Small Cell Neuroendocrine Carcinoma of Liver – A Rare Entity

Background: Small cell neuroendocrine carcinoma (SCNEC) of the liver is an exceptionally rare and aggressive malignancy. It is classified as a high-grade poorly differentiated neuroendocrine tumor, characterized by rapid disease progression, late-stage diagnosis, and poor prognosis. Due to its rarity, SCNEC presents significant diagnostic and therapeutic challenges, distinguishing it from more common hepatic tumors, such as hepatocellular carcinoma (HCC), and metastatic neuroendocrine tumors. Case Presentation: We present the case of a 47-year-old postmenopausal woman who presented with right-sided abdominal pain, progressive swelling, significant weight loss, and anorexia. Imaging studies revealed a large hepatic mass with evidence of metastatic disease. Histopathological examination and immunohistochemistry confirmed the diagnosis of primary SCNEC of the liver, stage cT3 cN1 cM1. The patient was treated with first-line platinum-based chemotherapy (cisplatin and etoposide), along with supportive care. Discussion: SCNEC of the liver exhibits distinct pathophysiological features, including high mitotic activity and genetic abnormalities such as TP53 and RB1 mutations. Accurate diagnosis relies on histopathology and immunohistochemical markers like synaptophysin and chromogranin A. Management involves a multidisciplinary approach, with systemic chemotherapy as the cornerstone of treatment. Surgical resection and liver-directed therapies may be feasible in select cases. Emerging therapies, such as immune checkpoint inhibitors and targeted treatments, offer potential but require further clinical validation. Conclusion: SCNEC of the liver is a rare and aggressive malignancy with limited therapeutic options and poor prognosis. Early detection, molecular profiling, and personalized treatment strategies are critical for improving outcomes. This case contributes to the limited literature, emphasizing the importance of a collaborative approach and the need for further research to optimize management and survival.