, Holzhausen HJ, Dralle H 2013 Hypercalcitoninemia in thyroid conditions other than medullary thyroid carcinoma: a comparative analysis of calcium and pentagastrin stimulation of serum calcitonin. Langenbecks Arch Surg 398: 403-409.
149. Trimboli P, Giovanella L, Crescenzi A, Romanelli F, Valabrega S, Spriano G, Cremonini N, Guglielmi R, Papini E 2014 Medullary thyroid cancer diagnosis: an appraisal. Head Neck 36:1216-1233.
150. Colombo C, Verga U, Mian C, Ferrero S, Perrino M, Vicentini L, Dazzi D, Opocher G, Pelizzo MR, Beck-Peccoz P, Fugazzola L 2012 Comparison of calcium and pentagastrin tests for the diagnosis and follow-up of medullary thyroid cancer. J Clin Endocrinol Metab 97:905-913.
151. Main C, Perrino MN, Colombo C, Cavedon E, Pennelli G, Ferrero S, De Leo S, Sarais C, Cacciatore C, Manfredi GI, Verga U, Iacobone M, De Pasquale L Pelizzo MR, Vicentini L, Persani L, Fugazzola L 2014 Refining calcium test for the diagnosis of medullary thyroid cancer: cutoffs, procedures, and safety. J Clin Endocrinol Metab 99:1656-1664.
152. Wells SA Jr, Haagensen DE Jr, Linehan WM, Farrell RE, Dilley WG 1978 The detection of elevated plasma levels of carcinoembryonic antigen in patients with suspected or established medullary thyroid carcinoma. Cancer 42: 1498-1503.
153. Mendelsohn G, Wells SA Jr, Baylin SB 1984 Relationship of tissue carcinoembryonic antigen and calcitonin to tumor virulence in medullary thyroid carcinoma. An immunohistochemical study in early, localized, and virulent disseminated stages of disease. Cancer 54:657-662.
154. Bockhorn M, Frilling A, Rewerk S, Liedke M, Dirsch O, Schmid KW, Broelsch CE 2004 Lack of elevated serum carcinoembryonic antigen and calcitonin in medullary thyroid carcinoma. Thyroid 14:468-470.
155. Dora JM, Canalli MH, Capp C, Punales MK, Vieira JG, Maia AL 2008 Normal perioperative serum calcitonin levels in patients with advanced medullary thyroid carcinoma: case report and review of the literature. Thyroid 18:895-899.
156. Frank-Raue K, Machens A, Leidig-Bruckner G, Rondot S, Haag C, Schulze E, Lorenz A, Kreissl MC, Dralle H, Raue F, Schmid KW 2013 Prevalence and clinical spectrum of nonsecretory medullary thyroid carcinoma in a series of 839 patients with sporadic medullary thyroid carcinoma. Thyroid 23:294-300.
157. Khurana R, Agarwal A, Bajpai VK, Verma N, Sharma AK, Gupta RP, Madhusudan K. P 2004 Unraveling the amyloid associated with human medullary thyroid carcinoma. Endocrinology 145:5465-5470.
158. Nangue C, Bron L, Portmann L, Volante M, Ris HB, Monnier P, Andrejevic-Blant S 2009 Mixed medullarypapillary carcinoma of the thyroid: report of a case and review of the literature. Head Neck 31:968-974.
159. Griffith C, Zhang S, Mukhopadhyay S 2010 Synchronous metastatic medullary and papillary thyroid carcinomas in a patient with germline RET mutation: case report, molecular analysis, and implications for pathogenesis. Endocr Pathol 21:115-119.
160. Machens A, Dralle H 2012 Simultaneous medullary and papillary thyroid cancer: a novel entity? Ann Surg Oncol 19:37-44.
161. Biscolla RP, Ugolini C, Sculli M, Bottici V, Castagna MG, Romei C, Cosci B, Molinaro E, Faviana P, Basolo F, Miccoli P, Pacini F, Pinchera A, Elisei R 2004 Medullary and papillary tumors are frequently associated in the same thyroid gland without evidence of reciprocal influence in their biologic behavior. Thyroid 14:946-952.
162. Kim WG, Gong G, Kim EY, Kim TY, Hong SJ, Kim WB, Shong YK 2010 Concurrent occurrence of medullary thyroid carcinoma and papillary thyroid carcinoma in the same thyroid should be considered as coincidental. Clin Endocrinol (Oxf) 72:256-263.
163. Pastolero GC, Coire CI, Asa SL 1996 Concurrent medullary and papillary carcinomas of thyroid with lymph node metastases. A collision phenomenon. Am J Surg Pathol 20:245-250.
164. Kostoglou-Athanassiou I, Athanassiou P, Vecchini G, Gogou L, Kaldrymides P 2004 Mixed medullary-follicular thyroid cancer: report of a case and review of the literature. Horm Res 61:300-304.
165. Papotti M, Komminoth P, Sobrinho-Simoes M, Bussolati G 2000 Thyroid carcinoma with mixed follicular and C-cell differentiation patterns. Semin Diagn Pathol 17: 109-119.
166. Wolfe HJ, Melvin KE, Cervi-Skinner SJ, Saadi AA, Juliar JF, Jackson CE, Tashjian AH Jr 1973 C-cell hyperplasia preceding medullary thyroid carcinoma. N Engl J Med 289:437-441.
167. Mete O, Asa SL 2013 Precursor lesions of endocrine system neoplasms. Pathology 45:316-330.
168. Biddinger PW, Brennan MF, Rosen PP 1991 Symptomatic C-cell hyperplasia associated with chronic lymphocytic thyroiditis. Am J Surg Pathol 15:599-604.
169. LiVolsi VA, Feind CR, LoGerfo P, Tashjian AH Jr 1973 Demonstration by immunoperoxidase staining of hyperplasia of parafollicular cells in the thyroid gland in hyperparathyroidism. J Clin Endocrinol Metab 37:550-559.
170. O'Toole K, Fenoglio-Preiser C, Pushparaj N 1985 Endocrine changes associated with the human aging process: III. Effect of age on the number of calcitonin immunoreactive cells in the thyroid gland. Hum Pathol 16:991-1000.
171. Tomita T, Millard DM 1992 C-cell hyperplasia in secondary hyperparathyroidism. Histopathology 21:469-474.
172. Machens A, Dralle H 2012 Biological relevance of medullary thyroid microcarcinoma. J Clin Endocrinol Metab 97:1547-1553.
173. Valenta LJ, Michel-Bechet M, Mattson JC, Singer FR 1977 Microfollicular thyroid carcinoma with amyloid rich stroma resembling the medullary carcinoma of the thyroid (MCT). Cancer 39:1573-1586.
174. Chang TC, Wu SL, Hsiao YL 2005 Medullary thyroid carcinoma: pitfalls in diagnosis by fine needle aspiration cytology and relationship of cytomorphology to RET proto-oncogene mutations. Acta Cytol 49:477-482.
175. Papaparaskeva K, Nagel H, Droese M 2000 Cytologic diagnosis of medullary carcinoma of the thyroid gland. Diagn Cytopathol 22:351-358.
176. Trimboli P, Treglia, G, Guidobaldi L, Romanelli F, Nigri G, Valabrega S, Sadeghi R, Crescenzi A, Faquin WC, Bongiovanni M, Giovanella L 2015 Detection rate of FNA cytology in medullary thyroid carcinoma: a meta-analysis. Clin Endocrinol (Oxf) 82:280-285.
177. Trimboli P, Cremonini N, Ceriani L, Saggiorato E, Guidobaldi L, Romanelli F, Ventura C, Laurenti O, Messuti I, Solaroli E, Madaio R, Bongiovanni M, Orlandi F, Crescenzi A, Valabrega S, Giovanella L 2014 Calcitonin measurement in aspiration needle washout fluids has higher sensitivity than cytology in detecting medullary thyroid cancer: a retrospective multicentre study. Clin Endocrinol 80:135-140.
178. Elisei R, Bottici V, Luchetti F, Di Coscio G, Romei C, Grasso L, Miccoli P, Iacconi P, Basolo F, Pinchera A, Pacini F 2004 Impact of routine measurement of serum calcitonin on the diagnosis and outcome of medullary thyroid cancer: experience in 10, 864 patients with nodular thyroid disorders. J Clin Endocrinol Metab 89:163-168.
179. Vierhapper H, Raber W, Bieglmayer C, Kaserer K, Weinhausl A, Niederle B 1997 Routine measurement of plasma calcitonin in nodular thyroid diseases. J Clin Endocrinol Metab 82:1589-1593.
180. Costante G, Meringolo D, Durante C, Bianchi D, Nocera M, Tumino S, Crocetti U, Attard M, Maranghi M, Torlontano M, Filetti S 2007 Predictive value of serum calcitonin levels for preoperative diagnosis of medullary thyroid carcinoma in a cohort of 5817 consecutive patients with thyroid nodules. J Clin Endocrinol Metab 92: 450-455.
181. Cheung K, Roman SA, Wang TS, Walker HD, Sosa JA 2008 Calcitonin measurement in the evaluation of thyroid nodules in the United States: a cost-effectiveness and decision analysis. J Clin Endocrinol Metab 93:2173-2180.
182. Daniels GH 2011 Screening for medullary thyroid carcinoma with serum calcitonin measurements in patients with thyroid nodules in the United States and Canada. Thyroid 21:1199-1207.
183. Boi F, Maurelli I, Pinna G, Atzeni F, Piga M, Lai ML, Mariotti S 2007 Calcitonin measurement in wash-out fluid from fine needle aspiration of neck masses in patients with primary and metastatic medullary thyroid carcinoma. J Clin Endocrinol Metab 92:2115-2118.
184. Kudo T, Miyauchi A, Ito Y, Takamura Y, Amino N, Hirokawa M 2007 Diagnosis of medullary thyroid carcinoma by calcitonin measurement in fine-needle aspiration biopsy specimens. Thyroid 17:635-638.
185. Miyauchi A, Matsuzuka F, Hirai K, Yokozawa T, Kobayashi K, Ito Y, Nakano K, Kuma K, Futami H, Yamaguchi K 2002 Prospective trial of unilateral surgery for nonhereditary medullary thyroid carcinoma in patients without germline RET mutations. World J Surg 26:1023-1028.
186. Machens A, Hauptmann S, Dralle H 2007 Increased risk of lymph node metastasis in multifocal hereditary and sporadic medullary thyroid cancer. World J Surg 31: 1960-1965.
187. Kaserer K, Scheuba C, Neuhold N, Weinhausel A, Haas OA, Vierhapper H, Niederle B 2001 Sporadic versus familial medullary thyroid microcarcinoma: a histopathologic study of 50 consecutive patients. Am J Surg Pathol 25:1245-1251.
188. Machens A, Dralle H 2010 Biomarker-based risk stratification for previously untreated medullary thyroid cancer. J Clin Endocrinol Metab 95:2655-2663.
189. Giraudet AL, Vanel D, Leboulleux S, Auperin A, Dromain C, Chami L, Ny Tovo N, Lumbroso J, Lassau N, Bonniaud G, Hartl D, Travagli JP, Baudin E, Schlumberger M 2007 Imaging medullary thyroid carcinoma with persistent elevated calcitonin levels. J Clin Endocrinol Metab 92:4185-4190.
190. Weber T, Schilling T, Frank-Raue K, Colombo-Benkmann M, Hinz U, Ziegler R, Klar E 2001 Impact of modified radical neck dissection on biochemical cure in medullary thyroid carcinomas. Surgery 130:1044-1049.
191. Moley JF, DeBenedetti MK 1999 Patterns of nodal metastases in palpable medullary thyroid carcinoma: recommendations for extent of node dissection. Ann Surg 229:880-887; discussion 887-888.
192. Zhang L, Wei WJ, Ji QH, Zhu YX, Wang ZY, Wang Y, Huang CP, Shen Q, Li DS, Wu Y 2012 Risk factors for neck nodal metastasis in papillary thyroid microcarcinoma: a study of 1066 patients. J Clin Endocrinol Metab 97:1250-1257.
193. Park JH, Lee YS, Kim BW, Chang HS, Park CS 2012 Skip lateral neck node metastases in papillary thyroid carcinoma. World J Surg 36:743-747.
194. Machens A, Hauptmann S, Dralle H 2008 Prediction of lateral lymph node metastases in medullary thyroid cancer. Br J Surg 95:586-591.
195. Machens A, Holzhausen HJ, Dralle H 2006 Contralateral cervical and mediastinal lymph node metastasis in medullary thyroid cancer: systemic disease? Surgery 139: 28-32.
196. Franc S, Niccoli-Sire P, Cohen R, Bardet S, Maes B, Murat A, Krivitzky A, Modigliani E; French Med Study Group 2001 Complete surgical lymph node resection does not prevent authentic recurrences of medullary thyroid carcinoma. Clin Endocrinol (Oxf) 55: 403-409.
197. Machens A, Ukkat J, Hauptmann S, Dralle H 2007 Abnormal carcinoembryonic antigen levels and medullary thyroid cancer progression: a multivariate analysis. Arch Surg 142:289-293; discussion 294.
198. Lorenz K, Abuazab M, Sekulla C, Nguyen-Thanh P, Brauckhoff M, Dralle H 2010 Management of lymph fistulas in thyroid surgery. Langenbecks Arch Surg 395: 911-917.
199. Roh JL, Kim DH, Park CI 2008 Prospective identification of chyle leakage in patients undergoing lateral neck dissection for metastatic thyroid cancer. Ann Surg Oncol 15: 424-429.
200. Sobol S, Jensen C, Sawyer W 2nd, Costiloe P, Thong N 1985 Objective comparison of physical dysfunction after neck dissection. Am J Surg 150:503-509.
201. Pelizzo MR, Boschin IM, Bernante P, Toniato A, Piotto A, Pagetta C, Nibale O, Rampin L, Muzzio PC, Rubello D 2007 Natural history, diagnosis, treatment and outcome of medullary thyroid cancer: 37 years experience on 157 patients. Eur J Surg Oncol 33:493-497.
202. Scollo C, Baudin E, Travagli JP, Caillou B, Bellon N, Leboulleux S, Schlumberger M 2003 Rationale for central and bilateral lymph node dissection in sporadic and hereditary medullary thyroid cancer. J Clin Endocrinol Metab 88:2070-2075.
203. Leggett MD, Chen SL, Schneider PD, Martinez SR 2008 Prognostic value of lymph node yield and metastatic lymph node ratio in medullary thyroid carcinoma. Ann Surg Oncol 15:2493-2499.
204. Machens A, Gimm O, Ukkat J, Hinze R, Schneyer U, Dralle H 2000 Improved prediction of calcitonin normalization in medullary thyroid carcinoma patients by quantitative lymph node analysis. Cancer 88:1909-1915.
205. Machens A, Dralle H 2013 Prognostic impact of N staging in 715 medullary thyroid cancer patients: proposal for a revised staging system. Ann Surg 257:323-329.
206. Brauckhoff M, Machens A, Thanh PN, Lorenz K, Schmeil A, Stratmann M, Sekulla C, Brauckhoff K, Dralle H 2010 Impact of extent of resection for thyroid cancer invading the aerodigestive tract on surgical morbidity, local recurrence, and cancer-specific survival. Surgery 148: 1257-1266.
207. Machens A, Schneyer U, Holzhausen HJ, Dralle H 2005 Prospects of remission in medullary thyroid carcinoma according to basal calcitonin level. J Clin Endocrinol Metab 90:2029-2034.
208. Dralle H, Damm I, Scheumann GF, Kotzerke J, Kupsch E, Geerlings H, Pichlmayr R 1994 Compartment-oriented microdissection of regional lymph nodes in medullary thyroid carcinoma. Surg Today 24:112-121.
209. Machens A, Dralle H 2013 Benefit-risk balance of reoperation for persistent medullary thyroid cancer. Ann Surg 257:751-757.
210. Schneider R, Randolph GW, Sekulla C, Phelan E, Thanh PN, Bucher M, Machens A, Dralle H, Lorenz K 2013 Continuous intraoperative vagus nerve stimulation for identification of imminent recurrent laryngeal nerve injury. Head Neck 35:1591-1598.
211. Olson JA Jr, DeBenedetti MK, Baumann DS, Wells SA Jr 1996 Parathyroid autotransplantation during thyroidectomy. Results of long-term follow-up. Ann Surg 223:472-478; discussion 478-480.
212. Machens A, Dralle H 2007 Genotype-phenotype based surgical concept of hereditary medullary thyroid carcinoma. World J Surg 31:957-968.
213. Marsh DJ, McDowall D, Hyland VJ, Andrew SD, Schnitzler M, Gaskin EL, Nevell DF, Diamond T, Delbridge L, Clifton-Bligh P, Robinson BG 1996 The identification of false positive responses to the pentagastrin stimulation test in RET mutation negative members of MEN 2A families. Clin Endocrinol (Oxf) 44:213-220.
214. Machens A, Ukkat J, Brauckhoff M, Gimm O, Dralle H 2005 Advances in the management of hereditary medullary thyroid cancer. J Intern Med 257:50-59.
215. Elisei R, Romei C, Renzini G, Bottici V, Cosci B, Molinaro E, Agate L, Cappagli V, Miccoli P, Berti P, Faviana P, Ugolini C, Basolo F, Vitti P, Pinchera A 2012 The timing of total thyroidectomy in RET gene mutation carriers could be personalized and safely planned on the basis of serum calcitonin: 18 years experience at one single center. J Clin Endocrinol Metab 97:426-435.
216. Sosa JA, Tuggle CT, Wang TS, Thomas DC, Boudourakis L, Rivkees S, Roman SA 2008 Clinical and economic outcomes of thyroid and parathyroid surgery in children. J Clin Endocrinol Metab 93:3058-3065.
217. Tuggle CT, Roman SA, Wang TS, Boudourakis L, Thomas DC, Udelsman R, Ann Sosa J 2008 Pediatric endocrine surgery: who is operating on our children? Surgery 144:869-877; discussion 877.
218. Frank-Raue K, Buhr H, Dralle H, Klar E, Senninger N, Weber T, Rondot S, Hoppner W, Raue F 2006 Long-term outcome in 46 gene carriers of hereditary medullary thyroid carcinoma after prophylactic thyroidectomy: impact of individual RET genotype. Eur J Endocrinol 155:229-236.
219. Bihan H, Baudin E, Meas T, Leboulleux S, Al Ghuzlan A, Hannoteaux V, Travagli JP, Valleur P, Guillausseau PJ, Cohen R; French Group of Endocrine Tumors 2012 Role of prophylactic thyroidectomy in RET 790 familial medullary thyroid carcinoma. Head Neck 34:493-498.
220. Machens A, Gimm O, Hinze R, Hoppner W, Boehm BO, Dralle H 2001 Genotype-phenotype correlations in hereditary medullary thyroid carcinoma: oncological features and biochemical properties. J Clin Endocrinol Metab 86:1104-1109.
221. Frohnauer MK, Decker RA 2000 Update on the MEN 2A c804 RET mutation: is prophylactic thyroidectomy indicated? Surgery 128:1052-1057; discussion 1057-1058.
222. Szinnai G, Meier C, Komminoth P, Zumsteg UW 2003 Review of multiple endocrine neoplasia type 2A in children: therapeutic results of early thyroidectomy and prognostic value of codon analysis. Pediatrics 111:E132-E139.
223. Calva D, O'Dorisio TM, Sue O'Dorisio M, Lal G, Sugg S, Weigel RJ, Howe JR 2009 When is prophylactic thyroidectomy indicated for patients with the RET codon 609 mutation? Ann Surg Oncol 16:2237-2244.
224. Igaz P, Patócs A, Rácz K, Klein I, Váradi A, Esik O 2002 Occurrence of pheochromocytoma in a MEN2A family with codon 609 mutation of the RET proto-oncogene. J Clin Endocrinol Metab 87:2994.
225. Kinlaw WB, Scott SM, Maue RA, Memoli VA, Harris RD, Daniels GH, Porter DM, Belloni DR, Spooner ET, Ernesti MM, Noll WW 2005 Multiple endocrine neoplasia 2A due to a unique C609S RET mutation presents with pheochromocytoma and reduced penetrance of medullary thyroid carcinoma. Clin Endocrinol (Oxf) 63:676-682.
226. Mian C, Barollo S, Zambonin L, Pennelli G, Bernante P, Pelizzo MR, Nacamulli D, Mantero F, Girelli ME, Opocher G 2009 Characterization of the largest kindred with MEN2A due to a Cys609Ser RET mutation. Fam Cancer 8:379-382.
227. Frilling A, Weber F, Tecklenborg C, Broelsch CE 2003 Prophylactic thyroidectomy in multiple endocrine neoplasia: the impact of molecular mechanisms of RET protooncogene. Langenbecks Arch Surg 388:17-26.
228. Skinner MA, Moley JA, Dilley WG, Owzar K, Debenedetti MK, Wells SA Jr 2005 Prophylactic thyroidectomy in multiple endocrine neoplasia type 2A. N Engl J Med 353:1105-1113.
229. Heizmann O, Haecker FM, Zumsteg U, Muller B, Oberholzer M, Oertli D 2006 Presymptomatic thyroidectomy in multiple endocrine neoplasia 2a. Eur J Surg Oncol 32: 98-102.
230. Lallier M, St-Vil D, Giroux M, Huot C, Gaboury L, Oligny L, Desjardins JG 1998 Prophylactic thyroidectomy for medullary thyroid carcinoma in gene carriers of MEN2 syndrome. J Pediatr Surg 33:846-848.
231. Lau GS, Lang BH, Lo CY, Tso A, Garcia-Barcelo MM, Tam PK, Lam KS 2009 Prophylactic thyroidectomy in ethnic Chinese patients with multiple endocrine neoplasia type 2A syndrome after the introduction of genetic testing. Hong Kong Med J 15:326-331.
232. Learoyd DL, Gosnell J, Elston MS, Saurine TJ, Richardson AL, Delbridge LW, Aglen JV, Robinson BG 2005 Experience of prophylactic thyroidectomy in multiple endocrine neoplasia type 2A kindreds with RET codon 804 mutations. Clin Endocrinol (Oxf) 63:636-641.
233. Rodriguez GJ, Balsalobre MD, Pomares F, Torregrosa NM, Rios A, Carbonell P, Glower G, Sola J, Tebar J, Parrilla P 2002 Prophylactic thyroidectomy in MEN 2A syndrome: experience in a single center. J Am Coll Surg 195:159-166.
234. Niccoli-Sire P, Murat A, Baudin E, Henry JF, Proye C, Bigorgne JC, Bstandig B, Modigliani E, Morange S, Schlumberger M, Conte-Devolx B 1999 Early or prophylactic thyroidectomy in MEN 2/FMTC gene carriers: results in 71 thyroidectomized patients. The French Calcitonin Tumours Study Group (GETC). Eur J Endocrinol 141:468-474.
235. Rohmer V, Vidal-Trecan G, Bourdelot A, Niccoli P, Murat A, Wemeau JL, Borson-Chazot F, Schvartz C, Tabarin A, Chabre O, Chabrier G, Caron P, Rodien P, Schlumberger M, Baudin E; Groupe Francais des Tumeurs Endocrines 2011 Prognostic factors of disease-free survival after thyroidectomy in 170 young patients with a RET germline mutation: a multicenter study of the Groupe Francais d'Etude des Tumeurs Endocrines. J Clin Endocrinol Metab 96:E509-E518.
236. Niccoli-Sire P, Murat A, Rohmer V, Gibelin H, Chabrier G, Conte-Devolx B, Visset J, Ronceray J, Jaeck D, Henry JF, Proye C, Carnaille B, Kraimps JL 2003 When should thyroidectomy be performed in familial medullary thyroid carcinoma gene carriers with non-cysteine RET mutations? Surgery 134:1029-1036.
237. Machens A, Schneyer U, Holzhausen HJ, Dralle H 2005 Prospects of remission in medullary thyroid carcinoma according to basal calcitonin level. J Clin Endocrinol Metab 90:2029-2034.
238. Machens A, Niccoli-Sire P, Hoegel J, Frank-Raue K, van Vroonhoven TJ, Roeher HD, Wahl RA, Lamesch P, Raue F, Conte-Devolx B, Dralle H; European Multiple Endocrine Neoplasia Study Group 2003 Early malignant progression of hereditary medullary thyroid cancer. N Engl J Med 349:1517-1525.
239. Leboulleux S, Travagli JP, Caillou B, Laplanche A, Bidart JM, Schlumberger M, Baudin E 2002 Medullary thyroid carcinoma as part of a multiple endocrine neoplasia type 2B syndrome: influence of the stage on the clinical course. Cancer 94:44-50.
240. Zenaty D, Aigrain Y, Peuchmaur M, Philippe-Chomette P, Baumann C, Cornelis F, Hugot JP, Chevenne D, Barbu V, Guillausseau PJ, Schlumberger M, Carel JC, Travagli JP, Leger J 2009 Medullary thyroid carcinoma identified within the first year of life in children with hereditary multiple endocrine neoplasia type 2A (codon 634) and 2B. Eur J Endocrinol 160:807-813.
241. Wray CJ, Rich TA, Waguespack SG, Lee JE, Perrier ND, Evans DB 2008 Failure to recognize multiple endocrine neoplasia 2B: more common than we think? Ann Surg Oncol 15:293-301.
242. Brauckhoff M, Machens A, Hess S, Lorenz K, Gimm O, Brauckhoff K, Sekulla C, Dralle H 2008 Premonitory symptoms preceding metastatic medullary thyroid cancer in MEN 2B: an exploratory analysis. Surgery 144:1044-1050; discussion 1050-1043.
243. Waguespack SG, Rich TA 2010 Multiple endocrine neoplasia [corrected] syndrome type 2B in early childhood: long-term benefit of prophylactic thyroidectomy. Cancer 116:2284.
244. Brauckhoff M, Machens A, Lorenz K, Bjoro T, Varhaug JE, Dralle H 2014 Surgical curability of medullary thyroid cancer in multiple endocrine neoplasia 2B: a changing perspective. Ann Surg 259:800-806.
245. Brauckhoff M, Gimm O, Weiss CL, Ukkat J, Sekulla C, Brauckhoff K, Thanh PN, Dralle H, 2004 Multiple endocrine neoplasia 2B syndrome due to codon 918 mutation: clinical manifestation and course in early and late onset disease. World J Surg 28:1305-1311.
246. Asari R, Scheuba C, Kaczirek K, Niederle B 2006 Estimated risk of pheochromocytoma recurrence after adrenal-sparing surgery in patients with multiple endocrine neoplasia type 2A. Arch Surg 141:1199-1205; discussion 1205.
247. Brunt LM, Doherty GM, Norton JA, Soper NJ, Quasebarth MA, Moley JF 1996 Laparoscopic adrenalectomy compared to open adrenalectomy for benign adrenal neoplasms. J Am Coll Surg 183:1-10.
248. Miccoli P, Materazzi G, Brauckhoff M, Ambrosini CE, Miccoli M, Dralle H 2011 No outcome differences between a laparoscopic and retroperitoneoscopic approach in synchronous bilateral adrenal surgery. World J Surg 35: 2698-2702.
249. Walz MK, Groeben H, Alesina PF 2010 Single-access retroperitoneoscopic adrenalectomy (SARA) versus conventional retroperitoneoscopic adrenalectomy (CORA): a case-control study. World J Surg 34:1386-1390.
250. Scholten A, Valk GD, Ulfman D, Borel Rinkes IH, Vriens MR 2011 Unilateral subtotal adrenalectomy for pheochromocytoma in multiple endocrine neoplasia type 2 patients: a feasible surgical strategy. Ann Surg 254:1022-1027.
251. Brauckhoff M, Stock K, Stock S, Lorenz K, Sekulla C, Brauckhoff K, Thanh PN, Gimm O, Spielmann RP, Dralle H 2008 Limitations of intraoperative adrenal remnant volume measurement in patients undergoing subtotal adrenalectomy. World J Surg 32:863-872.
252. Brauckhoff M, Dralle H 2012 Funktionserhaltende Adrenalektomie bei Nebennierentumoren. Chirug 83: 519-527.
253. Machens A, Brauckhoff M, Holzhausen HJ, Thanh PN, Lehnert H, Dralle H 2005 Codon-specific development of pheochromocytoma in multiple endocrine neoplasia type 2. J Clin Endocrinol Metab 90:3999-4003.
254. Rowland KJ, Chernock RD, Moley JF 2013 Pheochromocytoma in an 8-year-old patient with multiple endocrine neoplasia type 2A: implications for screening. J Surg Oncol 108:203-206.
255. Nguyen L, Niccoli-Sire P, Caron P, Bastie D, Maes B, Chabrier G, Chabre O, Rohmer V, Lecomte P, Henry JF, Conte-Devolx B; French Calcitonin Tumors Study Group 2001 Pheochromocytoma in multiple endocrine neoplasia type 2: a prospective study. Eur J Endocrinol 144:37-44.
256. Quayle FJ, Fialkowski EA, Benveniste R, Moley JF 2007 Pheochromocytoma penetrance varies by RET mutation in MEN 2A. Surgery 142:800-805; discussion 805.e1.
257. Thosani S, Ayala-Ramirez M, Palmer L, Hu MI, Rich T, Gagel RF, Cote G, Waguespack SG, Habra MA, Jimenez C 2013 The characterization of pheochromocytoma and its impact on overall survival in multiple endocrine neoplasia type 2. J Clin Endocrinol Metab 98:E1813-E1819.
258. Machens A, Lorenz K, Dralle H 2013 Peak incidence of pheochromocytoma and primary hyperparathyroidism in multiple endocrine neoplasia 2: need for age-adjusted biochemical screening. J Clin Endocrinol Metab 98: E336-E345.
259. Modigliani E, Vasen HM, Raue K, Dralle H, Frilling A, Gheri RG, Brandi ML, Limbert E, Niederle B, Forgas L, Rosenberg-Bourgin M, Calmettes C 1995 Pheochromocytoma in multiple endocrine neoplasia type 2: European study. The Euromen Study Group. J Intern Med 238:363-367.
260. Schuffenecker I, Virally-Monod M, Brohet R, Goldgar D, Conte-Devolx B, Leclerc L, Chabre O, Boneu A, Caron J, Houdent C, Modigliani E, Rohmer V, Schlumberger M, Eng C, Guillausseau PJ, Lenoir GM 1998 Risk and penetrance of primary hyperparathyroidism in multiple endocrine neoplasia type 2A families with mutations at codon 634 of the RET proto-oncogene. Groupe D'etude des Tumeurs a Calcitonine. J Clin Endocrinol Metab 83: 487-491.
261. Jackson MB, Guttenberg M, Hedrick H, Moshang T Jr 2005 Multiple endocrine neoplasia type 2A in a kindred with C634Y mutation. Pediatrics 116:e468-e471.
262. Kraimps JL, Denizot A, Carnaille B, Henry JF, Proye C, Bacourt F, Sarfati E, Dupond JL, Maes B, Travagli JP, Boneu A, Roger P, Houdent C, Barbier J, Modigliani E 1996 Primary hyperparathyroidism in multiple endocrine neoplasia type IIa: retrospective French multicentric study. Groupe d'Etude des Tumeurs a Calcitonine (GETC, French Calcitonin Tumors Study Group), French Association of Endocrine Surgeons. World J Surg 20:808-812; discussion 812-813.
263. Raue F, Kraimps JL, Dralle H, Cougard P, Proye C, Frilling A, Limbert E, Llenas LF, Niederle B 1995 Primary hyperparathyroidism in multiple endocrine neoplasia type 2A. J Intern Med 238:369-373.
264. Carling T, Udelsman R 2005 Parathyroid surgery in familial hyperparathyroid disorders. J Intern Med 257: 27-37.
265. Scholten A, Schreinemakers JM, Pieterman CR, Valk GD, Vriens MR, Borel Rinkes IH 2011 Evolution of surgical treatment of primary hyperparathyroidism in patients with multiple endocrine neoplasia type 2A. Endocr Pract 17: 7-15.
266. Irvin GL 3rd, Carneiro DM, Solorzano CC 2004 Progress in the operative management of sporadic primary hyperparathyroidism over 34 years. Ann Surg 239:704-708; discussion 708-711.
267. Agarwal A, Waghray A, Gupta S, Sharma R, Milas M 2013 Cryopreservation of parathyroid tissue: an illustrated technique using the cleveland clinic protocol. J Am Coll Surg 216:e1-e9.
268. Luque-Fernandez I, Garcia-Martin A, Luque-Pazos A 2013 Experience with cinacalcet in primary hyperparathyroidism: results after 1 year of treatment. Ther Adv Endocrinol Metab 4:77-81.
269. Peacock M, Bilezikian JP, Klassen PS, Guo MD, Turner SA, Shoback D 2005 Cinacalcet hydrochloride maintains long-term normocalcemia in patients with primary hyperparathyroidism. J Clin Endocrinol Metab 90:135-141.
270. Engelbach M, Gorges R, Forst T, Pfutzner A, Dawood R, Heerdt S, Kunt T, Bockisch A, Beyer J 2000 Improved diagnostic methods in the follow-up of medullary thyroid carcinoma by highly specific calcitonin measurements. J Clin Endocrinol Metab 85:1890-1894.
271. Ismailov SI, Piulatova NR 2004 Postoperative calcitonin study in medullary thyroid carcinoma. Endocr Relat Cancer 11:357-363.
272. Elisei R, Pinchera A 2012 Advances in the follow-up of differentiated or medullary thyroid cancer. Nat Rev Endocrinol 8:466-475.
273. Pellegriti G, Leboulleux S, Baudin E, Bellon N, Scollo C, Travagli JP, Schlumberger M 2003 Long-term outcome of medullary thyroid carcinoma in patients with normal postoperative medical imaging. Br J Cancer 88:1537-1542.
274. Barbot N, Calmettes C, Schuffenecker I, Saint-Andre JP, Franc B, Rohmer V, Jallet P, Bigorgne JC 1994 Pentagastrin stimulation test and early diagnosis of medullary thyroid carcinoma using an immunoradiometric assay of calcitonin: comparison with genetic screening in hereditary medullary thyroid carcinoma. J Clin Endocrinol Metab 78:114-120.
275. Kouvaraki MA, Shapiro SE, Fornage BD, Edeiken-Monro BS, Sherman SI, Vassilopoulou-Sellin R, Lee JE, Evans DB 2003 Role of preoperative ultrasonography in the surgical management of patients with thyroid cancer. Surgery 134:946-954; discussion 954-945.
276. Koopmans K. P, de Groot JW, Plukker JT, de Vries EG, Kema IP, Sluiter WJ, Jager PL, Links TP 2008 18F-dihydroxyphenylalanine PET in patients with biochemical evidence of medullary thyroid cancer: relation to tumor differentiation. J Nucl Med 49:524-531.
277. Szakall S Jr, Esik O, Bajzik G, Repa I, Dabasi G, Sinkovics I, Agoston P, Tron L 2002 18F-FDG PET detection of lymph node metastases in medullary thyroid carcinoma. J Nucl Med 43:66-71.
278. Gourgiotis L, Sarlis NJ, Reynolds JC, VanWaes C, Merino MJ, Pacak K 2003 Localization of medullary thyroid carcinoma metastasis in a multiple endocrine neoplasia type 2A patient by 6-[18F]-fluorodopamine positron emission tomography. J Clin Endocrinol Metab 88:637-641.
279. Hoegerle S, Altehoefer C, Ghanem N, Brink I, Moser E, Nitzsche E 2001 18F-DOPA positron emission tomography for tumour detection in patients with medullary thyroid carcinoma and elevated calcitonin levels. Eur J Nucl Med 28:64-71.
280. Rubello D, Rampin L, Nanni C, Banti E, Ferdeghini M, Fanti S, Al-Nahhas A, Gross MD 2008 The role of 18FFDG PET/CT in detecting metastatic deposits of recurrent medullary thyroid carcinoma: a prospective study. Eur J Surg Oncol 34:581-586.
281. Ong SC, Schoder H, Patel SG, Tabangay-Lim IM, Doddamane I, Gonen M, Shaha AR, Tuttle RM, Shah JP, Larson SM 2007 Diagnostic accuracy of 18F-FDG PET in restaging patients with medullary thyroid carcinoma and elevated calcitonin levels. J Nucl Med 48:501-507.
282. Baudin E, Lumbroso J, Schlumberger M, Leclere J, Giammarile F, Gardet P, Roche A, Travagli JP, Parmentier C 1996 Comparison of octreotide scintigraphy and conventional imaging in medullary thyroid carcinoma. J Nucl Med 37:912-916.
283. Behr TM, Gratz S, Markus PM, Dunn RM, Hufner M, Schauer A, Fischer M, Munz DL, Becker H, Becker W 1997 Anti-carcinoembryonic antigen antibodies versus somatostatin analogs in the detection of metastatic medullary thyroid carcinoma: are carcinoembryonic antigen and somatostatin receptor expression prognostic factors? Cancer 80:2436-2457.
284. Behr TM, Béhé MP 2002 Cholecystokinin-B/gastrin receptor-targeting peptides for staging and therapy of medullary thyroid cancer and other cholecystokinin-B receptor-expressing malignancies. Semin Nucl Med 32: 97-109.
285. Clarke SE, Lazarus CR, Wraight P, Sampson C, Maisey MN 1988 Pentavalent Tc99m DMSA, I-131 MIBG, and Tc-99m MDP-an evaluation of three imaging techniques in patients with medullary carcinoma of the thyroid. J Nucl Med 29:33-38.
286. Frank-Raue K, Bihl H, Dorr U, Buhr H, Ziegler R, Raue F 1995 Somatostatin receptor imaging in persistent medullary thyroid carcinoma. Clin Endocrinol (Oxf) 42: 31-37.
287. Kwekkeboom DJ, Reubi JC, Lamberts SW, Bruining HA, Mulder AH, Oei HY, Krenning EP 1993 In vivo somatostatin receptor imaging in medullary thyroid carcinoma. J Clin Endocrinol Metab 76:1413-1417.
288. Beheshti M, Pocher S, Vali R, Waldenberger P, Broinger G, Nader M, Kohlfurst S, Pirich C, Dralle H, Langsteger W 2009 The value of 18F-DOPA PET-CT in patients with medullary thyroid carcinoma: comparison with 18F-FDG PET-CT. Eur Radiol 19:1425-1434.
289. Kauhanen S, Schalin-Jantti C, Seppanen M, Kajander S, Virtanen S, Schildt J, Lisinen I, Ahonen A, Heiskanen I, Vaisanen M, Arola J, Korsoff P, Ebeling T, Sane T, Minn H, Valimaki MJ, Nuutila P 2011 Complementary roles of 18F-DOPA PET/CT and 18F-FDG PET/CT in medullary thyroid cancer. J Nucl Med 52:1855-1863.
290. Luster M, Karges W, Zeich K, Pauls S, Verburg FA, Dralle H, Glatting G, Buck AK, Solbach C, Neumaier B, Reske SN, Mottaghy FM 2010 Clinical value of 18-fluorine-fluorodihydroxyphenylalanine positron emission tomography/computed tomography in the follow-up of medullary thyroid carcinoma. Thyroid 20:527-533.
291. Treglia G, Castaldi P, Villani MF, Perotti G, de Waure C, Filice A, Ambrosini V, Cremonini N, Santimaria M, Versari A, Fanti S, Giordano A, Rufini V 2012 Comparison of 18F-DOPA, 18F-FDG and 68Ga-somatostatin analogue PET/CT in patients with recurrent medullary thyroid carcinoma. Eur J Nucl Med Mol Imaging 39: 569-580.
292. Verbeek HH, Plukker JT, Koopmans K. P, de Groot JW, Hofstra RM, Muller Kobold AC, van der Horst-Schrivers AN, Brouwers AH, Links TP 2012 Clinical relevance of 18F-FDG PET and 18F-DOPA PET in recurrent medullary thyroid carcinoma. J Nucl Med 53:1863-1871.
293. Therasse P, Arbuck SG, Eisenhauer EA, Wanders J, Kaplan RS, Rubinstein L, Verweij J, Van Glabbeke M, van Oosterom AT, Christian MC, Gwyther SG 2000 New guidelines to evaluate the response to treatment in solid tumors. European Organization for Research and Treatment of Cancer, National Cancer Institute of the United States, National Cancer Institute of Canada. J Natl Cancer Inst 92:205-216.
294. Barbet J, Campion L, Kraeber-Bodéré F, Chatal JF; GTE Study Group 2005 Prognostic impact of serum calcitonin and carcinoembryonic antigen doubling-times in patients with medullary thyroid carcinoma. J Clin Endocrinol Metab 90:6077-6084.
295. Laure Giraudet A, Al Ghulzan A, Auperin A, Leboulleux S, Chehboun A, Troalen F, Dromain C, Lumbroso J, Baudin E, Schlumberger M 2008 Progression of medullary thyroid carcinoma: assessment with calcitonin and carcinoembryonic antigen doubling times. Eur J Endocrinol 158:239-246.
296. Miyauchi A, Onishi T, Morimoto S, Takai S, Matsuzuka F, Kuma K, Maeda M, Kumahara Y 1984 Relation of doubling time of plasma calcitonin levels to prognosis and recurrence of medullary thyroid carcinoma. Ann Surg 199:461-466.
297. Saad MF, Fritsche HA Jr, Samaan NA 1984 Diagnostic and prognostic values of carcinoembryonic antigen in medullary carcinoma of the thyroid. J Clin Endocrinol Metab 58:889-894.
298. Fialkowski E, DeBenedetti M, Moley J 2008 Long-term outcome of reoperations for medullary thyroid carcinoma. World J Surg 32:754-765.
299. Kebebew E, Kikuchi S, Duh QY, Clark OH 2000 Longterm results of reoperation and localizing studies in patients with persistent or recurrent medullary thyroid cancer. Arch Surg 135:895-901.
300. Tisell LE, Hansson G, Jansson S, Salander H 1986 Reoperation in the treatment of asymptomatic metastasizing medullary thyroid carcinoma. Surgery 99:60-66.
301. Tisell LE, Jansson S 1988 Recent results of reoperative surgery in medullary carcinoma of the thyroid. Wien Klin Wochenschr 100:347-348.
302. Deftos LJ, Stein MF 1980 Radioiodine as an adjunct to the surgical treatment of medullary thyroid carcinoma. J Clin Endocrinol Metab 50:967-968.
303. Hellman DE, Kartchner M, Van Antwerp JD, Salmon SE, Patton DD, O'Mara R 1979 Radioiodine in the treatment of medullary carcinoma of the thyroid. J Clin Endocrinol Metab 48:451-455.
304. Meijer JA, Bakker LE, Valk GD, de Herder WW, de Wilt JH, Netea-Maier RT, Schaper N, Fliers E, Lips P, Plukker JT, Links TP, Smit JA 2013 Radioactive iodine in the treatment of medullary thyroid carcinoma: a controlled multicenter study. Eur J Endocrinol 168:779-786.
305. Brierley JD, Tsang RW 2008 External beam radiation therapy for thyroid cancer. Endocrinol Metab Clin North Am 37:497-509, xi.
306. Fife KM, Bower M, Harmer CL 1996 Medullary thyroid cancer: the role of radiotherapy in local control. Eur J Surg Oncol 22:588-591.
307. Martinez SR, Beal SH, Chen A, Chen SL, Schneider PD 2010 Adjuvant external beam radiation for medullary thyroid carcinoma. J Surg Oncol 102:175-178.
308. Schwartz DL, Rana V, Shaw S, Yazbeck C, Ang KK, Morrison WH, Rosenthal DI, Hoff A, Evans DB, Clayman GL, Garden AS, Sherman SI 2008 Postoperative radiotherapy for advanced medullary thyroid cancer-local disease control in the modern era. Head Neck 30:883-888.
309. Terezakis SA, Lee NY 2010 The role of radiation therapy in the treatment of medullary thyroid cancer. J Natl Compr Canc Netw 8:532-540; quiz 541.
310. Brierley J, Tsang R, Simpson WJ, Gospodarowicz M, Sutcliffe S, Panzarella T 1996 Medullary thyroid cancer: analyses of survival and prognostic factors and the role of radiation therapy in local control. Thyroid 6:305-310.
311. Call JA, Caudill JS, McIver B, Foote RL 2013 A role for radiotherapy in the management of advanced medullary thyroid carcinoma: the mayo clinic experience. Rare Tumors 5:e37.
312. Bhandare N, Mendenhall WM 2012 A literature review of late complications of radiation therapy for head and neck cancers: incidence and dose response. J Nucl Med Radiat Ther S2:009. DOI:10.4172/2155-9619.S2-009.
313. Tung WS, Vesely TM, Moley JF 1995 Laparoscopic detection of hepatic metastases in patients with residual or recurrent medullary thyroid cancer. Surgery 118:1024-1029; discussion 1029-1030.
314. Cupisti K, Wolf A, Raffel A, Schott M, Miersch D, Yang Q, Eisenberger CF, Roher HD, Knoefel WT 2007 Longterm clinical and biochemical follow-up in medullary thyroid carcinoma: a single institution's experience over 20 years. Ann Surg 246:815-821.
315. de Groot JW, Plukker JT, Wolffenbuttel BH, Wiggers T, Sluiter WJ, Links TP 2006 Determinants of life expectancy in medullary thyroid cancer: age does not matter. Clin Endocrinol (Oxf) 65:729-736.
316. Rendl G, Manzl M, Hitzl W, Sungler P, Pirich C 2008 Long-term prognosis of medullary thyroid carcinoma. Clin Endocrinol (Oxf) 69:497-505.
317. Börcek P, Asa SL, Gentili F, Ezzat S, Kiehl TR 2010 Brain metastasis from medullary thyroid carcinoma. BMJ Case Rep pii:bcr0920103301.
318. Kim IY, Kondziolka D, Niranjan A, Flickinger JC, Lunsford LD 2009 Gamma knife radiosurgery for metastatic brain tumors from thyroid cancer. J Neurooncol 93: 355-359.
319. McWilliams RR, Giannini C, Hay ID, Atkinson JL, Stafford SL, Buckner JC 2003 Management of brain metastases from thyroid carcinoma: a study of 16 pathologically confirmed cases over 25 years. Cancer 98: 356-362.
320. Wexler JA 2011 Approach to the thyroid cancer patient with bone metastases. J Clin Endocrinol Metab 96: 2296-2307.
321. Quan GM, Pointillart V, Palussiere J, Bonichon F 2012 Multidisciplinary treatment and survival of patients with vertebral metastases from thyroid carcinoma. Thyroid 22: 125-130.
322. Frassica DA 2003 General principles of external beam radiation therapy for skeletal metastases. Clin Orthop Relat Res 145:S158-164.
323. Vitale G, Fonderico F, Martignetti A, Caraglia M, Ciccarelli A, Nuzzo V, Abbruzzese A, Lupoli G 2001 Pamidronate improves the quality of life and induces clinical remission of bone metastases in patients with thyroid cancer. Br J Cancer 84:1586-1590.
324. Abrahamsen B, Eiken P, Eastell R 2009 Subtrochanteric and diaphyseal femur fractures in patients treated with alendronate: a register-based national cohort study. J Bone Miner Res 24:1095-1102.
325. Khosla S, Burr D, Cauley J, Dempster DW, Ebeling PR, Felsenberg D, Gagel RF, Gilsanz V, Guise T, Koka S, McCauley LK, McGowan J, McKee MD, Mohla S, Pendrys DG, Raisz LG, Ruggiero SL, Shafer DM, Shum L, Silverman SL, Van Poznak CH, Watts N, Woo SB, Shane E 2007 Bisphosphonate-associated osteonecrosis of the jaw: report of a task force of the American Society for Bone and Mineral Research. J Bone Miner Res 22: 1479-1491.
326. Tsutsui H, Kubota M, Yamada M, Suzuki A, Usuda J, Shibuya H, Miyajima K, Sugino K, Ito K, Furukawa K, Kato H 2008 Airway stenting for the treatment of laryngotracheal stenosis secondary to thyroid cancer. Respirology 13:632-638.
327. Fromigue J, De Baere T, Baudin E, Dromain C, Leboulleux S, Schlumberger M 2006 Chemoembolization for liver metastases from medullary thyroid carcinoma. J Clin Endocrinol Metab 91:2496-2499.
328. Isozaki T, Kiba T, Numata K, Saito S, Shimamura T, Kitamura T, Morita K, Tanaka K, Sekihara H 1999 Medullary thyroid carcinoma with multiple hepatic metastases: treatment with transcatheter arterial embolization and percutaneous ethanol injection. Intern Med 38: 17-21.
329. Lorenz K, Brauckhoff M, Behrmann C, Sekulla C, Ukkat J, Brauckhoff K, Gimm O, Dralle H 2005 Selective arterial chemoembolization for hepatic metastases from medullary thyroid carcinoma. Surgery 138:986-993; discussion 993.
330. Wertenbroek MW, Links TP, Prins TR, Plukker JT, van der Jagt EJ, de Jong K. P 2008 Radiofrequency ablation of hepatic metastases from thyroid carcinoma. Thyroid 18: 1105-1110.
331. Santarpia L, El-Naggar AK, Sherman SI, Hymes SR, Gagel RF, Shaw S, Sarlis NJ 2008 Four patients with cutaneous metastases from medullary thyroid cancer. Thyroid 18:901-905.
332. Bajetta E, Rimassa L, Carnaghi C, Seregni E, Ferrari L, Di Bartolomeo M, Regalia E, Cassata A, Procopio G, Mariani L 1998 5-Fluorouracil, dacarbazine, and epirubicin in the treatment of patients with neuroendocrine tumors. Cancer 83:372-378.
333. Orlandi F, Caraci P, Berruti A, Puligheddu B, Pivano G, Dogliotti L, Angeli A 1994 Chemotherapy with dacarba-zine and 5-fluorouracil in advanced medullary thyroid cancer. Ann Oncol 5:763-765.
334. Petursson SR 1988 Metastatic medullary thyroid carcinoma. Complete response to combination chemotherapy with dacarbazine and 5-fluorouracil. Cancer 62:1899-1903.
335. Iten F, Muller B, Schindler C, Rochlitz C, Oertli D, Macke HR, Muller-Brand J, Walter MA 2007 Response to [90Yttrium-DOTA]-TOC treatment is associated with long-term survival benefit in metastasized medullary thyroid cancer: a phase II clinical trial. Clin Cancer Res 13:6696-6702.
336. Chatal JF, Campion L, Kraeber-Bodere F, Bardet S, Vuillez JP, Charbonnel B, Rohmer V, Chang CH, Sharkey RM, Goldenberg DM, Barbet J; French Endocrine Tumor Group 2006 Survival improvement in patients with medullary thyroid carcinoma who undergo pretargeted anti-carcinoembryonic-antigen radioimmunotherapy: a collaborative study with the French Endocrine Tumor Group. J Clin Oncol 24:1705-1711.
337. Kraeber-Bodere F, Rousseau C, Bodet-Milin C, Ferrer L, Faivre-Chauvet A, Campion L, Vuillez JP, Devillers A, Chang CH, Goldenberg DM, Chatal JF, Barbet J 2006 Targeting, toxicity, and efficacy of 2-step, pretargeted radioimmunotherapy using a chimeric bispecific antibody and 131I-labeled bivalent hapten in a phase I optimization clinical trial. J Nucl Med 47:247-255.
338. Maiza JC, Grunenwald S, Otal P, Vezzosi D, Bennet A, Caron P 2012 Use of 131I-MIBG therapy in MIBGpositive metastatic medullary thyroid carcinoma. Thyroid 22:654-655.
339. Pasieka JL, McEwan AJ, Rorstad O 2004 The palliative role of 131I-MIBG and 111In-octreotide therapy in patients with metastatic progressive neuroendocrine neoplasms. Surgery 136:1218-1226.
340. Capp C, Wajner SM, Siqueira DR, Brasil BA, Meurer L, Maia AL 2010 Increased expression of vascular endothelial growth factor and its receptors, VEGFR-1 and VEGFR-2, in medullary thyroid carcinoma. Thyroid 20: 863-871.
341. Schlumberger MJ, Elisei R, Bastholt L, Wirth LJ, Martins RG, Locati LD, Jarzab B, Pacini F, Daumerie C, Droz JP, Eschenberg MJ, Sun YN, Juan T, Stepan DE, Sherman SI 2009 Phase II study of safety and efficacy of motesanib in patients with progressive or symptomatic, advanced or metastatic medullary thyroid cancer. J Clin Oncol 27: 3794-3801.
342. Cohen EE, Rosen LS, Vokes EE, Kies MS, Forastiere AA, Worden FP, Kane MA, Sherman E, Kim S, Bycott P, Tortorici M, Shalinsky DR, Liau KF, Cohen RB 2008 Axitinib is an active treatment for all histologic subtypes of advanced thyroid cancer: results from a phase II study. J Clin Oncol 26:4708-4713.
343. de Groot JW, Zonnenberg BA, van Ufford-Mannesse PQ, de Vries MM, Links TP, Lips CJ, Voest EE 2007 A phase II trial of imatinib therapy for metastatic medullary thyroid carcinoma. J Clin Endocrinol Metab 92:3466-3469.
344. Frank-Raue K, Fabel M, Delorme S, Haberkorn U, Raue F 2007 Efficacy of imatinib mesylate in advanced medullary thyroid carcinoma. Eur J Endocrinol 157:215-220.
345. Kurzrock R, Sherman SI, Ball DW, Forastiere AA, Cohen RB, Mehra R, Pfister DG, Cohen EE, Janisch L, Nauling F, Hong DS, Ng CS, Ye L, Gagel RF, Frye J, Muller T, Ratain MJ, Salgia R 2011 Activity of XL184 (Cabozantinib), an oral tyrosine kinase inhibitor, in patients with medullary thyroid cancer. J Clin Oncol 29:2660-2666.
346. Lam ET, Ringel MD, Kloos RT, Prior TW, Knopp MV, Liang J, Sammet S, Hall NC, Wakely PE Jr, Vasko VV, Saji M, Snyder PJ, Wei L, Arbogast D, Collamore M, Wright JJ, Moley JF, Villalona-Calero MA, Shah MH 2010 Phase II clinical trial of sorafenib in metastatic medullary thyroid cancer. J Clin Oncol 28:2323-2330.
347. Pennell NA, Daniels GH, Haddad RI, Ross DS, Evans T, Wirth LJ, Fidias PH, Temel JS, Gurubhagavatula S, Heist RS, Clark JR, Lynch TJ 2008 A phase II study of gefitinib in patients with advanced thyroid cancer. Thyroid 18: 317-323.
348. Wells SA Jr, Gosnell JE, Gagel RF, Moley J, Pfister D, Sosa JA, Skinner M, Krebs A, Vasselli J, Schlumberger M 2010 Vandetanib for the treatment of patients with locally advanced or metastatic hereditary medullary thyroid cancer. J Clin Oncol 28:767-772.
349. Robinson BG, Paz-Ares L, Krebs A, Vasselli J, Haddad R 2010 Vandetanib (100 mg) in patients with locally advanced or metastatic hereditary medullary thyroid cancer. J Clin Endocrinol Metab 95:2664-2671.
350. Wells SA Jr, Robinson BG, Gagel RF, Dralle H, Fagin JA, Santoro M, Baudin E, Elisei R, Jarzab B, Vasselli JR, Read J, Langmuir P, Ryan AJ, Schlumberger MJ 2012 Vandetanib in patients with locally advanced or metastatic medullary thyroid cancer: a randomized, double-blind phase III trial. J Clin Oncol 30:134-141.
351. Elisei R, Schlumberger MJ, Müller SP, Schöffski P, Brose MS, Shah MH, Licitra L, Jarzab B, Medvedev V, Kreissl MC, Niederle B, Cohen EE, Wirth LJ, Ali H, Hessel C, Yaron Y, Ball D, Nelkin B, Sherman SI 2013 Cabozantinib in progressive medullary thyroid cancer. J Clin Oncol 31:3639-3646.
352. Sherman SI, Cohen EEW, Schoffski P, Elisei R, Schlumberger M, Wirth LJ, Mangeshkar M, Aftab DT, Clary DO, Brose MS 2013 Efficacy of cabozantinib (Cabo) in medullary thyroid cancer (MTC) patients with RAS or RET mutations: results from a phase III study. J Clin Oncol 31: abstract 6000.
353. Brassard M, Neraud B, Trabado S, Salenave S, Brailly-Tabard S, Borget I, Baudin E, Leboulleux S, Chanson P, Schlumberger M, Young J 2011 Endocrine effects of the tyrosine kinase inhibitor vandetanib in patients treated for thyroid cancer. J Clin Endocrinol Metab 96:2741-2749.
354. Abdulrahman RM, Verloop H, Hoftijzer H, Verburg E, Hovens GC, Corssmit EP, Reiners C, Gelderblom H, Pereira AM, Kapiteijn E, Romijn JA, Visser TJ, Smit JW 2010 Sorafenib-induced hypothyroidism is associated with increased type 3 deiodination. J Clin Endocrinol Metab 95:3758-3762.
355. Pinto A 2014 Cabozantinib: a novel agent with a dual mechanism of action for castration-resistant prostate carcinoma. Cancer Chemother Pharmacol 73:219-222.
356. Cox TM, Fagan EA, Hillyard CJ, Allison DJ, Chadwick VS 1979 Role of calcitonin in diarrhoea associated with medullary carcinoma of the thyroid. Gut 20:629-633.
357. Rambaud JC, Jian R, Flourie B, Hautefeuille M, Salmeron M, Thuillier F, Ruskone A, Florent C, Chaoui F, Bernier JJ 1988 Pathophysiological study of diarrhoea in a patient with medullary thyroid carcinoma. Evidence against a secretory mechanism and for the role of shortened colonic transit time. Gut 29:537-543.
358. Mahler C, Verhelst J, de Longueville M, Harris A 1990 Long-term treatment of metastatic medullary thyroid carcinoma with the somatostatin analogue octreotide. Clin Endocrinol (Oxf) 33:261-269.
359. Vainas I, Koussis C, Pazaitou-Panayiotou K, Drimonitis A, Chrisoulidou A, Iakovou I, Boudina M, Kaprara A, Maladaki A 2004 Somatostatin receptor expression in vivo and response to somatostatin analog therapy with or without other antineoplastic treatments in advanced medullary thyroid carcinoma. J Exp Clin Cancer Res 23:549-559.
360. Lupoli G, Cascone E, Arlotta F, Vitale G, Celentano L, SalvatoreM, LombardiG1996Treatment of advancedmedullary thyroid carcinoma with a combination of recombinant interferon alpha-2b and octreotide. Cancer 78:1114-1118.
361. Vitale G, Tagliaferri P, Caraglia M, Rampone E, Ciccarelli A, Bianco AR, Abbruzzese A, Lupoli G 2000 Slow release lanreotide in combination with interferon-alpha2b in the treatment of symptomatic advanced medullary thyroid carcinoma. J Clin Endocrinol Metab 85:983-988.
362. Ilias I, Torpy DJ, Pacak K, Mullen N, Wesley RA, Nieman LK 2005 Cushing's syndrome due to ectopic corticotropin secretion: twenty years' experience at the National Institutes of Health. J Clin Endocrinol Metab 90: 4955-4962.
363. Wajchenberg BL, Mendonca BB, Liberman B, Pereira MA, Carneiro PC, Wakamatsu A, Kirschner MA 1994 Ectopic adrenocorticotropic hormone syndrome. Endocr Rev 15:752-787.
364. Pozza C, Graziadio C, Giannetta E, Lenzi A, Isidori AM 2012 Management strategies for aggressive Cushing's syndrome: from macroadenomas to ectopics. J Oncol 2012:685213.
365. Baudry C, Paepegaey AC, Groussin L 2013 Reversal of Cushing's syndrome by vandetanib in medullary thyroid carcinoma. N Engl J Med 369:584-586.
366. Fox E, Widemann BC, Chuk MK, Marcus L, Aikin A, Whitcomb PO, Merino MJ, Lodish M, Dombi E, Steinberg SM, Wells SA, Balis FM 2013 Vandetanib in children and adolescents with multiple endocrine neoplasia type 2B associated medullary thyroid carcinoma. Clin Cancer Res 19:4239-4248.