The north american neuroendocrine tumor society consensus guideline for the diagnosis and management of neuroendocrine tumors: Pheochromocytoma, paraganglioma, and medullary thyroid cancer

Pancreas

Volumn 39, Issue 6, 2010, Pages 775-783

  Chen, Herbert ^a   Sippel, Rebecca S ^a   O'Dorisio, M Sue ^b   Vinik, Aaron I ^c   Lloyd, Ricardo V ^d   Pacak, Karel ^e  

^a UNIVERSITY OF WISCONSIN SCHOOL OF MEDICINE AND PUBLIC HEALTH   (United States)

^b UNIVERSITY OF IOWA   (United States)

^c EASTERN VIRGINIA MEDICAL SCHOOL   (United States)

^d UNIVERSITY OF WISCONSIN MADISON   (United States)

^e EUNICE KENNEDY SHRIVER NATIONAL INSTITUTE OF CHILD HEALTH AND HUMAN DEVELOPMENT   (United States)

Author keywords

medullary thyroid cancer; neuroendocrine tumors; paraganglioma; pheochromocytoma

Indexed keywords

(3 IODOBENZYL)GUANIDINE I 123; 1,4,7,10 TETRAAZACYCLODODECANNE OCTREOTATE LUTETIUM 177; ADRENALIN; ANTINEOPLASTIC AGENT; BETA ADRENERGIC RECEPTOR BLOCKING AGENT; CALCITONIN; CALCIUM; CARCINOEMBRYONIC ANTIGEN; CATECHOLAMINE; CHROMOGRANIN A; CYCLOPHOSPHAMIDE; DACARBAZINE; DOXAZOSIN; FLUORODEOXYGLUCOSE F 18; FLUORODIHYDROXYPHENYLALANINE FLUORINE 18; IMATINIB; METADRENALIN; MOTESANIB; NORADRENALIN; NORMETADRENALIN; PHENOXYBENZAMINE; PROTEIN RET; RADIOPHARMACEUTICAL AGENT; SOMATOSTATIN DERIVATIVE; SUCCINATE DEHYDROGENASE; TERAZOSIN; UNCLASSIFIED DRUG; VANDETANIB; VANILMANDELIC ACID; VINCRISTINE; VITAMIN D;

ADRENALECTOMY; ADRENALIN BLOOD LEVEL; ALPHA ADRENERGIC RECEPTOR BLOCKING; ANGINA PECTORIS; BETA ADRENERGIC RECEPTOR BLOCKING; BLOOD PRESSURE REGULATION; BONE METASTASIS; CANCER COMBINATION CHEMOTHERAPY; CANCER DIAGNOSIS; CANCER MORTALITY; CANCER PALLIATIVE THERAPY; CANCER PATIENT; CANCER RADIOTHERAPY; CANCER RECURRENCE; CANCER SCINTISCANNING; CANCER SURGERY; CATECHOLAMINE RELEASE; CLINICAL TRIAL; COMPUTER ASSISTED TOMOGRAPHY; CONSENSUS; CYTOREDUCTIVE SURGERY; DIAGNOSTIC IMAGING; DISEASE ASSOCIATION; DISEASE SEVERITY; DISTANT METASTASIS; DRUG HALF LIFE; DRUG MEGADOSE; EXTERNAL BEAM RADIOTHERAPY; GENE MUTATION; GENETIC SCREENING; HISTOPATHOLOGY; HUMAN; HYPERPARATHYROIDISM; HYPERTENSION; LAPAROSCOPIC SURGERY; LIVER METASTASIS; LYMPH NODE METASTASIS; LYMPHADENECTOMY; MEDICAL SOCIETY; MICROMETASTASIS; MOLECULAR GENETICS; MULTIPLE ENDOCRINE NEOPLASIA; NECK DISSECTION; NEUROENDOCRINE TUMOR; NEUROFIBROMATOSIS; NORADRENALIN BLOOD LEVEL; NUCLEAR MAGNETIC RESONANCE IMAGING; PARAGANGLIOMA; PHEOCHROMOCYTOMA; POINT MUTATION; POSITRON EMISSION TOMOGRAPHY; PREVALENCE; PRIORITY JOURNAL; REVIEW; SENSITIVITY AND SPECIFICITY; SUCCINATE DEHYDROGENASE TYPE 2A; SUCCINATE DEHYDROGENASE TYPE 2B; TACHYCARDIA; THYROID MEDULLARY CARCINOMA; THYROIDECTOMY; VASOCONSTRICTION; VON HIPPEL LINDAU DISEASE;

HUMANS; NEUROENDOCRINE TUMORS; PARAGANGLIOMA; PHEOCHROMOCYTOMA; THYROID NEOPLASMS;

EID: 77955214443     PISSN: 08853177     EISSN: None     Source Type: Journal    
DOI: 10.1097/MPA.0b013e3181ebb4f0     Document Type: Review

References (77)

1. Lenders JW, Eisenhofer G, Mannelli M, et al. Phaeochromocytoma. Lancet. 2005;366(9486):665-675.
2. Manger WM, Gifford RW. Pheochromocytoma. J Clin Hypertens. 2002;4(1):62-72.
3. Van Duinen N, Steenvoorden D, Kema IP, et al. Increased urinary excretion of 3-methoxytyramine in patients with head and neck paragangliomas. J Clin Endocrinol Metab. 2010;95:209-214.
4. DeLellis RA, Lloyd RV, Heitz PU, et al. Tumours of Endocrine Organs. Lyon: IARC Press; 2004.
5. Elder EE, Elder G, Larsson C. Pheochromocytoma and functional paraganglioma syndrome: no longer the 10% tumor. J Surg Oncol. 2005;89(3):193-201.
6. Neumann HP, Bausch B, McWhinney SR, et al. Germ-line mutations in nonsyndromic pheochromocytoma. N Engl J Med. 2002;346(19):1459-1466.
7. Barontini M, Levin G, Sanso G. Characteristics of pheochromocytoma in a 4- to 20-year-old population. Ann N Y Acad Sci. 2006;1073:30-37.
8. De Krijger RR, Van Nederveen FH, Korpershoek E, et al. Frequent genetic changes in childhood pheochromocytomas. Ann N Y Acad Sci. 2006;1073:166-176.
9. Ludwig AD, Feig DI, Brandt ML, et al. Recent advances in the diagnosis and treatment of pheochromocytoma in children. Am J Surg. 2007;194(6):792-796; discussion 6-7.
10. Benn DE, Gimenez-Roqueplo AP, Reilly JR, et al. Clinical presentation and penetrance of pheochromocytoma/paraganglioma syndromes. J Clin Endocrinol Metab. 2006;91(3):827-836.
11. Gimenez-Roqueplo AP, Favier J, Rustin P, et al. Mutations in the SDHB gene are associated with extra-adrenal and/or malignant phaeochromocytomas. Cancer Res. 2003;63(17):5615-5621.
12. Bryant J, Farmer J, Kessler LJ, et al. Pheochromocytoma: the expanding genetic differential diagnosis. J Natl Cancer Inst. 2003;95(16):1196-1204.
13. Brouwers FM, Eisenhofer G, Tao JJ, et al. High frequency of SDHB germline mutations in patients with malignant catecholamine-producing paragangliomas: implications for genetic testing. J Clin Endocrinol Metab. 2006;91(11):4505- 4509.
14. Timmers HJ, Kozupa A, Eisenhofer G, et al. Clinical presentations, biochemical phenotypes, and genotype-phenotype correlations in patients with succinate dehydrogenase subunit B-associated pheochromocytomas and paragangliomas. J Clin Endocrinol Metab. 2007;92(3):779-786.
15. Amar L, Bertherat J, Baudin E, et al. Genetic testing in pheochromocytoma or functional paraganglioma. J Clin Oncol. 2005;23(34):8812-8818.
16. Lenders JW, Pacak K, Walther MM, et al. Biochemical diagnosis of pheochromocytoma: which test is best? JAMA. 2002;287(11):1427-1434.
17. Eisenhofer G, Lenders J, Linehan W, et al. Plasma normetanephrine and metanephrine for detecting pheochromocytoma in von Hippel-Lindau disease and multiple endocrine neoplasia type 2. N Engl J Med. 1999;340:1872-1879.
18. Sawka AM, Jaeschke R, Singh RJ, et al. A comparison of biochemical tests for pheochromocytoma: measurement of fractionated plasma metanephrines compared with the combination of 24-hour urinary metanephrines and catecholamines. J Clin Endocrinol Metab. 2003;88(2):553-558.
19. Lenders JW, Willemsen JJ, Eisenhofer G, et al. Is supine rest necessary before blood sampling for plasma metanephrines? Clin Chem. 2007;53(2):352-354.
20. Grossman A, Pacak K, Sawka A, et al. Biochemical diagnosis and localization of pheochromocytoma: can we reach a consensus? Ann N Y Acad Sci. 2006;1073:332-347.
21. Eisenhofer G, Goldstein DS, Walther MM, et al. Biochemical diagnosis of pheochromocytoma: how to distinguish true- from false-positive test results. J Clin Endocrinol Metab. 2003;88(6):2656-2666.
22. Baid S, Lai EW, Wesley RA, et al. Contrast enhanced computed tomography does not induce catecholamine release in patients with pheochromocytoma. Ann Intern Med. 2009;150:27-32.
23. Pacak K, Eisenhofer G, Goldstein DS. Functional imaging of endocrine tumors: role of positron emission tomography. Endocr Rev. 2004;25(4):568-580.
24. Lumachi F, Tregnaghi A, Zucchetta P, et al. Sensitivity and positive predictive value of CT, MRI and 123I-MIBG scintigraphy in localizing pheochromocytomas: a prospective study. Nucl Med Commun. 2006;27(7):583-587.
25. van der Harst E, de Herder WW, Bruining HA, et al. [(123)I] metaiodobenzylguanidine and [(111)In]octreotide uptake in benign and malignant pheochromocytomas. J Clin Endocrinol Metab. 2001;86(2):685-693.
26. Van Der Horst-Schrivers AN, Jager PL, Boezen HM, et al. Iodine-123 metaiodobenzylguanidine scintigraphy in localising phaeochromocytomasVexperience and meta-analysis. Anticancer Res. 2006;26(2B):1599-1604.
27. Bhatia KS, Ismail MM, Sahdev A, et al. 123I-metaiodobenzylguanidine (MIBG) scintigraphy for the detection of adrenal and extra-adrenal phaeochromocytomas: CT and MRI correlation. Clin Endocrinol (Oxf ). 2008;69(2):181-188.
28. Mackenzie IS, Gurnell M, Balan KK, et al. The use of 18-fluoro- dihydroxyphenylalanine and 18-fluorodeoxyglucose positron emission tomography scanning in the assessment of metaiodobenzylguanidine-negative phaeochromocytoma. Eur J Endocrinol. 2007;157(4):533-537.
29. Ilias I, Yu J, Carrasquillo JA, et al. Superiority of 6-[18F]-fluorodopamine positron emission tomography versus [131I]- metaiodobenzylguanidine scintigraphy in the localization of metastatic pheochromocytoma. J Clin Endocrinol Metab. 2003;88(9): 4083-4087.
30. Hoegerle S, Nitzsche E, Altehoefer C, et al. Pheochromocytomas: detection with 18F DOPA whole body PETVinitial results. Radiology. 2002;222(2):507-512.
31. Timmers HJ, Hadi M, Carrasquillo JA, et al. The effects of carbidopa on uptake of 6-18F-Fluoro-L-DOPA in PET of pheochromocytoma and extraadrenal abdominal paraganglioma. J Nucl Med. 2007;48(10):1599-1606.
32. Shulkin BL, Koeppe RA, Francis IR, et al. Pheochromocytomas that do not accumulate metaiodobenzylguanidine: localization with PET and administration of FDG. Radiology. 1993;186(3):711-715.
33. Shulkin BL, Thompson NW, Shapiro B, et al. Pheochromocytomas: imaging with 2-[fluorine-18]fluoro-2-deoxy-D-glucose PET. Nucl Med. 1999;212:35-41.
34. Timmers HJ, Kozupa A, Chen CC, et al. Superiority of fluorodeoxyglucose positron emission tomography to other functional imaging techniques in the evaluation of metastatic SDHB-associated pheochromocytoma and paraganglioma. J Clin Oncol. 2007;25(16): 2262-2269.
35. Pacak KLJ, Eisenhofer G. Pheochromocytoma; diagnosis, localization and treatment. 2007.
36. Pacak K, Eisenhofer G, Ahlman H, et al. Pheochromocytoma: recommendations for clinical practice from the first international symposium. Nat Clin Pract Endocrinol Metab. 2007;3(2):92-102.
37. Pacak K. Preoperative management of the pheochromocytoma patient. J Clin Endocrinol Metab. 2007;92(11):4069-4079.
38. Goldstein RE, O'Neill JA Jr, Holcomb GW 3rd, et al. Clinical experience over 48 years with pheochromocytoma. Ann Surg. 1999;229(6):755-764.
39. Hack HA. The perioperative management of children with phaeochromocytoma. Paediatr Anaesth. 2000;10(5):463-476.
40. Gedik GK, Hoefnagel CA, Bais E, et al. 131I-MIBG therapy in metastatic phaeochromocytoma and paraganglioma. Eur J Nucl Med Mol Imaging. 2008;35(4):725-733.
41. Averbuch SD, Steakley CS, Young RC, et al. Malignant pheochromocytoma: effective treatment with a combination of cyclophosphamide, vincristine, and dacarbazine. Ann Intern Med. 1988;109(4):267-273.
42. van Essen M, Krenning EP, Kooij PP, et al. Effects of therapy with [177Lu-DOTA0, Tyr3]octreotate in patients with paraganglioma, meningioma, small cell lung carcinoma, and melanoma. J Nucl Med. 2006;47(10):1599-1606.
43. Pelizzo MR, Boschin IM, Bernante P, et al. Natural history, diagnosis, treatment and outcome of medullary thyroid cancer: 37 years experience on 157 patients. Eur J Surg Oncol. 2007;33:493-497.
44. Moley JF, Lairmore TC, Phay JE. Hereditary endocrinopathies. Curr Probl Surg. 1999;36:653-762.
45. Ball DW. Medullary thyroid cancer: therapeutic targets and molecular markers. Curr Opin Oncol. 2007;19:18-23.
46. Roman S, Lin R, Sosa JA. Prognosis of medullary thyroid carcinoma: demographic, clinical, and pathologic predictors of survival in 1252 cases. Cancer. 2006;107:2134-2142.
47. Lal A, Bianco J, Chen H. Radioguided parathyroidectomy in patients with familial hyperparathyroidism. Ann Surg Oncol. 2007;14:739-743.
48. Sippel RS, Kunnimalaiyaan M, Chen H. Current management of medullary thyroid cancer. Oncologist. 2008;13:539-547.
49. Hansford JR, Mulligan LM. Multiple endocrine neoplasia type 2 and RET: from neoplasia to neurogenesis [review]. J Med Genet. 2000;37:817-827.
50. Kouvaraki MA, Shapiro SE, Perrier ND, et al. RET proto-oncogene: a review and update of genotype-phenotype correlations in hereditary medullary thyroid cancer and associated endocrine tumors. Thyroid. 2005;15:531-544.
51. Brandi ML, Gagel RF, Angeli A, et al. Guidelines for diagnosis and therapy of MEN type 1 and type 2. J Clin Endocrinol Metab. 2001;86:5658-5671.
52. Cohen R, Campos JM, Salaun C, et al. Preoperative calcitonin levels are predictive of tumor size and postoperative calcitonin normalization in medullary thyroid carcinoma. Groupe d'Etudes des Tumeurs a Calcitonine (GETC). J Clin Endocrinol Metab. 2000;85:919-922.
53. Barbet J, Campion L, Kraeber-Bodere F, et al. Prognostic impact of serum calcitonin and carcinoembryonic antigen doubling-times in patients with medullary thyroid carcinoma. J Clin Endocrinol Metab. 2005;90:6077-6084.
54. Quayle FJ, Benveniste R, DeBenedetti MK, et al. Hereditary medullary thyroid carcinoma in patients greater than 50 years old. Surgery. 2004;136:1116-1121.
55. Tung WS, Vesely TM, Moley JF. Laparoscopic detection of hepatic metastases in patients with residual or recurrent medullary thyroid cancer. Surgery. 1995;118:1024-1029.
56. Skinner MA, Moley JA, Dilley WG, et al. Prophylactic thyroidectomy in multiple endocrine neoplasia type 2A. N Engl J Med. 2005;353:1105-1113.
57. Moley JF, DeBenedetti MK. Patterns of nodal metastases in palpable medullary thyroid carcinoma: recommendations for extent of node dissection. Ann Surg. 1999;229:880-887.
58. Greenblatt DY, Elson D, Mack E, et al. Initial lymph node dissection increases cure rates in patients with medullary thyroid cancer. Asian J Surg. 2007;30:108-112.
59. Machens A, Hauptmann S, Dralle H. Increased risk of lymph node metastasis in multifocal hereditary and sporadic medullary thyroid cancer. World J Surg. 2007;31:1960-1965.
60. Scollo C, Baudin E, Travagli JP, et al. Rationale for central and bilateral lymph node dissection in sporadic and hereditary medullary thyroid cancer. J Clin Endocrinol Metab. 2003;88:2070-2075.
61. Kebebew E, Greenspan FS, Clark OH, et al. Extent of disease and practice patterns for medullary thyroid cancer. J Am Coll Surg. 2005;200:890-896.
62. Chen H, Roberts JR, Ball DW, et al. Effective long-term palliation of symptomatic, incurable metastatic medullary thyroid cancer by operative resection. Ann Surg. 1998;227:887-895.
63. de Groot JW, Zonnenberg BA, Ufford-Mannesse PQ, et al. A phase II trial of imatinib therapy for metastatic medullary thyroid carcinoma. J Clin Endocrinol Metab. 2007;92:3466-3469.
64. Wells SA, Gosnell JE, Gagel RF, et al. Vandetanib for the treatment of patients with locally advanced or metastatic hereditary medullary thyroid cancer. J Clin Oncol. 2010;28:767-772.
65. Schlumberger MJ, Elisei R, Bastholt L, et al. Phase II study of safety and efficacy of motesanib in patients with progressive or symptomatic, advanced or metastatic medullary thyroid cancer. J Clin Oncol. 2009;27:3794-3801.
66. Sherman SI, Wirth LJ, Droz JP, et al. Motesanib diphosphate in progressive differentiated thyroid cancer. N Engl J Med. 2008;359: 31-42.
67. Moley JF, DeBenedetti MK, Dilley WG, et al. Surgical management of patients with persistent or recurrent medullary thyroid cancer. J Intern Med. 1998;243:521-526.
68. Moley JF, Wells SA, Dilley WG, et al. Reoperation for recurrent or persistent medullary thyroid cancer. Surgery. 1993;114:1090-1095.
69. Van Gompel JJ, Kunnimalaiyaan M, Holen K, et al. ZM336372, a Raf-1 activator, suppresses growth and neuroendocrine hormone levels in carcinoid tumor cells. Mol Cancer Ther. 2005;4:910-917.
70. Ning L, Jaskula-Sztul R, Kunnimalaiyaan M, et al. Suberoyl bishydroxamic acid activates notch1 signaling and suppresses tumor progression in an animal model of medullary thyroid carcinoma. Ann Surg Oncol. 2008;15:2600-2605.
71. Kunnimalaiyaan M, Vaccaro AM, Ndiaye MA, et al. Inactivation of glycogen synthase kinase-3 beta, a downstream target of the raf-1 pathway, is associated with growth suppression in medullary thyroid cancer cells. Mol Cancer Ther. 2007;6:1151-1158.
72. Kunnimalaiyaan M, Ndiaye M, Chen H. Apoptosis-mediated medullary thyroid cancer growth suppression by the PI3K inhibitor LY294002. Surgery. 2006;140:1009-1014.
73. Kunnimalaiyaan M, Vaccaro AM, Ndiaye MA, et al. Overexpression of the NOTCH1 intracellular domain inhibits cell proliferation and alters the neuroendocrine phenotype of medullary thyroid cancer cells. J Biol Chem. 2006;281:39819-39830.
74. Sippel RS, Carpenter JE, Kunnimalaiyaan M, et al. The role of human achaete-scute homolog-1 in medullary thyroid cancer cells. Surgery. 2003;134:866-871.
75. Greenblatt DY, Cayo MA, Adler JT, et al. Valproic acid activates Notch1 signaling and induces apoptosis in medullary thyroid cancer cells. Ann Surg. 2008;247:1036-1040.
76. Weise M, Merke D, Pacak K, et al. Utility of plasma free metanephrines for detecting childhood pheochromocytoma. J Clin Endocrinol Metab. 2002;87:1955-1960.
77. Zelinka T, Eisenhofer G, Pacak K. Pheochromocytoma as a catecholamine producing tumor: implications for clinical practice. Stress. 2007;10(2):195-203.