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Abstract

A case of Wiskott-Aldrich Syndrome with survival to nineteen-years old is reported. The patient developed reticulum cell sarcoma primary in the brain, which was biopsied and treated with irradiation and chemotherapy. When the patient expired 4 1/2 months postoperatively, no tumor was present; death was the result of bronchopneumonia. Review of the literature revealed 14 prior cases of neoplasm in Wiskott-Aldrich Syndrome, 11 of them lymphoreticular. In only one other case was lymphoreticular neoplasm confined to the brain. In no other cases is there necropsy-documented absence of neoplasm. The incidence of neoplasia occurring with this syndrome in this review is approximately 10%, and is predominantly lymphoreticular. Current diagnostic and therapeutic measures may make such localized neoplasms amenable to cure.