Primary neuroendocrine tumors of the gall bladder: Morphology with corroborative immunohistochemistry is the key to diagnosis

Abstract

Neoplasms with neuroendocrine differentiation comprising both neuroendocrine tumors (NETs) and neuroendocrine carcinomas (NECs) arising in gall bladder are extremely rare. NETs of the gall bladder represent only 0.2% of all NETs, whereas NECs comprise of 4% cases of all the malignant neoplasms of the gall bladder. NEC is the most aggressive variant of all gall bladder carcinomas. In most of the cases diagnosis is incidental and patient usually presents at advanced stage as there are no specific clinical or radiological features to detect NECs pre-operatively. Histopathology of the resected specimen and immunochemical analysis remain the main stay of diagnosing these malignancies. This case report includes 3 cases of NECs gall bladder comprising of two cases of small cell NECs and one case of large cell NEC. Although surgery followed by chemotherapy play important role in improving the quality of life of the patients, the prognosis of this combative disease can be upgraded by understanding the biological behavior of these tumours to adapt a definitive treatment protocol. The main aim of this study is to highlight on the need for better assessment, detection and management of this rare and aggressive entity to improve the survival rate of the patients of NECs.