Association of dysgerminoma and gaucher's disease.

A 22-year-old woman presented with splenomegaly and pancytopenia of over two years' duration. A splenectomy was performed because of a suspicion of Gaucher's disease. At the time of operation a left ovarian tumor was found and was removed. A diagnosis of Gaucher's disease was made on the basis of massive infiltration of histiocytes with typical cytoplasmic striations in the spleen, liver, and bone marrow. The ovarian tumor was characterized by a monotonous, diffuse proliferation of large, rounded cells and was diagnosed as dysgerminoma. The association of Gaucher's disease and malignant tumors is rare, only several cases have been reported in the English literature. An additional feature of this case was the appearance of histiocytes showing the characteristic cytoplasmic striations of Gaucher's cells in the stroma of the tumor.