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Pancreatic endocrine carcinoma

MedGen UID:
233070
•Concept ID:
C1328479
•
Neoplastic Process
Synonyms: Carcinoma, Islet Cell; Carcinomas, Islet Cell; Islet Cell Carcinoma; Islet Cell Carcinomas; Islet Cell Tumor, Malignant
SNOMED CT: Islet cell carcinoma (60346004); Islet cell adenocarcinoma (60346004); Malignant neoplasm of Islets of Langerhans (187794005); Malignant Islet cell tumor (187794005); Malignant tumor of Islets of Langerhans (187794005); Pancreatic endocrine tumor, malignant (60346004); Carcinoma of endocrine pancreas (254612002); Endocrine pancreatic carcinoma (254612002)
 
Monarch Initiative: MONDO:0005893
Orphanet: ORPHA506098

Definition

A rare neuroendocrine neoplasm of pancreas characterized by a high-grade malignant epithelial tumor with neuroendocrine differentiation. Based on histopathologic appearance, a small cell (composed of diffuse sheets of cells) and a large cell type (showing a nesting/trabecular pattern) are distinguished. Synaptophysin and chromogranin are positive on immunohistochemistry. The Ki-67 proliferation index is typically very high (>60 - 80%). Patients present with back pain, jaundice, and/or non-specific abdominal symptoms. Serum hormone activity is unusual. The tumor is highly aggressive with poor prognosis. [from ORDO]

Professional guidelines

PubMed

Long-term clinical outcome of somatostatin analogues for treatment of progressive, metastatic, well-differentiated entero-pancreatic endocrine carcinoma.
Panzuto F, Di Fonzo M, Iannicelli E, Sciuto R, Maini CL, Capurso G, Milione M, Cattaruzza MS, Falconi M, David V, Ziparo V, Pederzoli P, Bordi C, Delle Fave G
Ann Oncol 2006 Mar;17(3):461-6. Epub 2005 Dec 19 doi: 10.1093/annonc/mdj113. PMID: 16364959
Fluorouracil, doxorubicin, and streptozocin in the treatment of patients with locally advanced and metastatic pancreatic endocrine carcinomas.
Kouvaraki MA, Ajani JA, Hoff P, Wolff R, Evans DB, Lozano R, Yao JC
J Clin Oncol 2004 Dec 1;22(23):4762-71. doi: 10.1200/JCO.2004.04.024. PMID: 15570077
The doxorubicin-streptozotocin combination for the treatment of advanced well-differentiated pancreatic endocrine carcinoma; a judicious option?
Delaunoit T, Ducreux M, Boige V, Dromain C, Sabourin JC, Duvillard P, Schlumberger M, de Baere T, Rougier P, Ruffie P, Elias D, Lasser P, Baudin E
Eur J Cancer 2004 Mar;40(4):515-20. doi: 10.1016/j.ejca.2003.09.035. PMID: 14962717

Recent clinical studies

Etiology

Pancreatic cystic endocrine tumors: a different morphological entity associated with a less aggressive behavior.
Boninsegna L, Partelli S, D'Innocenzio MM, Capelli P, Scarpa A, Bassi C, Pederzoli P, Falconi M
Neuroendocrinology 2010;92(4):246-51. Epub 2010 Aug 5 doi: 10.1159/000318771. PMID: 20689265
Primary tumour resection in metastatic nonfunctioning pancreatic endocrine carcinomas.
Bettini R, Mantovani W, Boninsegna L, Crippa S, Capelli P, Bassi C, Scarpa A, Pederzoli P, Falconi M
Dig Liver Dis 2009 Jan;41(1):49-55. Epub 2008 May 7 doi: 10.1016/j.dld.2008.03.015. PMID: 18463008
Proteomic analysis of pancreatic endocrine tumor cell lines treated with the histone deacetylase inhibitor trichostatin A.
Cecconi D, Donadelli M, Rinalducci S, Zolla L, Scupoli MT, Scarpa A, Palmieri M, Righetti PG
Proteomics 2007 May;7(10):1644-53. doi: 10.1002/pmic.200600811. PMID: 17443844
Fluorouracil, doxorubicin, and streptozocin in the treatment of patients with locally advanced and metastatic pancreatic endocrine carcinomas.
Kouvaraki MA, Ajani JA, Hoff P, Wolff R, Evans DB, Lozano R, Yao JC
J Clin Oncol 2004 Dec 1;22(23):4762-71. doi: 10.1200/JCO.2004.04.024. PMID: 15570077
Testicular metastases from solid tumors: an autopsy study.
García-González R, Pinto J, Val-Bernal JF
Ann Diagn Pathol 2000 Apr;4(2):59-64. doi: 10.1016/s1092-9134(00)90012-1. PMID: 10760317

Diagnosis

A case of pancreatic endocrine carcinoma with a different clinical diagnosis before chemotherapy and pathological autopsy.
Jubashi A, Yamaguchi D, Ogata R, Nagatsuma G, Hara Y, Tanaka Y, Yoshioka W, Hino N, Morisaki T, Ario K, Matsuoka Y, Taba M, Naito S, Tsunada S
Clin J Gastroenterol 2022 Feb;15(1):237-243. Epub 2021 Oct 23 doi: 10.1007/s12328-021-01538-9. PMID: 34689312
Pancreatic cystic endocrine tumors: a different morphological entity associated with a less aggressive behavior.
Boninsegna L, Partelli S, D'Innocenzio MM, Capelli P, Scarpa A, Bassi C, Pederzoli P, Falconi M
Neuroendocrinology 2010;92(4):246-51. Epub 2010 Aug 5 doi: 10.1159/000318771. PMID: 20689265
Primary tumour resection in metastatic nonfunctioning pancreatic endocrine carcinomas.
Bettini R, Mantovani W, Boninsegna L, Crippa S, Capelli P, Bassi C, Scarpa A, Pederzoli P, Falconi M
Dig Liver Dis 2009 Jan;41(1):49-55. Epub 2008 May 7 doi: 10.1016/j.dld.2008.03.015. PMID: 18463008
Predictive factors of efficacy of the somatostatin analogue octreotide as first line therapy for advanced pancreatic endocrine carcinoma.
Butturini G, Bettini R, Missiaglia E, Mantovani W, Dalai I, Capelli P, Ferdeghini M, Pederzoli P, Scarpa A, Falconi M
Endocr Relat Cancer 2006 Dec;13(4):1213-21. doi: 10.1677/erc.1.01200. PMID: 17158766
Somatostatin-producing pancreatic endocrine carcinoma presented as relapsing cholangitis -- a case report.
Marakis G, Ballas K, Rafailidis S, Alatsakis M, Patsiaoura K, Sakadamis A
Pancreatology 2005;5(2-3):295-9. Epub 2005 Apr 21 doi: 10.1159/000085286. PMID: 15849491

Therapy

Phase II study of first-line FOLFIRI for progressive metastatic well-differentiated pancreatic endocrine carcinoma.
Brixi-Benmansour H, Jouve JL, Mitry E, Bonnetain F, Landi B, Hentic O, Bedenne L, Cadiot G
Dig Liver Dis 2011 Nov;43(11):912-6. Epub 2011 Aug 9 doi: 10.1016/j.dld.2011.07.001. PMID: 21831734
Multimodality management of a polyfunctional pancreatic endocrine carcinoma with markedly elevated serum vasoactive intestinal polypeptide and calcitonin levels.
Nasir A, Gardner NM, Strosberg J, Ahmad N, Choi J, Malafa MP, Coppola D, Kwekkeboom DJ, Teunissen JJ, Kvols LK
Pancreas 2008 Apr;36(3):309-13. doi: 10.1097/MPA.0b013e31815b321c. PMID: 18362846
Predictive factors of efficacy of the somatostatin analogue octreotide as first line therapy for advanced pancreatic endocrine carcinoma.
Butturini G, Bettini R, Missiaglia E, Mantovani W, Dalai I, Capelli P, Ferdeghini M, Pederzoli P, Scarpa A, Falconi M
Endocr Relat Cancer 2006 Dec;13(4):1213-21. doi: 10.1677/erc.1.01200. PMID: 17158766
Long-term clinical outcome of somatostatin analogues for treatment of progressive, metastatic, well-differentiated entero-pancreatic endocrine carcinoma.
Panzuto F, Di Fonzo M, Iannicelli E, Sciuto R, Maini CL, Capurso G, Milione M, Cattaruzza MS, Falconi M, David V, Ziparo V, Pederzoli P, Bordi C, Delle Fave G
Ann Oncol 2006 Mar;17(3):461-6. Epub 2005 Dec 19 doi: 10.1093/annonc/mdj113. PMID: 16364959
Fluorouracil, doxorubicin, and streptozocin in the treatment of patients with locally advanced and metastatic pancreatic endocrine carcinomas.
Kouvaraki MA, Ajani JA, Hoff P, Wolff R, Evans DB, Lozano R, Yao JC
J Clin Oncol 2004 Dec 1;22(23):4762-71. doi: 10.1200/JCO.2004.04.024. PMID: 15570077

Prognosis

Pancreatic cystic endocrine tumors: a different morphological entity associated with a less aggressive behavior.
Boninsegna L, Partelli S, D'Innocenzio MM, Capelli P, Scarpa A, Bassi C, Pederzoli P, Falconi M
Neuroendocrinology 2010;92(4):246-51. Epub 2010 Aug 5 doi: 10.1159/000318771. PMID: 20689265
Primary tumour resection in metastatic nonfunctioning pancreatic endocrine carcinomas.
Bettini R, Mantovani W, Boninsegna L, Crippa S, Capelli P, Bassi C, Scarpa A, Pederzoli P, Falconi M
Dig Liver Dis 2009 Jan;41(1):49-55. Epub 2008 May 7 doi: 10.1016/j.dld.2008.03.015. PMID: 18463008
Successful pancreatectomy with en-bloc resection of the celiac artery and portal vein for pancreatic endocrine carcinoma.
Akatsu T, Aiura K, Shimazu M, Ueda M, Wakabayashi G, Tanabe M, Kawachi S, Shinoda M, Kameyama K, Kitajima M, Kitagawa Y
Hepatogastroenterology 2007 Jun;54(76):1269-71. PMID: 17629086
Somatostatin-producing pancreatic endocrine carcinoma presented as relapsing cholangitis -- a case report.
Marakis G, Ballas K, Rafailidis S, Alatsakis M, Patsiaoura K, Sakadamis A
Pancreatology 2005;5(2-3):295-9. Epub 2005 Apr 21 doi: 10.1159/000085286. PMID: 15849491
Fluorouracil, doxorubicin, and streptozocin in the treatment of patients with locally advanced and metastatic pancreatic endocrine carcinomas.
Kouvaraki MA, Ajani JA, Hoff P, Wolff R, Evans DB, Lozano R, Yao JC
J Clin Oncol 2004 Dec 1;22(23):4762-71. doi: 10.1200/JCO.2004.04.024. PMID: 15570077

Clinical prediction guides

Primary tumour resection in metastatic nonfunctioning pancreatic endocrine carcinomas.
Bettini R, Mantovani W, Boninsegna L, Crippa S, Capelli P, Bassi C, Scarpa A, Pederzoli P, Falconi M
Dig Liver Dis 2009 Jan;41(1):49-55. Epub 2008 May 7 doi: 10.1016/j.dld.2008.03.015. PMID: 18463008
Long-term clinical outcome of somatostatin analogues for treatment of progressive, metastatic, well-differentiated entero-pancreatic endocrine carcinoma.
Panzuto F, Di Fonzo M, Iannicelli E, Sciuto R, Maini CL, Capurso G, Milione M, Cattaruzza MS, Falconi M, David V, Ziparo V, Pederzoli P, Bordi C, Delle Fave G
Ann Oncol 2006 Mar;17(3):461-6. Epub 2005 Dec 19 doi: 10.1093/annonc/mdj113. PMID: 16364959
Somatostatin-producing pancreatic endocrine carcinoma presented as relapsing cholangitis -- a case report.
Marakis G, Ballas K, Rafailidis S, Alatsakis M, Patsiaoura K, Sakadamis A
Pancreatology 2005;5(2-3):295-9. Epub 2005 Apr 21 doi: 10.1159/000085286. PMID: 15849491
Fluorouracil, doxorubicin, and streptozocin in the treatment of patients with locally advanced and metastatic pancreatic endocrine carcinomas.
Kouvaraki MA, Ajani JA, Hoff P, Wolff R, Evans DB, Lozano R, Yao JC
J Clin Oncol 2004 Dec 1;22(23):4762-71. doi: 10.1200/JCO.2004.04.024. PMID: 15570077
Parathyroid hormone-like peptide in pancreatic endocrine carcinoma and adenocarcinoma associated with hypercalcemia.
Miraliakbari BA, Asa SL, Boudreau SF
Hum Pathol 1992 Aug;23(8):884-7. doi: 10.1016/0046-8177(92)90399-n. PMID: 1322859

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