Rapid progression of primary hepatic neuroendocrine carcinoma: a case report demonstrating drastic oncological behavior

Primary hepatic neuroendocrine carcinoma is an extremely small proportion of all malignant tumors of this organ. Ac cording to published data, their proportion does not exceed 0.46 % among primary malignant liver neoplasms. Although well-differentiated primary hepatic neuroendocrine neoplasms are characterized by slow growth, poorly differentiated neuroendocrine carcinomas of the liver represent a distinct subgroup with inherently aggressive behavior. The diagnosis of such neoplasms is challenging due to the absence of specific imaging features and their similarity to hepatocellular carcinoma, cholangiocellular carcinoma, and metastatic liver lesions.

This article presents a case of primary neuroendocrine carcinoma of the liver (PNCL) in a 73-year-old patient. The comorbid background included chronic hepatitis B, type 2 diabetes mellitus, and liver cirrhosis. The tumor was incidentally detected during routine abdominal computed tomography. Over an 82-day observation period, rapid enlargement of the primary lesion from 42 to 95 mm was documented, along with progression of tumor thrombosis into the portal and hepatic veins and the development of distant lymphogenous metastases, including involvement of a cervical lymph node. Morphological verification was performed using biopsy material obtained from the cervical lymph node. The Ki-67 index exceeded 90 %, corresponding to the small-cell variant of poorly differentiated neuroendocrine carcinoma according to the 2019 WHO classification. The patient died on day 82 after the initial diagnosis before scheduled systemic chemotherapy could be administered.

The presented case demonstrates the potential for aggressive clinical behavior of PNCL even in the absence of features typical of hepatocellular carcinoma. Clinicians should consider PNCL in the differential diagnosis of rapidly progressive liver tumors and promptly pursue morphological verification of the diagnosis.

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