Primary Biliary Mixed Adenoneuroendocrine Carcinoma (MANEC): A Short Review

Abstract

Mixed adenoneuroendocrine carcinomas (MANECs) are composite neoplasms with areas of adenocarcinoma or squamous cell carcinoma intermingled with neuroendocrine carcinoma or neuroendocrine tumor, each composing at least 30% of the neoplasm. MANECs are very infrequent overall, and they are more commonly diagnosed in the appendix, colon, and stomach. Biliary MANECs are particularly rare, and their histogenesis is debated because neuroendocrine cells are seldom identified in the normal biliary tract. They can show one of the 3 different architectural patterns described in Lewin's original classification: collision tumors, combined lesions, or amphicrine neoplasms. The neuroendocrine component is usually of a high grade, with small or large cell cytomorphology, whereas the adenocarcinoma component is either an intestinal or biliary type. Clinical presentation is characterized by locally advanced disease at the time of initial diagnosis. Recent studies suggest that treatment should be guided by the most aggressive histologic component.

Large Cell Neuroendocrine Carcinoma (LCNEC) of the Colon Arising from a Tubulovillous Adenoma: A Case Report and Literature Review

“…In 20 cases of colorectal AC with high-grade poorly-differentiated cell clusters, Gurzu et al reported that all AC expressed synaptophysin, but were negative for chromogranin (12). Further supporting this hypothesis, CD133 expression, a marker of cancer stem cells, has been found in varying amounts within different GI malignancies; including NET, NEC, AC, and in mixed adenoneuroendocrine carcinoma (MANEC) (13). MANEC tumors, recently characterized by the WHO in 2010, are composite neoplasms containing areas of adenocarcinoma or squamous cell carcinoma integrated with NEC or NET (with each component forming at least 30% of the neoplasm).…”

Carcinoma adenoneuroendocrino mixto de la ampolla de Vater: reporte de caso

“…Sin embargo, para pacientes que no puedan tolerar la escisión radical, la escisión local puede ser una opción válida. Posterior a esto se debe adoptar un tratamiento multimodal con radioterapia adyuvante y/o quimioterapia 7,8 .…”

Primary Mixed Adenoneuroendocrine Carcinoma (MANEC) of Gallbladder, Report of Two Cases with Different Histologic and Immunohistochemical Features

“…Mixed adenoneuroendocrine carcinoma (MANEC), has been described by WHO as a rare gastrointestinal neoplasm [1], histologically characterized by the presence of at least 30% each of epithelial and neuroendocrine components [2]. The tumor may appear in various levels of the digestive tract including the esophagus, stomach, colon and appendix [3][4][5][6][7], as well as other sites such as the pancreas [8], and urinary bladder [9][10][11][12].…”

“…The tumor may appear in various levels of the digestive tract including the esophagus, stomach, colon and appendix [3][4][5][6][7], as well as other sites such as the pancreas [8], and urinary bladder [9][10][11][12]. Presence of MANEC in the gallbladder is exceedingly rare [2,[13][14][15]. Biological behaviour of MANEC seems to be quite unpredictable and the prognosis uncertain.…”

“…Biological behaviour of MANEC seems to be quite unpredictable and the prognosis uncertain. Paucity of neuroendocrine cells in normal gallbladder has led to speculations regarding possible origins regarding MANEC [2]. The pathogenesis of this neoplasm may involve intestinal metaplasiadysplasia-carcinoma with synchronous or asynchronous progression to an epithelial and a neuroendocrine malignancy in the gallbladder.…”