Small-Cell Type, Poorly Differentiated Neuroendocrine Carcinoma of the Gallbladder: A Case Report and Review of the Literature

Abstract

The poorly differentiated small-cell type, neuroendocrine carcinoma (NEC) of the gallbladder is a very uncommon subtype of a neuroendocrine tumor of the gastrointestinal tract. Nonsecretory NEC by virtue of its nonspecific and subtle clinical presentation of the tumor is usually diagnosed at an advance stage with presenting symptoms related to either locally advance disease or from metastatic disease. Though the radiologic imaging does identify the gall bladder cancer, the tumor lacks a specific diagnostic test; therefore, the diagnosis is almost always confirmed on histopathologic and immunohistochemical staining. We present a case of a poorly differentiated, small-cell neuroendocrine tumor of the gallbladder. The patient died within 3 months after the definitive diagnosis was made. Survival from this deadly malignancy can be improved with aggressive surgical treatment followed by chemotherapy and radiotherapy on a case-by-case scenario. The systemic chemotherapy remained the treatment of choice for an unresectable tumor (Chen et al., 2014).

A metastatic neuroendocrine small cell carcinoma of the gall bladder: case report and a comprehensive review of the literatures

“…The various chemotherapy regimens used include platinum-based agentscisplatin, carboplatin with or without etoposide, and 5-FU (4,8,10). Despite any modality of treatment, prognosis remains poor with overall survival ranging from 2.5-31.5 months (5,11). This case is reported for its rarity and to convey the importance of detailed pathological examination of tissue even in cases which may clinically and radiologically look very obvious.…”

“…Clinical presentation varies among patients and includes vague upper abdominal pain, jaundice, weight loss, bleeding, and an incidental finding of a large tumour mass indistinguishable from other tumours arising from GB (1,4). Functionally they can be no secretory, secretory, and metastatic (5). These are usually large tumour with extensive necrosis, have diffuse growth pattern that mostly involves the submucosa.…”

Outcomes of metastatic neuroendocrine carcinoma of the gallbladder

“…It is usually classified into four major categories based on tumour grade and mitotic index: well-differentiated neuroendocrine tumour (typical carcinoid), well-differentiated NEC (atypical carcinoid), poorly differentiated NEC (small or large cell) types and mixed exocrine-endocrine carcinomas. Sometimes there can be both the components of adenocarcinoma and neuroendocrine carcinoma (MANEC) [9]. Here in this study, we have analysed only the poorly differentiated NEC (small or large cell type).…”

“…The median age of presentation is usually in the range of 60-70 years [2,7,9]. Female individuals are more commonly affected than males, which is 80% of this population.…”

Gallbladder Cancer: Imaging Appearance and Pitfalls in Diagnosis

“…13 Neuroendocrine tumors. 14,15 small-cell tumors, 16 lymphoma, 17 and metastases 18 have also been reported.…”