Primary and recurrent embryonal sarcoma of the liver: clinicopathological and immunohistochemical analysis

Abstract

Aims: To evaluate primary and recurrent embryonal sarcoma of the liver and to improve recognition of its morphological variants and immunohistochemical features. Methods and results: Fourteen primary and two recurrent cases of hepatic embryonal sarcoma were evaluated histologically and investigated immunohistochemically with a panel of antibodies using the EnVision+ system. They were usually single, large, globular masses with solid and cystic gelatinous areas. Microscopic features included spindle, oval, stellate, epithelioid or multinucleated cells loosely or densely arranged in a myxomatous matrix. Entrapped bile ducts and hepatic cords were often present at the periphery of the tumours. Intracellular and extracellular periodic acid–Schiff‐positive, diastase‐resistant hyaline globules were commonly present. Recurrent tumours showed greater cellularity, anaplasia and pluripotential differentiation compared with the primary tumour. Immunohistochemistry showed evidence of widely divergent differentiation into mesenchymal and epithelial phenotypes. Conclusions: Embryonal sarcoma of the liver may undergo pluripotential differentiation and diagnosis should be based mainly on morphological features. Immunohistochemistry has no specific or diagnostic relevance, but, by using a panel of antibodies, may help to exclude other tumours.