Neuroendocrine carcinoma of unknown primary: A systematic review of the literature and a comparative study with other neuroendocrine tumors

Cancer Treatment Reviews

Volumn 37, Issue 5, 2011, Pages 358-365

  Stoyianni, Aikaterini ^a   Pentheroudakis, George ^a   Pavlidis, Nicholas ^a  

^a UNIVERSITY HOSPITAL OF IOANNINA   (Greece)

Author keywords

Carcinoma of unknown primary; Neuroendocrine tumors; Prognosis; Survival

Indexed keywords

BEVACIZUMAB; BLEOMYCIN; DOXORUBICIN; ERLOTINIB; ETOPOSIDE; FLUOROURACIL; IMATINIB; INTERFERON; OXALIPLATIN; SOMATOSTATIN DERIVATIVE; STREPTOZOCIN; SUNITINIB;

CANCER CHEMOTHERAPY; CANCER GRADING; CANCER LOCALIZATION; CANCER OF UNKNOWN PRIMARY SITE; CANCER PATIENT; CANCER SURVIVAL; CARCINOID; CARCINOMA; CELL DIFFERENTIATION; COHORT ANALYSIS; COMPARATIVE STUDY; DISEASE COURSE; GASTROINTESTINAL DISEASE; HISTOPATHOLOGY; HUMAN; LUNG CARCINOMA; LUNG SMALL CELL CANCER; NEUROENDOCRINE TUMOR; PANCREAS ISLET CELL TUMOR; PROGNOSIS; PUBLICATION; RETROSPECTIVE STUDY; REVIEW; SYSTEMATIC REVIEW; TREATMENT OUTCOME;

CARCINOMA, NEUROENDOCRINE; COMBINED MODALITY THERAPY; DISEASE-FREE SURVIVAL; FEMALE; HUMANS; MALE; NEOPLASM INVASIVENESS; NEOPLASM STAGING; NEOPLASMS, UNKNOWN PRIMARY; PROGNOSIS; RISK ASSESSMENT; SURVIVAL ANALYSIS; TREATMENT OUTCOME;

EID: 79957483619     PISSN: 03057372     EISSN: 15321967     Source Type: Journal    
DOI: 10.1016/j.ctrv.2011.03.002     Document Type: Review

References (87)

1. Pavlidis N., Fizazi K. Cancer of unknown primary (CUP). Crit Rev Oncol Hematol 2005, 54(3):243-250.
2. Pavlidis N., Briasoulis E., Hainsworth J., Greco F.A. Diagnostic and therapeutic management of cancer of an unknown primary. Eur J Cancer 2004, 40(9):1454-1455.
3. Spigel D.R., Hainsworth J., Greco F.A. Neuroendocrine carcinoma of unknown primary site. Semin Oncol 2009, 36(1):52-59.
4. Hainsworth J.D., Johnson D.H., Greco F.A. Poorly differentiated neuroendocrine carcinoma of unknown primary site. A newly recognized clinicopathologic entity. Ann Intern Med 1988, 109(5):364-371.
5. Abbruzzese J.L., Abbruzzese M.C., Hess K.R., Raber M.N., et al. Unknown primary carcinoma: natural history and prognostic factors in 657 consecutive patients. J Clin Oncol 1994, 12(6):1272-1280.
6. Lenzi R., Hess K.R., Abbruzzese M.C., Raber M.N., et al. Poorly differentiated carcinoma and poorly differentiated adenocarcinoma of unknown origin: favorable subsets of patients with unknown-primary carcinoma?. J Clin Oncol 1997, 15(5):2056-2066.
7. Kirshbom P.M., Kherani A.R., Onaitis M.W., Feldman J.M., et al. Carcinoids of unknown origin: comparative analysis with foregut, midgut, and hindgut carcinoids. Surgery 1998, 124(6):1063-1070.
8. Greco F.A., Burris H.A., Erland J.B., Gray J.R., et al. Carcinoma of unknown primary site. Cancer 2000, 89(12):2655-2660.
9. Greco F.A., Erland J.B., Morrissey L.H., Burris H.A., et al. Carcinoma of unknown primary site: phase II trials with docetaxel plus cisplatin or carboplatin. Ann Oncol 2000, 11(2):211-215.
10. Greco F.A., Gray J., Burris H.A., Erland J.B., et al. Taxane-based chemotherapy for patients with carcinoma of unknown primary site. Cancer J 2001, 7(3):203-212.
11. Pape U.F., Böhmig M., Berndt U., Tiling N., et al. Survival and clinical outcome of patients with neuroendocrine tumors of the gastroenteropancreatic tract in a german referral center. Ann NY Acad Sci 2004, 1014:222-233.
12. Pavel M.E., Baum U., Hahn E.G., Hensen J. Doxorubicin and streptozotocin after failed biotherapy of neuroendocrine tumors. Int J Gastrointest Cancer 2005, 35(3):179-185.
13. Hainsworth J.D., Spigel D.R., Litchy S., Greco F.A. Phase II trial of paclitaxel, carboplatin, and etoposide in advanced poorly differentiated neuroendocrine carcinoma: a Minnie Pearl Cancer Research Network Study. J Clin Oncol 2006, 24(22):3548-3554.
14. Pimiento J.M., Teso D., Malkan A., Dudrick S.J., et al. Cancer of unknown primary origin: a decade of experience in a community-based hospital. Am J Surg 2007, 194(6):833-837.
15. Rothenstein J., Cleary S.P., Pond G.R., Dale D., et al. Neuroendocrine tumors of the gastrointestinal tract: a decade of experience at the Princess Margaret Hospital. Am J Clin Oncol 2008, 31(1):64-70.
16. Ochsenreither S., Marnitz-Schultze S., Schneider A., Koehler C., et al. Extrapulmonary small cell carcinoma (EPSCC): 10 years' multi-disciplinary experience at Charité. Anticancer Res 2009, 29(8):3411-3415.
17. Moertel C.G., Kvols L.K., O'Connell M.J., Rubin J. Treatment of neuroendocrine carcinomas with combined etoposide and cisplatin. Evidence of major therapeutic activity in the anaplastic variants of these neoplasms. Cancer 1991, 68(2):227-232.
18. Saltz L., Trochanowski B., Buckley M., Heffernan B., et al. Octreotide as an antineoplastic agent in the treatment of functional and nonfunctional neuroendocrine tumors. Cancer 1993, 72(1):244-248.
19. Lenzi R., Raber M.N., Frost P., Schmidt S., et al. Phase II study of cisplatin, 5-fluorouracil and folinic acid in patients with carcinoma of unknown primary origin. Eur J Cancer 1993, 29A(11):1634.
20. Andreyev H.J., Scott-Mackie P., Cunningham D., Nicolson V., et al. Phase II study of continuous infusion fluorouracil and interferon alfa-2b in the palliation of malignant neuroendocrine tumors. J Clin Oncol 1995, 13(6):1486-1492.
21. Hainsworth J.D., Erland J.B., Kalman L.A., Schreeder M.T., et al. Carcinoma of unknown primary site: treatment with 1-hour paclitaxel, carboplatin, and extended-schedule etoposide. J Clin Oncol 1997, 15(6):2385-2393.
22. Mitry E., Baudin E., Ducreux M., Sabourin J.C., et al. Treatment of poorly differentiated neuroendocrine tumours with etoposide and cisplatin. Br J Cancer 1999, 81(8):1351-1355.
23. Hainsworth J.D., Burris H.A., Meluch A.A., Baker M.N., et al. Paclitaxel, carboplatin, and long-term continuous infusion of 5-fluorouracil in the treatment of advanced squamous and other selected carcinomas: results of a Phase II trial. Cancer 2001, 92(3):642-649.
24. Lortholary A., Abadie-Lacourtoisie S., Guérin O., Mege M., et al. Cancers of unknown origin: 311 cases. Bull Cancer 2001 Jun, 88(6):619-627.
25. Bajetta E., Ferrari L., Procopio G., Catena L., et al. Efficacy of a chemotherapy combination for the treatment of metastatic neuroendocrine tumours. Ann Oncol 2002, 13(4):614-621.
26. Faiss S., Pape U.F., Böhmig M., Dörffel Y., et al. Prospective, randomized, multicenter trial on the antiproliferative effect of lanreotide, interferon alfa, and their combination for therapy of metastatic neuroendocrine gastroenteropancreatic tumors - the International Lanreotide and Interferon Alfa Study Group. J Clin Oncol 2003, 21(14):2689-2696.
27. Kulke M.H., Kim H., Clark J.W., Enzinger P.C., et al. A phase II trial of gemcitabine for metastatic neuroendocrine tumors. Cancer 2004, 101(5):934-939.
28. Gonzalez M.A., Biswas S., Clifton L., Corrie P.G. Treatment of neuroendocrine tumours with infusional 5-fluorouracil, folinic acid and streptozocin. Br J Cancer 2003, 89(3):455-456.
29. Boudreaux J.P., Putty B., Frey D.J., Woltering E., et al. Surgical treatment of advanced-stage carcinoid tumors: lessons learned. Ann Surg 2005, 241(6):839-845.
30. Sun W., Lipsitz S., Catalano P., Mailliard J.A., et al. Phase II/III study of doxorubicin with fluorouracil compared with streptozocin with fluorouracil or dacarbazine in the treatment of advanced carcinoid tumors: Eastern Cooperative Oncology Group Study E1281. J Clin Oncol 2005, 23(22):4897-4904.
31. Kulke M.H., Bergsland E.K., Ryan D.P., Enzinger P.C., et al. Phase II study of recombinant human endostatin in patients with advanced neuroendocrine tumors. J Clin Oncol 2006, 24(22):3555-3561.
32. Bajetta E., Catena L., Procopio G., De Dosso S., et al. Are capecitabine and oxaliplatin (XELOX) suitable treatments for progressing low-grade and high-grade neuroendocrine tumours?. Cancer Chemother Pharmacol 2007, 59(5):637-642.
33. Cicin I., Karagol H., Uzunoglu S., Uygun K., et al. Extrapulmonary small-cell carcinoma compared with small-cell lung carcinoma: a retrospective single-center study. Cancer 2007, 110(5):1068-1076.
34. Hainsworth J.D., Spigel D.R., Farley C., Thompson D.S., et al. Phase II trial of bevacizumab and erlotinib in carcinomas of unknown primary site: the Minnie Pearl Cancer Research Network. J Clin Oncol 2007, 25(13):1747-1752.
35. Faggiano A., Sabourin J.C., Ducreux M., Lumbroso J., et al. Pulmonary and extrapulmonary poorly differentiated large cell neuroendocrine carcinomas: diagnostic and prognostic features. Cancer 2007, 110(2):265-274.
36. Yao J.C., Zhang J.X., Rashid A., Yeung S.C., et al. Clinical and in vitro studies of imatinib in advanced carcinoid tumors. Clin Cancer Res 2007, 13(1):234-240.
37. Yao J.C., Phan A., Hoff P.M., Chen H.X., et al. Targeting vascular endothelial growth factor in advanced carcinoid tumor: a random assignment phase II study of depot octreotide with bevacizumab and pegylated interferon alpha-2b. J Clin Oncol 2008, 26(8):1316-1323.
38. Kulke M.H., Lenz H.J., Meropol N.J., Posey J., et al. Activity of sunitinib in patients with advanced neuroendocrine tumors. J Clin Oncol 2008, 26(20):3403-3410.
39. Cassier P.A., Walter T., Eymard B., Ardisson P., et al. Gemcitabine and oxaliplatin combination chemotherapy for metastatic well-differentiated neuroendocrine carcinomas: a single-center experience. Cancer 2009, 115(15):3392-3399.
40. Turner N.C., Strauss S.J., Sarker D., Gillmore R., et al. Chemotherapy with 5-fluorouracil, cisplatin and streptozocin for neuroendocrine tumours. Br J Cancer 2010, 102(7):1106-1112.
41. Wang S.C., Parekh J.R., Zuraek M.B., Venook A.P., et al. Identification of unknown primary tumors in patients with neuroendocrine liver metastases. Arch Surg 2010, 145(3):276-280.
42. Bertino E.M., Confer P.D., Colonna J.E., Ross P., et al. Pulmonary neuroendocrine/carcinoid tumors: a review article. Cancer 2009, 115(19):4434-4441.
43. Fink G., Krelbaum T., Yellin A., Bendayan D., et al. Pulmonary carcinoid: presentation, diagnosis, and outcome in 142 cases in Israel and review of 640 cases from the literature. Chest 2001, 119(6):1647-1651.
44. Cooper W.A., Thourani V.H., Gal A.A., Lee R.B., et al. The surgical spectrum of pulmonary neuroendocrine neoplasms. Chest 2001, 119(1):14-18.
45. Ducrocq X., Thomas P., Massard G., Barsotti P., et al. Operative risk and prognostic factors of typical bronchial carcinoid tumors. Ann Thorac Surg 1998, 65(5):1410-1414.
46. Skuladottir H., Hirsch F.R., Hansen H.H., Olsen J.H. Pulmonary neuroendocrine tumors: incidence and prognosis of histological subtypes. A population-based study in Denmark. Lung Cancer 2002, 37(2):127-135.
47. Modlin I.M., Sandor A. An analysis of 8305 cases of carcinoid tumors. Cancer 1997, 79(4):813-829.
48. Rea F., Rizzardi G., Zuin A., Marulli G., et al. Outcome and surgical strategy in bronchial carcinoid tumors: single institution experience with 252 patients. Eur J Cardiothorac Surg 2007, 31(2):186-191.
49. Huang Q., Muzitansky A., Mark E.J. Pulmonary neuroendocrine carcinomas. A review of 234 cases and a statistical analysis of 50 cases treated at one institution using a simple clinicopathologic classification. Arch Pathol Lab Med 2002, 126(5):545-553.
50. Travis W.D. Advances in neuroendocrine lung tumors. Ann Oncol 2010, 21(Suppl. 7):vii65-vii71.
51. Delaunoit T., Van den Eynde M., Borbath I., Demetter P., et al. Role of chemotherapy in gastro-entero-pancreatic neuroendocrine tumors: the end of a story?. Acta Gastroenterol Belg 2009, 72(1):49-53.
52. Hou Z., Elasmar S.A., Lozano R., et al. Apilot study of irinotecan plus cisplatin in patients with metastatic high-grade neuroendocrine carcinoma. J Clin Oncol 2003, 24:3548-3554.
53. Moertel C.G., Hanley J.A., Johnson L.A. Streptozocin alone compared with streptozocin plus fluorouracil in the treatment of advanced islet-cell carcinoma. N Engl J Med 1980, 303(21):1189-1194.
54. Moertel C.G., Lefkopoulo M., Lipsitz S., Hahn R.G., et al. Streptozocin-doxorubicin, streptozocin-fluorouracil or chlorozotocin in the treatment of advanced islet-cell carcinoma. N Engl J Med 1992, 326(8):519-523.
55. Delaunoit T., Ducreux M., Boige V., Dromain C., et al. The doxorubicin-streptozotocin combination for the treatment of advanced well-differentiated pancreatic endocrine carcinoma; a judicious option?. Eur J Cancer 2004, 40(4):515-520.
56. Cheng P.N., Saltz L.B. Failure to confirm major objective antitumor activity for streptozocin and doxorubicin in the treatment of patients with advanced islet cell carcinoma. Cancer 1999, 86(6):944-948.
57. Kouvaraki M.A., Ajani J.A., Hoff P., Wolff R., et al. Fluorouracil, doxorubicin, and streptozocin in the treatment of patients with locally advanced and metastatic pancreatic endocrine carcinomas. J Clin Oncol 2004, 22(23):4762-4771.
58. Rougier P., Oliveira J., Ducreux M., Theodore C., et al. Metastatic carcinoid and islet cell tumours of the pancreas: a phase II trial of the efficacy of combination chemotherapy with 5-fluorouracil, doxorubicin and cisplatin. Eur J Cancer 1991, 27(11):1380-1382.
59. Rivera E., Ajani J.A. Doxorubicin, streptozocin, and 5-fluorouracil chemotherapy for patients with metastatic islet-cell carcinoma. Am J Clin Oncol 1998, 21(1):36-38.
60. Broder L.E., Carter S.K. Pancreatic islet cell carcinoma. II. Results of therapy with streptozotocin in 52 patients. Ann Intern Med 1973, 79(1):108-118.
61. Moertel C.G., Lavin P.T., Hahn R.G. Phase II trial of doxorubicin therapy for advanced islet cell carcinoma. Cancer Treat Rep 1982, 66(7):1567-1569.
62. Ramanathan R.K., Cnaan A., Hahn R.G., Carbone P.P., et al. Phase II trial of dacarbazine (DTIC) in advanced pancreatic islet cell carcinoma. Study of the Eastern Cooperative Oncology Group-E6282. Ann Oncol 2001, 12(8):1139-1143.
63. Ansell S.M., Pitot H.C., Burch P.A., Kvols L.K., et al. A Phase II study of high-dose paclitaxel in patients with advanced neuroendocrine tumors. Cancer 2001, 91(8):1543-1548.
64. Moertel C.G., Hanley J.A. Combination chemotherapy trials in metastatic carcinoid tumor and the malignant carcinoid syndrome. Cancer Clin Trials 1979, 2(4):327-334.
65. Engstrom P.F., Lavin P.T., Moertel C.G., Folsch E., et al. Streptozocin plus fluorouracil versus doxorubicin therapy for metastatic carcinoid tumor. J Clin Oncol 1984, 2(11):1255-1259.
66. Oberg K., Norheim I., Lundqvist G., Wide L. Cytotoxic treatment in patients with malignant carcinoid tumors. Response to streptozocin - alone or in combination with 5-FU. Acta Oncol 1987, 26(6):429-432.
67. Bukowski R.M., Johnson K.G., Peterson R.F., Stephens R.L., et al. A phase II trial of combination chemotherapy in patients with metastatic carcinoid tumors. A Southwest Oncology Group Study. Cancer 1987, 60(12):2891-2895.
68. Foster N.R., Mandrekar S.J., Schild S.E., Nelson G.D., et al. Prognostic factors differ by tumor stage for small cell lung cancer: a pooled analysis of North Central Cancer Treatment Group trials. Cancer 2009, 115(12):2721-2731.
69. Maksymiuk A.W., Jett J.R., Earle J.D., et al. Sequencing and schedule effects of cisplatin plus etoposide in small-cell lung cancer: results of a North Central Cancer Treatment Group randomized clinical trial. J Clin Oncol 1994, 12(1):70-76.
70. Frytak S., Shaw E.G., Jett J.R., et al. Infusion cisplatin chemotherapy and hyperfractionated thoracic radiotherapy for small-cell lung cancer. Am J Clin Oncol 1996, 19(2):193-198.
71. Rowland K.M., Loprinzi C.L., Shaw E.G., et al. Randomized double-blind placebo-controlled trial of cisplatin and etoposide plus megestrol acetate/placebo in extensive-stage small-cell lung cancer: a North Central Cancer Treatment Group study. J Clin Oncol 1996, 14(1):135-141.
72. Kirschling R.J., Grill J.P., Marks R.S., et al. Paclitaxel and G-CSF in previously untreated patients with extensive stage small-cell lung cancer: a phase II study of the North Central Cancer Treatment Group. Am J Clin Oncol 1999, 22(5):517-522.
73. Marschke R.F., Grill J.P., Sloan J.A., et al. Phase II study of high-dose somatostatin analogue in patients either previously treated or untreated who have extensive-stage small cell lung cancer. Am J Clin Oncol 1999, 22(1):15-17.
74. Schaefer P.L., Marks R.S., Mahoney M.R., et al. Randomized phase II study of daily versus continuous-infusion schedules of topotecan in the treatment of extensive-stage small cell lung cancers. Am J Clin Oncol 2003, 26(3):236-240.
75. Perez E.A., Geoffroy F.J., Hillman S., et al. Phase II study of oral etoposide and intravenous paclitaxel in extensive-stage small cell lung cancer. Lung cancer 2004, 44(3):347-353.
76. Jett J.R., Hatfield A.K., Hillman S., et al. Alternating chemotherapy with etoposide plus cisplatin and topotecan plus paclitaxel in patients with untreated, extensive-stage small cell lung carcinoma: a phase II trial of the North Central Cancer Treatment Group. Cancer 2003, 97(10):2498-2503.
77. Molina J.R., Jett J.R., Foster N., et al. Phase II NCCTG trial of oral topotecan and paclitaxel with G-CSF (filgrastim) support in patients with previously untreated extensive-stage small cell lung cancer. Am J Clin Oncol 2006, 29(3):246-251.
78. Lee S.S., Lee J.L., Ryu M.H., Chang H.M., et al. Extrapulmonary small cell carcinoma: single center experience with 61 patients. Acta Oncol 2007, 46(6):846-851.
79. Kim J.H., Lee S.H., Park J., Kim H.Y., et al. Extrapulmonary small-cell carcinoma: a single-institution experience. Jpn J Clin Oncol 2004, 34(5):250-254.
80. Huang T.L., Huang C.H., Tang Y., Rau K.M., et al. Extrapulmonary small cell carcinoma - a medical center's experience. Chang Gung Med J 2006, 29(6):590-595.
81. Yuan Z.Y., Guan Z.Z., Zhou Z.M., Xia Y., et al. Extrapulmonary small cell carcinoma in 52 patients. Ai Zheng 2006, 25(9):1131-1133.
82. Walenkamp A.M., Sonke G.S., Sleijfer D.T. Clinical and therapeutic aspects of extrapulmonary small cell carcinoma. Cancer Treat Rev 2009, 35(3):228-236.
83. Cicin I., Usta U., Karagol H., Uzunoglu S., et al. Extrapulmonary small cell carcinoma localized in lymph nodes: is it a different clinical entity?. Acta Oncol 2009, 48(3):354-360.
84. Hess K.R., Abbruzzese M.C., Lenzi R., Raber M.N., et al. Classification and regression tree analysis of 1000 consecutive patients with unknown primary carcinoma. Clin Cancer Res 1999, 5(11):3403-3410.
85. Yao J.C., Hassan M., Phan A., Dagohoy C., et al. One hundred years after " carcinoid": epidemiology of and prognostic factors for neuroendocrine tumors in 35, 825 cases in the United States. J Clin Oncol 2008 Jun, 26(18):3063-3072.
86. Oberg K. Neuroendocrine gastrointestinal tumours. Ann Oncol 1996, 7(5):453-463.
87. Lobins R., Floyd J. Small cell carcinoma of unknown primary. Semin Oncol 2007, 34(1):39-42.