The occurrence of soft tissue sarcomas in three siblings with Werner's syndrome

Abstract

Werner's syndrome, a relatively rare and autosomal recessive disorder, is well known to be characterized by a high frequency of malignant neoplasm. The occurrence of familial neoplasm in patients with this condition, however, has been recorded only once before in the literature. Reported are the findings with regard to the occurrence of sarcomas in three siblings with Werner's syndrome. Two of the current three cases were of malignant fibrous histiocytomas, one in a 36-year-old man and one in a 32-year-old woman. The other case was of a leiomyosarcoma in a 26-year-old man. Two of the patients died of the tumors, although the third is still alive. The exact cause of the high incidence of malignant tumor in this family remains unknown, as is still so in other cases of Werner's syndrome.