Primary hepatic neuroendocrine carcinoma is an extremely rare liver tumor. We herein report a case of primary hepatic neuroendocrine carcinoma coexisting with a hemangioma in a 66-year-old man. Ultrasonography, computed tomography, and magnetic resonance imaging showed a tumor (1.5 cm in diameter) coexisting with a hemangioma in the lateral segment of the liver. Liver biopsy showed malignant cells, and several examinations revealed no alternative primary source. We performed a lateral segmentectomy. Microscopically, the tumor cells had round to oval nuclei and eosinophilic cytoplasm, proliferated in thick trabeculae or solid nests, and formed a focal rosette pattern. Mitotic cells were frequently observed. Immunohistochemically, the tumor cells were positive for the endocrine markers chromogranin A, neuron-specific enolase, and neural cell adhesion molecule, but negative for α-fetoprotein and hepatocyte-specific antigen. The patient is still alive after 3 months, without recurrence.
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