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Wiskott‐Aldrich syndrome and cerebral neoplasia: Report of a case with localized reticulum cell sarcoma
Author(s) -
Heidelberger Kathleen P.,
Legolvan Dennis P.
Publication year - 1974
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/1097-0142(197401)33:1<280::aid-cncr2820330141>3.0.co;2-h
Subject(s) - reticulum cell sarcoma , medicine , neoplasm , sarcoma , pathology , wiskott–aldrich syndrome , gene , biochemistry , chemistry
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.