Malignancies in pediatric patients with ataxia telangiectasia.
BACKGROUND
Patients with ataxia telangiectasia (AT), known to have an inherent increased susceptibility to the development of cancer, may present with malignancies that are unusual for the patient's age, are often difficult to diagnose clinically and radiographically and respond poorly to conventional therapy.


METHODS
We reviewed the clinical presentation and imaging studies of 12 AT patients who developed malignancies.


RESULTS
Eight of the twelve patients developed non-Hodgkin's lymphoma (CNS, thorax, bone), two developed Hodgkin's disease, and two were diagnosed with gastrointestinal mucinous adenocarcinoma.


CONCLUSIONS
The lymphomas were commonly extra nodal, and infiltrative rather than mass-like. The recognition of the tumors was often delayed due to confusion with the known infectious complications in AT patients.
Murphy
R C
RC
Babies & Children's Hospital of New York, Department of Radiology, Division of Pediatric Radiology, 3959 Broadway, BHN 3-318, New York, NY 10032, USA.
Berdon
W E
WE
Ruzal-Shapiro
C
C
Hall
E J
EJ
Kornecki
A
A
Daneman
A
A
Brunelle
F
F
Campbell
J B
JB
D000230:Adenocarcinoma / Q000150:complications / Q000000981:diagnostic imaging; D002288:Adenocarcinoma, Mucinous / Q000150:complications / Q000000981:diagnostic imaging; D000293:Adolescent; D000328:Adult; D001260:Ataxia Telangiectasia / Q000150:complications*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005770:Gastrointestinal Neoplasms / Q000150:complications / Q000000981:diagnostic imaging; D006801:Humans; D008223:Lymphoma / Q000150:complications / Q000000981:diagnostic imaging; D008297:Male; D009369:Neoplasms / Q000150:complications* / Q000000981:diagnostic imaging; D011859:Radiography; D012189:Retrospective Studies