Mixed adenoneuroendocrine carcinoma of the ampulla: Two case reports

Abstract

Mixed adenoneuroendocrine carcinoma (MANEC) is a malignant tumor with adenocarcinoma and neuroendocrine components, with ≥ 30% of each component required. MANEC of the ampulla is rare. To the best of our knowledge, only 15 cases of MANEC of the ampulla have been reported in the English-language literature. Here, we report two cases of MANEC of the ampulla in two women aged 43 and 60 years, which was confirmed by histology after pancreaticoduodenectomy. These tumors contained neuroendocrine and adenocarcinoma components. The neuroendocrine components were positive for chromogranin A (CgA), synaptophysin (Syn) and CD56 by immunostaining. The adenocarcinoma components were negative for CgA, Syn and CD56. Both cases were T3N0M0 (Stage ⅢA). They survived for 15 and 20 mo after surgery, respectively. A brief discussion about the histopathological features, clinical behavior and treatment of MANEC of ampulla, and review of the relevant literature are presented.

A case of mixed adenoneuroendocrine carcinoma of the pancreas

“…[2–8] Such features indicate that different components may derive from a single cancer stem cell. MANECs can be divided into 3 subtypes such as composite, collision, and amphicrine tumors.…”

Cytological features of mixed adenoneuroendocrine carcinoma of the ampulla of Vater: A case report with immunocytochemical analyses

“…In this report, we described the second reported cytological case of MANEC with adenoma component of the ampulla of Vater, and analyzed the immunocytochemical characteristics of this type of tumor for the first time. To the best of our knowledge, only 16 cases of MANEC of the ampulla of Vater have been reported in the English language literature . Table summarizes the clinicopathological features of the previously reported cases as well as the present one.…”

“…Preoperative diagnosis of MANEC of the ampulla of Vater is difficult, because only adenocarcinoma component was detected in the preoperative examination in the majority of cases . Of note, only one case of the previously reported cases achieved preoperative diagnosis of MANEC, in which the cytological examination successfully detected both small cell carcinoma and signet ring cell carcinoma components .…”

“…Mixed adenoneuroendocrine carcinoma (MANEC) is defined as a tumor that has morphologically recognizable both adenocarcinoma and neuroendocrine carcinoma (NEC) components comprising at least 30% of either component by the recent WHO Classification . The occurrence of MANEC in the ampulla of Vater is extremely rare; only 16 cases have been reported in the English language literature . Moreover, only two cases of cytological features of this type of rare tumor have been documented in one report …”

Mixed adenoneuroendocrine carcinoma of the ampulla of Vater: A case report

“…Mixed adenoneuroendocrine carcinomas (MANECs) are rare biphasic tumour types, which have only anecdotally been reported in the ampullary region (7,(9)(10)(11)(12). The current report described a patient with ampullary MANEC, presenting with widespread metastatic dissemination.…”