Mixed adenoneuroendocrine carcinoma (MANEC) of the ampulla of Vater in a Chinese patient: A case report

Abstract

Mixed adenoneuroendocrine carcinoma (MANEC) is a rare tumour of the gastrointestinal tract with both epithelial and neuroendocrine components. We describe a case of a 57-year-old man who presented with yellow sclera and dark urine. Contrast-enhanced computed tomography (CT) showed enlargement of the intra and extrahepatic bile ducts and a neoplastic lesion at the end of the common bile duct which was indistinguishable from the adjacent head of the pancreas and duodenum. A pancreaticoduodenectomy was performed and histopathological examination of resected samples showed that tumour was a complex lesion with adenocarcinoma and neuroendocrine components positive for neuroendocrine markers (chromogranin A and synaptophysin) with a Ki-67 labelling index of 40%. The patient was diagnosed with MANEC in the ampulla of Vater with a neuroendocrine carcinoma component of approximately 70%. Ampullary MANECs are highly aggressive tumours with a high risk for distant metastases and a poor prognosis. Therefore, establishing a standard therapeutic strategy is crucial.

Mixed neuroendocrine-nonneuroendocrine neoplasm of the ampulla: Four case reports

“…MiNEN is a rare tumor commonly found in the appendix and colorectum in the digestive tract, while MiNENs that occur in the ampulla are very rare. The literature search found only 16 cases,and most of them were case reports[ 1 , 2 , 5 ]. Patients with a MiNEN of the ampulla usually present with nonspecific clinical symptoms, such as abdominal pain, jaundice, nausea, vomiting, and upper abdominal discomfort[ 6 ].…”

“…At present, the best treatment for MiNEN is not clear[ 5 ]. It has been reported that for MiNEN without distant metastases, radical surgery can be the first choice for treatment, and palliative chemotherapy can be selected when the patient has distant metastases[ 10 ].…”

“…Among the reported cases of MiNEN in the ampulla, most cases undergo surgical radical resection (pancreaticoduodenectomy). Postoperative chemotherapy regimens are different, such as oxaliplatin-based combination chemotherapy and S-1, and chemotherapy for NEC components is usually recommended[ 2 , 5 ].Among the 3 patients undergoing chemotherapy in this study, the regimen was different from the previously reported regimen. After the tumor recurrence was observed in these patients, they were treated with chemotherapeutic drugs, and the reduction of metastases was observed, suggesting that it may be effective.…”

Ampullary Large-Cell Neuroendocrine Carcinoma, a Diagnostic Challenge of a Rare Aggressive Neoplasm: A Case Report and Literature Review

“…We found 19 cases of NENs reported in the literature in a two-year time period, from May 2020 until April 2022 ( Table 1 ) [ 9 , 14 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 ].…”

Mixed neuroendocrine nonneuroendocrine neoplasms of the pancreas: a case report and literature review of pancreatic mixed neuroendocrine nonneuroendocrine neoplasm

“…HE-stained sections served for the morphological identification of both neuroendocrine and nonneuroendocrine components. Immunohistochemistry is mandatory to test the nature of the neuroendocrine component, which is also recommended to evaluate the subtype of the nonneuroendocrine (11)(12)(13)15,16,19,20,22,23,25). Preoperative diagnosis was rarely established, and only a few cases reported had been diagnosed through preoperative biopsy with endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) specimens (17)(18)(19)21,24).…”