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Wiskott-Aldrich syndrome and cerebral neoplasia: Report of a case with localized reticulum cell sarcoma

✍ Scribed by Kathleen P. Heidelberger; Dennis P. Legolvan


Publisher
John Wiley and Sons
Year
1974
Tongue
English
Weight
474 KB
Volume
33
Category
Article
ISSN
0008-543X

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✦ Synopsis


A case of Wiskott-Aldrich Syndrome with survival to nineteen-years old is reported. T h e patient developed reticulum cell sarcoma primary in the brain, which was biopsied and treated with irradiation and chemotherapy.

When the patient expired 4 '/z 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. I n only one other case was lymphoreticular neoplasm confined to the brain. I n no other cases is there necropsy-documented absence of neoplasm. The incidence of neoplasia occurring with this syndrome in this review is approximately lo%, and is predominantly lymphoreticular. Current diagnostic and therapeutic measures may make such localized neoplasms amenable to cure.