Neuroendocrine Tumor: A Rare, Aggressive Tumor of the Gallbladder

Abstract

We report a case of rare and aggressive gallbladder neuroendocrine carcinoma (GB-NEC), diagnosed with the help of endoscopic ultrasound (EUS). A 65-year-old asymptomatic male, with a past medical history of hypertension, underwent abdominal ultrasound for the screening of an abdominal aortic aneurysm. He was found to have a mixed echogenicity area near the stomach, an incidental finding on abdominal ultrasound. The patient had an upper gastrointestinal (GI) endoscopy exam, which revealed an antral mass that was biopsied. The tissue specimen showed an epithelioid mesenchymal tumor of unclassified type and, eventually, the patient underwent partial gastrectomy. Surgical pathology reported a low-grade sub-serosal gastrointestinal stromal tumor (GIST) of the resected tissue specimen. He was later discharged and advised to follow up with abdominal computed tomography (CT) every year. Two years later, his abdominal CT revealed a new 3.7 cm x 2.0 cm mass in the posterior gallbladder fundus. Subsequently, the patient underwent laparoscopic cholecystectomy and the excisional biopsy reported a T3NXM1 neuroendocrine small cell carcinoma. Then, he received six cycles of systemic chemotherapy with carboplatin and etoposide, showing excellent response initially. However, a repeat CT abdomen/pelvis with contrast, on his eighth-month follow-up, demonstrated the interval development of an infiltrative mass in the pancreatic head. The gastroenterology team was then consulted, who performed sphincterotomy with temporary stent placement and celiac plexus neurolysis. Also, a transduodenal fine-needle aspiration (FNA) of the pancreatic mass was performed, which revealed metastatic small cell carcinoma. Based on these findings, the patient received an additional three cycles of carboplatin/etoposide chemotherapy, along with one cycle of immunotherapy. However, the patient had a poor response to chemotherapy, and he eventually chose hospice care.

Neuroendocrine tumors of the gallbladder (Review)

“…Diagnostic comparisons indicate that immunohistochemical staining is the most effective tool for the diagnosis of GB-NENs. Immunohistochemistry is divided into two parts (32). First, neuroendocrine cell markers such as neuron-specific enolase (NSE), synaptophysin (Syn), pheochromin-A (CHG-A), protein gene product and Rankine are positively detected (Fig.…”

Age‐specific clinicopathological characteristics and prognostic analysis of neuroendocrine carcinomas of the gallbladder

“…Considering the subtypes, it is conventionally thought that most neuroendocrine neoplasms (NENs) of the gallbladder are NECs, and that NECs are predominantly small cell neuroendocrine carcinomas (SCNECs). [8][9][10][11] Some case reports and case series published recently reported that the proportions of SCNEC and large cell neuroendocrine carcinoma (LCNEC) of gallbladder are similar. 7,12 Compared with gallbladder ADCs, the general demographic features of gallbladder NECs such as age, sex, and BMI are similar.…”

“…At the time of diagnosis, most of them were at advanced stages. Considering the subtypes, it is conventionally thought that most neuroendocrine neoplasms (NENs) of the gallbladder are NECs, and that NECs are predominantly small cell neuroendocrine carcinomas (SCNECs) 8–11 . Some case reports and case series published recently reported that the proportions of SCNEC and large cell neuroendocrine carcinoma (LCNEC) of gallbladder are similar 7,12 …”

Clinicopathological characteristics and prognostic factors of patients with primary gallbladder neuroendocrine carcinomas

“…The exclusion criteria of the studies for literature review were as follows: (a) primary lesion arising from organs other than the gallbladder; (b) histological subtype of GB‐NET was not pathologically confirmed; (c) studies on GB‐NET or well‐differentiated MiNEN; (d) review articles, meta‐analyses, editorials, commentaries, or animal studies, etc; (e) the required clinicopathological data could not be extracted; and (f) insufficient data on the follow‐up or patient outcomes. After literature search data of additional 47 patients from 38 studies were obtained 5,7,9–44 . Finally, a total of 62 patients with GB‐NEC including poorly differentiated MiNEN were finally enrolled.…”