Mahansaria Shyam Sunder, Agrawal Nikhil, Arora Asit, Bihari Chhagan, Appukuttan Murali, Chattopadhyay Tushar Kanti
1 Institute of Liver & Biliary Sciences, New Delhi, India.
Int J Surg Pathol. 2017 Oct;25(7):585-591. doi: 10.1177/1066896917712454. Epub 2017 May 29.
Mixed adenoneuroendocrine carcinoma (MANEC) has recently been defined by the World Health Organization in 2010. These are rare tumors and MANECs of ampullary region are even rarer. Only 19 cases have been reported in literature. We present 3 cases; the largest series, second case of amphicrine tumor and first case associated with chronic pancreatitis.
Retrospective review of 3 patients who were diagnosed to have ampullary MANEC.
All 3 patients were diagnosed preoperatively as neuroendocrine carcinoma and underwent margin negative pancreaticoduodenectomy. The histopathology revealed MANECs of small cell, mixed type in 2 patients and large cell, amphicrine type in 1 patient. The neuroendocrine component was grade 3 in all, the tumor was T3 in 2 and T2 in 1 and all had nodal metastases. Two patients received adjuvant chemotherapy and 2 of them had recurrence at 13 and 16 months. The median survival was 15 months.
Ampullary MANECs are rare tumors. They are diagnosed on histopathologic examination of the resected specimen. Clinical presentation, management, and prognosis is similar to ampullary adenocarcinoma in literature.
混合性腺神经内分泌癌（MANEC）最近在2010年被世界卫生组织定义。这些是罕见肿瘤，壶腹区域的MANEC更为罕见。文献中仅报道了19例。我们报告3例；这是最大的病例系列，第二例两性肿瘤，第一例与慢性胰腺炎相关。
对3例被诊断为壶腹MANEC的患者进行回顾性研究。
所有3例患者术前均被诊断为神经内分泌癌，并接受了切缘阴性的胰十二指肠切除术。组织病理学显示，2例为小细胞混合型MANEC，1例为大细胞两性型MANEC。所有患者的神经内分泌成分均为3级，2例肿瘤为T3期，1例为T2期，均有淋巴结转移。2例患者接受了辅助化疗，其中2例分别在13个月和16个月复发。中位生存期为15个月。
壶腹MANEC是罕见肿瘤。它们通过对切除标本的组织病理学检查来诊断。文献中其临床表现、治疗和预后与壶腹腺癌相似。
