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Rectal Large Cell Neuroendocrine Carcinoma

MedGen UID:
1783224
•Concept ID:
C5447384
•
Neoplastic Process

Definition

A rare, aggressive, high-grade and poorly differentiated carcinoma with neuroendocrine differentiation that arises from the rectum. It is characterized by the presence of malignant large cells. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVRectal Large Cell Neuroendocrine Carcinoma

Professional guidelines

PubMed

Survival According to Primary Tumor Location, Stage, and Treatment Patterns in Locoregional Gastroenteropancreatic High-grade Neuroendocrine Carcinomas.
Dasari A, Shen C, Devabhaktuni A, Nighot R, Sorbye H
Oncologist 2022 Apr 5;27(4):299-306. doi: 10.1093/oncolo/oyab039. PMID: 35380711Free PMC Article

Recent clinical studies

Etiology

Rare histologic types of rectal cancer: A monocentric cases series report.
Vallard A, Diao P, Garcia MA, Pigné G, Vial N, Forest F, Peoc'h M, Langrand-Escure J, Magné N
Cancer Radiother 2019 Feb;23(1):17-22. Epub 2018 Nov 30 doi: 10.1016/j.canrad.2018.03.009. PMID: 30509567
Identification of high-risk human papillomavirus and Rb/E2F pathway genomic alterations in mutually exclusive subsets of colorectal neuroendocrine carcinoma.
Shamir ER, Devine WP, Pekmezci M, Umetsu SE, Krings G, Federman S, Cho SJ, Saunders TA, Jen KY, Bergsland E, Jones K, Kim GE, Kakar S, Chiu CY, Joseph NM
Mod Pathol 2019 Feb;32(2):290-305. Epub 2018 Sep 20 doi: 10.1038/s41379-018-0131-6. PMID: 30237525
Histopathologic study of the rectum in 1,464 consecutive rectal specimens in a single Japanese hospital: II. malignant lesions.
Terada T
Int J Clin Exp Pathol 2013;6(3):385-94. Epub 2013 Feb 15 PMID: 23412998Free PMC Article
Neuroendocrine carcinomas arising in ulcerative colitis: coincidences or possible correlations?
Grassia R, Bodini P, Dizioli P, Staiano T, Iiritano E, Bianchi G, Buffoli F
World J Gastroenterol 2009 Sep 7;15(33):4193-5. doi: 10.3748/wjg.15.4193. PMID: 19725156Free PMC Article
Neuroendocrine carcinomas of the colon and rectum.
Bernick PE, Klimstra DS, Shia J, Minsky B, Saltz L, Shi W, Thaler H, Guillem J, Paty P, Cohen AM, Wong WD
Dis Colon Rectum 2004 Feb;47(2):163-9. doi: 10.1007/s10350-003-0038-1. PMID: 15043285

Diagnosis

Mixed high-grade serous and large cell neuroendocrine carcinoma arising from rectal endometriosis 11 years after hysterectomy.
Yanagita T, Hikichi T, Waragai Y, Shimizu H, Takahashi Y, Abe N, Hashimoto C, Kumakawa H, Kobayakawa M, Ohira H
Clin J Gastroenterol 2023 Jun;16(3):366-371. Epub 2023 Feb 10 doi: 10.1007/s12328-023-01769-y. PMID: 36763235
Mixed neuroendocrine-non-neuroendocrine neoplasm with squamous cell carcinoma covered by tubulovillous adenoma in the rectum.
Sato H, Shiota M, Urano M, Tsukamoto T, Honda K, Toyama K, Uyama I
Clin J Gastroenterol 2021 Aug;14(4):1136-1141. Epub 2021 Apr 27 doi: 10.1007/s12328-021-01420-8. PMID: 33905093
Synchronous duodenum and descending colon metastasis from primary lung neuroendocrine small-cell carcinoma: A case report and review of the literature.
Tanriverdi O, Alkan A, Ozseker B, Solak-Ozseker H, Kilinc RM
J Oncol Pharm Pract 2020 Sep;26(6):1524-1529. Epub 2020 Feb 17 doi: 10.1177/1078155220904133. PMID: 32063106
hGH and GHR expression in large cell neuroendocrine carcinoma of the colon and rectum.
Jukić Z, Limani R, Luci LG, Nikić V, Mijić A, Tomas D, Krušlin B
Anticancer Res 2012 Aug;32(8):3377-81. PMID: 22843918
Neuroendocrine tumors of the gastrointestinal tract.
Chejfec G, Falkmer S, Askensten U, Grimelius L, Gould VE
Pathol Res Pract 1988 Apr;183(2):143-54. doi: 10.1016/S0344-0338(88)80042-6. PMID: 3290866

Therapy

Long-term survivor of pulmonary combined large cell neuroendocrine carcinoma treated with nivolumab.
Oda R, Okuda K, Yamashita Y, Sakane T, Tatematsu T, Yokota K, Endo K, Nakanishi R
Thorac Cancer 2020 Jul;11(7):2036-2039. Epub 2020 May 7 doi: 10.1111/1759-7714.13471. PMID: 32379390Free PMC Article
Long-term control with chemoradiation of initially metastatic mixed adenoneuroendocrine carcinoma of the rectum: a case report.
Semrau S, Agaimy A, Pavel M, Lubgan D, Schmidt D, Cavallaro A, Golcher H, Grützmann R, Fietkau R
J Med Case Rep 2019 Mar 23;13(1):82. doi: 10.1186/s13256-019-1995-x. PMID: 30902067Free PMC Article
Neuroendocrine small cell rectal cancer metastasizing to the liver: a unique treatment strategy, case report, and review of the literature.
Al-Jiffry BO, Al-Malki O
World J Surg Oncol 2013 Jul 11;11:153. doi: 10.1186/1477-7819-11-153. PMID: 23844568Free PMC Article
Does small-cell phenotype predict the natural history of prostate cancer? A case study in disease behavior.
Slovin SF
Nat Clin Pract Oncol 2007 Sep;4(9):551-4. doi: 10.1038/ncponc0910. PMID: 17728713
The effectiveness of chemotherapy with cisplatin and 5-fluorouracil for recurrent small cell neuroendocrine carcinoma of the rectum: report of a case.
Okuyama T, Korenaga D, Tamura S, Yao T, Maekawa S, Watanabe A, Ikeda T, Sugimachi K
Surg Today 1999;29(2):165-9. doi: 10.1007/BF02482243. PMID: 10030743

Prognosis

Survival According to Primary Tumor Location, Stage, and Treatment Patterns in Locoregional Gastroenteropancreatic High-grade Neuroendocrine Carcinomas.
Dasari A, Shen C, Devabhaktuni A, Nighot R, Sorbye H
Oncologist 2022 Apr 5;27(4):299-306. doi: 10.1093/oncolo/oyab039. PMID: 35380711Free PMC Article
Long-term survivor of pulmonary combined large cell neuroendocrine carcinoma treated with nivolumab.
Oda R, Okuda K, Yamashita Y, Sakane T, Tatematsu T, Yokota K, Endo K, Nakanishi R
Thorac Cancer 2020 Jul;11(7):2036-2039. Epub 2020 May 7 doi: 10.1111/1759-7714.13471. PMID: 32379390Free PMC Article
Colorectal large-cell neuroendocrine carcinoma with lymphoid stroma: further evidence confirming a unique subtype associated with MLH1/PMS2 loss, BRAF mutation, Epstein-Barr virus negativity, and the possibility of a better prognosis.
Chetty R, Capo-Chichi JM, Serra S
Histopathology 2019 Aug;75(2):247-253. Epub 2019 Jul 2 doi: 10.1111/his.13875. PMID: 30990915
Endocrine tumors of the small and large intestine.
Solcia E, Fiocca R, Rindi G, Villani L, Luinetti O, Burrell M, Bosi F, Silini E
Pathol Res Pract 1995 May;191(4):366-72. doi: 10.1016/S0344-0338(11)80890-3. PMID: 7479353
Neuroendocrine tumors of the gastrointestinal tract.
Chejfec G, Falkmer S, Askensten U, Grimelius L, Gould VE
Pathol Res Pract 1988 Apr;183(2):143-54. doi: 10.1016/S0344-0338(88)80042-6. PMID: 3290866

Clinical prediction guides

Mixed high-grade serous and large cell neuroendocrine carcinoma arising from rectal endometriosis 11 years after hysterectomy.
Yanagita T, Hikichi T, Waragai Y, Shimizu H, Takahashi Y, Abe N, Hashimoto C, Kumakawa H, Kobayakawa M, Ohira H
Clin J Gastroenterol 2023 Jun;16(3):366-371. Epub 2023 Feb 10 doi: 10.1007/s12328-023-01769-y. PMID: 36763235
Malignant presacral ghrelinoma with long-standing hyperghrelinaemia.
Falkmer UG, Gustafsson T, Wenzel R, Wierup N, Sundler F, Kulkarni H, Baum RP, Falkmer SE
Ups J Med Sci 2015;120(4):299-304. Epub 2015 Jun 20 doi: 10.3109/03009734.2015.1054453. PMID: 26095011Free PMC Article
Histopathologic study of the rectum in 1,464 consecutive rectal specimens in a single Japanese hospital: II. malignant lesions.
Terada T
Int J Clin Exp Pathol 2013;6(3):385-94. Epub 2013 Feb 15 PMID: 23412998Free PMC Article
Gastric carcinosarcoma with neuroendocrine differentiation as the carcinoma component and leiomyosarcomatous and myofibroblastic differentiation as the sarcomatous component.
Kuroda N, Oonishi K, Iwamura S, Ohara M, Hirouchi T, Mizumo K, Miyazaki E, Enzan H
APMIS 2006 Mar;114(3):234-8. doi: 10.1111/j.1600-0463.2006.apm_328.x. PMID: 16643190
Neuroendocrine tumors of the gastrointestinal tract.
Chejfec G, Falkmer S, Askensten U, Grimelius L, Gould VE
Pathol Res Pract 1988 Apr;183(2):143-54. doi: 10.1016/S0344-0338(88)80042-6. PMID: 3290866

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