Somatostatinoma Presented as Double-Duct Sign

Somatostatinoma is a rare neuroendocrine tumor with an incidence rate of 1 in 40 million people. It presents mostly as asymptomatic tumor diagnosed incidentally on imaging or surgery when evaluating or treating possible causes of abdominal pain. It also can present with vague symptoms, or as a clini...

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Main Authors: Ali Zakaria, Nour Hammad, Cynthia Vakhariya, Michael Raphael
Format: Article
Language:English
Published: Wiley 2019-01-01
Series:Case Reports in Gastrointestinal Medicine
Online Access:http://dx.doi.org/10.1155/2019/9506405
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author Ali Zakaria
Nour Hammad
Cynthia Vakhariya
Michael Raphael
author_facet Ali Zakaria
Nour Hammad
Cynthia Vakhariya
Michael Raphael
author_sort Ali Zakaria
collection DOAJ
description Somatostatinoma is a rare neuroendocrine tumor with an incidence rate of 1 in 40 million people. It presents mostly as asymptomatic tumor diagnosed incidentally on imaging or surgery when evaluating or treating possible causes of abdominal pain. It also can present with vague symptoms, or as a clinical triad of glucose intolerance, steatorrhea, and achlorhydria. The majority of somatostatinomas are present in the pancreatic head, followed by the duodenum, the pancreatic tail, and rarely the ampulla of Vater. The prognosis is poor as more than 77% of cases present as advanced disease with local invasion or distant metastasis. Surgical resection is the main treatment for early stage disease. Other treatment options include somatostatin analogue, molecular targeted therapy, and cytotoxic chemotherapy. The scarcity of somatostatinoma cases led to the lack of fully formulated treatment options. Herein, we present a 43-year old male patient who was referred by his primary care physician to our gastroenterology clinic due to elevated liver function test and double-duct sign on CT scan. We performed an ERCP, which revealed 2 cm ampullary lesion with upstream obstruction. Biopsies were taken and histopathology was unrevealing. He underwent a laparoscopic pancreaticoduodenectomy with histopathology revealed stage IIb somatostatinoma. Treating physicians should hold a high index of suspicion and maintain a broad differential diagnosis of elevated liver enzymes.
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spelling doaj-art-82d109dc6bc34afa867b255acc2bd0f62025-08-20T03:37:37ZengWileyCase Reports in Gastrointestinal Medicine2090-65282090-65362019-01-01201910.1155/2019/95064059506405Somatostatinoma Presented as Double-Duct SignAli Zakaria0Nour Hammad1Cynthia Vakhariya2Michael Raphael3Division of Gastroenterology, Providence-Providence Park Hospital and Medical Center, Michigan State University/College of Human Medicine, Southfield, Michigan, USADepartment of Internal Medicine, Providence-Providence Park Hospital and Medical Center, Michigan State University/College of Human Medicine, Southfield, Michigan, USADivision of Hematology & Oncology, Providence-Providence Park Hospital and Medical Center, Michigan State University/College of Human Medicine, Southfield, Michigan, USADivision of Gastroenterology, Providence-Providence Park Hospital and Medical Center, Michigan State University/College of Human Medicine, Southfield, Michigan, USASomatostatinoma is a rare neuroendocrine tumor with an incidence rate of 1 in 40 million people. It presents mostly as asymptomatic tumor diagnosed incidentally on imaging or surgery when evaluating or treating possible causes of abdominal pain. It also can present with vague symptoms, or as a clinical triad of glucose intolerance, steatorrhea, and achlorhydria. The majority of somatostatinomas are present in the pancreatic head, followed by the duodenum, the pancreatic tail, and rarely the ampulla of Vater. The prognosis is poor as more than 77% of cases present as advanced disease with local invasion or distant metastasis. Surgical resection is the main treatment for early stage disease. Other treatment options include somatostatin analogue, molecular targeted therapy, and cytotoxic chemotherapy. The scarcity of somatostatinoma cases led to the lack of fully formulated treatment options. Herein, we present a 43-year old male patient who was referred by his primary care physician to our gastroenterology clinic due to elevated liver function test and double-duct sign on CT scan. We performed an ERCP, which revealed 2 cm ampullary lesion with upstream obstruction. Biopsies were taken and histopathology was unrevealing. He underwent a laparoscopic pancreaticoduodenectomy with histopathology revealed stage IIb somatostatinoma. Treating physicians should hold a high index of suspicion and maintain a broad differential diagnosis of elevated liver enzymes.http://dx.doi.org/10.1155/2019/9506405
spellingShingle Ali Zakaria
Nour Hammad
Cynthia Vakhariya
Michael Raphael
Somatostatinoma Presented as Double-Duct Sign
Case Reports in Gastrointestinal Medicine
title Somatostatinoma Presented as Double-Duct Sign
title_full Somatostatinoma Presented as Double-Duct Sign
title_fullStr Somatostatinoma Presented as Double-Duct Sign
title_full_unstemmed Somatostatinoma Presented as Double-Duct Sign
title_short Somatostatinoma Presented as Double-Duct Sign
title_sort somatostatinoma presented as double duct sign
url http://dx.doi.org/10.1155/2019/9506405
work_keys_str_mv AT alizakaria somatostatinomapresentedasdoubleductsign
AT nourhammad somatostatinomapresentedasdoubleductsign
AT cynthiavakhariya somatostatinomapresentedasdoubleductsign
AT michaelraphael somatostatinomapresentedasdoubleductsign