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

Abstract

BACKGROUND Mixed neuroendocrine-nonneuroendocrine neoplasm (MiNEN) is a rare tumor that occurs in the gastrointestinal tract and pancreas, usually composed of adenocarcinoma and neuroendocrine carcinoma. MiNEN occurring in ampulla is even rarer. We report 4 cases of MiNEN in ampulla, combined with literature review to summarize the clinical features and treatment of the disease, in order to improve the understanding of the disease. CASE SUMMARY A retrospective analysis was performed in 4 cases of MiNEN of the ampulla diagnosed by pathology from 2014 to 2021. The 4 patients were all male, aged 67-81 years (average 72.25 years). Among them, 2 patients had jaundice, 1 patient had abdominal pain, and 1 patient had jaundice with abdominal pain as the first symptom. All 4 patients underwent enhanced CT or MRI, which all indicated that the tumors were located in the ampulla. Two patients underwent duodenoscopy, and a biopsy revealed ampullary adenocarcinoma. All 4 patients underwent radical pancreaticoduodenectomy. Four cases were followed up: One patient developed severe complications after the operation, his condition deteriorated, and he survived for 1 mo. In the other 3 patients, tumor recurrence was observed during follow-up, and 2 of them survived for 29 mo and 22 mo respectively. One case survived and is still being followed up. CONCLUSION MiNEN of the ampulla are extremely rare, lacking typical clinical symptoms and imaging features, and are usually diagnosed after postoperative histopathological and immunohistochemical examinations. The main treatment is radical surgical resection, which can be combined with chemotherapy. The best method of diagnosis and treatment needs further research.

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

“…There were only two cases of SCNECs [ 25 , 27 ] and one case of LCNEC [ 26 ] reported. Like our case, at least three NEN cases [ 14 , 31 ] had elevated carbohydrate antigen 19-9 (CA19-9) levels, although none of them was a poorly differentiated NEC. This field was not answered in the case of the LCNEC.…”

“…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 ].…”

“…According to the 2019 WHO classification of tumors of the digestive system, NENs were classified into well-differentiated neuroendocrine tumors (NETs), poorly differentiated neuroendocrine carcinomas (NECs), and mixed neuroendocrine–non-neuroendocrine neoplasms (MiNENs). There have been only few case reports of MiNEN in the ampulla of Vater [ 11 , 12 , 13 , 14 ]. Well-differentiated and poorly differentiated NENs reflect two genetically and biologically different entities [ 15 ].…”

Mixed neuroendocrine–non-neuroendocrine neoplasm of the ampulla of Vater: a case report

“…Reaching a definitive preoperative diagnosis of Amp-MiNEN by examining tissue biopsies obtained by duodenoscopy is challenging, because the adenocarcinoma component is characteristically located on the surface. In contrast, the neuroendocrine component is more difficult to access, because it is located in deeper layers: this difficulty commonly resulting in diagnoses of adenocarcinoma [ 4 , 5 ]. In the present patient, we collected more specimens than usual, because the tumor seemed softer than a typical adenocarcinoma.…”

Molecular factors, diagnosis and management of gastrointestinal tract neuroendocrine tumors: An update

“…MiNENs of the ampulla are extremely rare. The main treatment is radical surgical resection followed by adjuvant chemotherapy[ 10 ]. Other locations include the esophagus and gastroesophageal junction, stomach, small intestine, appendix pancreas, liver, gallbladder and biliary tree.…”