Small, low-grade ampullary neuroendocrine tumor presenting with metastasis and multiple synchronous tumors in a patient with neurofibromatosis type 1: a case report with literature review.

Authors

Kevin Xiao
Sharon Swierczynski, Department of Pathology, Reading Hospital-Tower Health, West Reading, PA, USA.Follow
Gary Xiao, Department of Surgery, Transplant and Hepato-Pancreato-Biliary Surgery, Reading Hospital-Tower Health, West Reading, PAFollow

Document Type

Article

Publication Date

3-2021

Abstract

Neurofibromatosis type 1 (NF1) is a tumor syndrome and one of the most common genetic disorders. Patients have an increased risk of developing neurologic and gastrointestinal (GI) neoplasms, but GI lesions are often underrecognized since most cases are asymptomatic. It is extremely rare to see multiple types of abdominal tumors synchronously in NF1. In this case, we describe a patient presenting with a small, low-grade periampullary neuroendocrine tumor (NET) that underwent endoscopic submucosal dissection and later pancreaticoduodenectomy (Whipple procedure). This led to findings of lymph node and distant metastasis of her NET, and the incidental discovery of gastrointestinal stromal tumors, extensive pancreatic intraepithelial neoplasia, and main duct and side branch intraductal pancreatic mucinous neoplasm. The synchronous presence of these lesions has not been reported in the literature.

Publication Title

J Surg Case Rep

Volume

2021

Issue

3

First Page

076

Last Page

076

Recommended Citation

Xiao, K., Swierczynski, S., & Xiao, G. (2021). Small, low-grade ampullary neuroendocrine tumor presenting with metastasis and multiple synchronous tumors in a patient with neurofibromatosis type 1: a case report with literature review.. J Surg Case Rep, 2021 (3), 076-076. https://doi.org/https://doi.org/10.1093/jscr/rjab076