an unusual family cancer syndrome manifested in young siblings.

Abstract

Three siblings whose mother had an ovarian mass excised as a teenager all developed malignancies in late childhood. Two were diagnosed as having malignant germ cell tumors of the ovary, whereas the third was found to have a soft tissue sarcoma. No underlying familial disease or constitutional chromosomal aberration has been detected and no known carcinogenic chemical exposure has been identified. All three children have been successfully treated with chemotherapy and are doing well off treatment. This is the first family cancer syndrome reported to have an association of ovarian germ cell tumors with embryonal sarcoma.