Mixed adenoneuroendocrine carcinoma of the non-ampullary duodenum with mismatch repair deficiency: a rare case report

A non-ampullary duodenal mixed adenoneuroendocrine carcinoma (MANEC), consisting of a conventional adenocarcinoma and a neuroendocrine carcinoma (NEC), is exceedingly rare. Moreover, mismatch repair (MMR) deficient tumors have recently attracted attention. The patient, a 75-year-old woman with epiga...

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Published in:Medical molecular morphology 2022-09, Vol.55 (3), p.258-266
Main Authors: Nozawa, Yumi, Ishida, Kazuyuki, Maiko, Niki, Takada-Owada, Atsuko, Onozaki, Masato, Takaoka, Mina, Matsuyama, Kinichi, Sakuraoka, Yuhki, Nakazato, Yoshimasa, Kubota, Keiichi
Format: Article
Language:English
Subjects:
Adenocarcinoma
Anatomy
Biopsy
Cancer
Case Report
Case reports
Duodenum
Medicine
Medicine & Public Health
Mismatch repair
MLH1 protein
Molecular Medicine
Nausea
Neuroendocrine tumors
Pancreaticoduodenectomy
Pathology
Retina
Retinoblastoma
Small intestine
Synaptophysin
Tumor cells
Tumorigenesis
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Summary:A non-ampullary duodenal mixed adenoneuroendocrine carcinoma (MANEC), consisting of a conventional adenocarcinoma and a neuroendocrine carcinoma (NEC), is exceedingly rare. Moreover, mismatch repair (MMR) deficient tumors have recently attracted attention. The patient, a 75-year-old woman with epigastric pain and nausea, was found to have a type 2 tumor of the duodenum, which was diagnosed on biopsy as a poorly differentiated carcinoma. A pancreaticoduodenectomy specimen showed a well-defined 50 × 48 mm tumor in the duodenal bulb, which was morphologically composed of glandular, sheet-like, and pleomorphic components. The glandular component was a tubular adenocarcinoma, showing a MUC5AC-positive gastric type. The sheet-like component consisted of homogenous tumor cells, with chromogranin A and synaptophysin diffusely positive, and a Ki-67 index of 72.8%. The pleomorphic component was diverse and prominent atypical tumor cells proliferated, focally positive for chromogranin A, diffusely positive for synaptophysin, and the Ki-67 index was 67.1%. The sheet-like and pleomorphic components were considered NEC, showing aberrant expression of p53, retinoblastoma, and p16. Notably, all three components were deficient in MLH1 and PMS2. We diagnosed a non-ampullary duodenal MANEC with MMR deficiency. This tumor has a unique morphology and immunohistochemical profile, and is valuable for clarifying the tumorigenesis mechanism of a non-ampullary duodenal MANEC.
ISSN:1860-1480
1860-1499
DOI:10.1007/s00795-022-00324-x