Neuroendocrine carcinoma of the liver associated with dermatomyositis: autopsy case and review of the literature

Abstract

Reported herein is an autopsy case of primary hepatic neuroendocrine carcinoma associated with dermatomyositis. A 71-year-old Japanese man, who was diagnosed with dermatomyositis 5 months before death, had multiple tumors within a non-cirrhotic liver. Histopathologically, the tumors were composed of small- and medium-sized round cells with clear cytoplasm arranged in nests, sheets or rosettes. Immunohistochemically, the tumor cells were positive for chromogranin A, neuron-specific enolase and CD56 and were negative for synaptophysin. This tumor was diagnosed as a primary hepatic neuroendocrine carcinoma with metastasis to the lung, gallbladder and lymph nodes around the pancreas and aorta; no primary lesions were detected in any other organ. The tumor cells were also positive for cytokeratin 7, cytokeratin 19 and epithelial membrane antigen but were negative for anti-hepatocyte antibody and AFP. These findings suggest that the tumor originated in intrahepatic bile duct epithelium. Various cancers have been reported in patients with dermatomyositis, but only seven cases of dermatomyositis associated with primary liver cancer have been reported. To the best of the authors' knowledge, this is the first report of dermatomyositis associated with primary hepatic neuroendocrine carcinoma.