Louis J Ligthelm1, Belinda K Bunn2, Erich J Raubenheimer1,2, Willie F P van Heerden2. 1. Ampath Laboratories, Drs Du Buisson, Kramer, Swart, Bouwer Inc, South Africa. 2. Department of Oral Pathology and Oral Biology, School of Dentistry, Faculty of Health Sciences, University of Pretoria, South Africa.
Abstract
BACKGROUND: 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. Morphological 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.
BACKGROUND: 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. Morphological 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.