Ligthelm, Louis J.Bunn, Belinda KathleenRaubenheimer, E.J. (Erich)Van Heerden, Willem Francois Petrus2018-05-242018-05Ligthelm LJ, Bunn BK, Raubenheimer EJ, Heerden WFP. Mixed adenoneur-oendocrine carcinoma of the tongue arising within a congenital enteric cyst. Head & Neck. 2018;40:E53–E57. https://doi.org/10.1002/hed.25117.1043-3074 (print)1097-0347 (online)10.1002/hed.25117http://hdl.handle.net/2263/65009BACKGROUND : Primary intestinal‐type adenocarcinoma of the tongue is rare. This represents the first reported case of a primary mixed adenoneuroendocrine carcinoma (MANEC) of the tongue arising within a congenital enteric cyst. METHODS : A 52‐year‐old man presented with a midline tongue mass that was connected to the mucosal surface with an epithelial‐lined sinus tract. orphological examination and immunohistochemical profiling of the neoplasm were performed. RESULTS : Histopathological evaluation showed a primary MANEC arising within a preexisting congenital enteric cyst, comprising both adenocarcinoma and neuroendocrine components. The adenocarcinoma had a colonic‐type morphology and coexpressed CK7, CK20, and CDX2. Imaging and colonoscopy excluded a distant primary colorectal neoplasm. CONCLUSION : The association of primary MANEC of the tongue with a gastrointestinal heterotopic cyst supports an origin from entrapped endodermal elements as opposed to salivary duct origin. This case raises the awareness of a rare yet prognostically important complication of a gastrointestinal heterotopic cyst.en© 2018 Wiley Periodicals, Inc. This is the pre-peer reviewed version of the following article : Mixed adenoneur-oendocrine carcinoma of the tongue arising within a congenital enteric cyst, Head and Neck, vol. 40, pp. E53–E57, 2018, doi : 10.1002/hed.25117. The definite version is available at : https://http://wileyonlinelibrary.com/journal/hed.Mixed adenoneuroendocrine carcinoma (MANEC)TongueCongenital enteric cystHistogenesisRareTractEntityGastrointestinal heterotopic cystColonic‐type adenocarcinomaPrimary tongue adenocarcinomaMixed adenoneuroendocrine carcinoma of the tongue arising within a congenital enteric cystPostprint Article