Published online Aug 6, 2022. doi: 10.12998/wjcc.v10.i22.8045
Peer-review started: March 21, 2022
First decision: April 25, 2022
Revised: May 3, 2022
Accepted: June 22, 2022
Article in press: June 22, 2022
Published online: August 6, 2022
The ampulla of Vater is an anatomically and histologically complex region giving rise to a heterogenous group of tumors. This is, to the best of our knowledge, the first case of intra-ampullary papillary-tubular neoplasm combined with ampullary neuroendocrine carcinoma reported in the literature.
A 61-year-old woman presented to the emergency department for evaluation of painless jaundice. Contrast-enhanced computed tomography (CT) of the abdomen and chest showed a periampullary tumor mass measuring 15 mm × 12 mm × 14 mm, with no evidence of locoregional and distant metastases, for which she underwent pancreatoduodenectomy. Histopathologic examination of a resected specimen revealed an intra-ampullary papillary tubular neoplasm with high-grade dysplasia in combination with poorly differentiated grade 3 neuroendocrine carcinoma with a mitotic count of more than 20 mitoses per 10 high power fields and Ki-67 index of 100%. No positive lymph nodes were identified. Her postoperative course was uneventful. Postoperatively, she remained under close surveillance. Multiple liver metastases were observed on follow-up CT 8 mo after the surgery, so systemic therapy with cisplatin and etoposide was initiated.
The simultaneous occurrence of neuroendocrine and non-neuroendocrine tumors in the ampulla of Vater is rare and the pathogenesis of such tumors is largely unknown. Due to unpredictable clinical behavior and lack of solid evidence on optimal treatment strategy, close patient surveillance is advised after radical resection of the primary tumor.
Core Tip: The ampulla of Vater is a transitional region with various distinctive histomorphologic characteristics, although the simultaneous occurrence of neuroendocrine and non-neuroendocrine tumors in this region is rare. When present, problems arise in differentiation between mixed neuroendocrine–non-neuroendocrine neoplasm and the collision of two distinct tumors. Due to the rarity of such tumors, their clinical behavior remains largely unknown, as do appropriate treatment measures. After radical resection, if feasible, the standard of care for the most aggressive and/or predominant component of the tumor from the same site of origin may be adopted. Newly diagnosed cases should be discussed at multidisciplinary team meetings to tailor postoperative treatment and follow-up appropriately.