Primary hepatic neuroendocrine tumor: A rare neuroendocrine tumor

Abstract

Primary hepatic neuroendocrine tumors (PHNETs) account for only 0.3 % of all the primary neuroendocrine tumors(NETs). Since, liver is the most frequent site of metastasis of NETs from other sites in the body; a distinction between metastatic liver mass and primary NET is extremely necessary. This report describes the case of a 54-year-old male who presented with right hypochondrial pain and had a space occupying lesion in the left lobe of liver and multiple small lesions in both the lobes of liver. USG guided tru-cut biopsy showed malignant neoplasm of grade III comprising of sheets and nests of atypical plasmacytoid looking cells with brisk mitosis. Immunohistochemically, synaptophysin and cytokeratin were positive with Ki-67 index of 30 %. Hormonal studies were normal; hence, a diagnosis of non-functioning PHNET was made. Patient was treated stepwise with cisplatin/etoposide based chemotherapy, Everolimus, capecitabine and temozolomide; but, all the therapies failed to produce response and ultimately, patient was put on pembrolizumab as a palliative measure. This case illustrates that PHNET should be amongst the differentials of a liver mass and a comprehensive research is needed to devise successful management strategies.