Mixed Small Cell Carcinoma and Mucinous Adenocarcinoma of Appendix: A Rare Case Report with Review of Literature
Abstract
Gastrointestinal mixed neuroendocrine non-neuroendocrine neoplasms (MiNEN) are a heterogeneous group of tumors showing different morphological and prognostic features. The biological behavior of MiNEN is mostly driven by its high-grade component. Due to limitations in diagnostic methods and poor awareness, the incidence of MiNEN may be underestimated. The pathogenesis of MiNEN remains controversial. Molecular studies point towards a common monoclonal origin of the two components. Mixed tumors of the appendix are quite rare compared with those occurring elsewhere in the gastrointestinal tract. Although the appendix represents a common site of intestinal neuroendocrine tumors, primary extrapulmonary small cell carcinoma of the appendix is a rare entity. We describe a unique case of primary combined adenocarcinoma and small cell carcinoma of the appendix.References
Klimstra D, Klöppel G, La Rosa S, Rindi G. Classification of neuroendocrine neoplasms of the digestive system. In: WHO Classification of Tumours, 5th Edition, Digestive System Tumours. 2019. p. 16-9.
Rossi G, Bertolini F, Sartori G, Bigiani N, Cavazza A, Foroni M, et al. Primary mixed adenocarcinoma and small cell carcinoma of the appendix: a clinicopathologic, immunohistochemical, and molecular study of a hitherto unreported tumor. Am J Surg Pathol. 2004;28:1233-9.
Kim WS, Lee DG. Primary mixed adenocarcinoma and small-cell carcinoma of appendix: A case report (CARE-compliant). Medicine (Baltimore). 2019;98(19)
Frizziero M, Chakrabarty B, Nagy B, Lamarca A, Hubner RA, Valle JW, et al. Mixed Neuroendocrine Non-Neuroendocrine Neoplasms: A Systematic Review of a Controversial and Underestimated Diagnosis. J Clin Med. 2020;9(1):273.
Rindi G, Arnold R, Bosman FT. World Health Organization classification of tumours of the digestive system. 4th ed. Lyon: IARC Press; 2010. p. 13-4.
Chen ZZ, Huang W, Wei ZQ. Small-cell neuroendocrine carcinoma of the rectum - a rare tumor type with poor prognosis: A case report and review of literature. World J Clin Cases. 2020;8(23):6095-102.
O'Kane AM, O'Donnell ME, Shah R, Carey DP, Lee J. Small cell carcinoma of the appendix. World J Surg Oncol. 2008;6:4.
Capella C, La Rosa S, Uccella S, Billo P, Cornaggia M. Mixed endocrine-exocrine tumors of the gastrointestinal tract. Semin Diagn Pathol. 2000;17:91-103.
Vortmeyer AO, Lubensky IA, Merino MJ, Wang CY, Pham T, Furth EE, et al. Concordance of genetic alterations in poorly differentiated colorectal neuroendocrine carcinomas and associated adenocarcinomas. J Natl Cancer Inst. 1997;89(19):1448-53.
Furlan D, Cerutti R, Genasetti A, et al. Microallelotyping defines the monoclonal or the polyclonal origin of mixed and collision endocrine exocrine tumors of the gut. Lab Invest. 2003;83:963-71.
Kim KM, Kim MJ, Cho BK, Choi SW, Rhyu MG. Genetic evidence for the multi-step progression of mixed glandular-neuroendocrine gastric carcinomas. Virchows Arch. 2002;440:85-93.
La Rosa S, Marando A, Sessa F, Capella C. Mixed Adenoneuroendocrine Carcinomas (MANECs) of the Gastrointestinal Tract: An Update. Cancers (Basel). 2012;4(1):11-30.
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