Sequential development of Wilms tumor, T-cell acute lymphoblastic leukemia, medulloblastoma and myeloid leukemia in a child with type 1 neurofibromatosis: a clinical and cytogenetic case report.
In her 8 1/2 years of life, a girl with neurofibromatosis type 1 (NF1) developed four sequential primary malignant neoplasms: Wilms tumor, T-cell acute lymphoblastic leukemia, medulloblastoma and acute myeloid leukemia. The last three tumors were characterized by chromosomal abnormalities non-randomly associated with that particular disease. There was no evidence of germline p53 mutation or of mutation of p53 in the last two tumors. We hypothesize that an unusual mutation of the NF1 gene in this child promoted growth in tissues where the normal or mutated NF-1 gene product is usually silent or growth inhibitory.
Department of Pediatrics, Children's Hospital of Philadelphia, PA 19104.
G
G
Perilongo
C A
CA
Felix
A T
AT
Meadows
P
P
Nowell
J
J
Biegel
B J
BJ
Lange
D001853:Bone Marrow / Q000473:pathology; D001932:Brain Neoplasms / Q000209:etiology / Q000473:pathology; D002648:Child; D005260:Female; D016158:Genes, p53; D006801:Humans; D015470:Leukemia, Myeloid, Acute / Q000209:etiology* / Q000235:genetics; D015459:Leukemia-Lymphoma, Adult T-Cell / Q000209:etiology* / Q000235:genetics; D008527:Medulloblastoma / Q000209:etiology* / Q000235:genetics; D009378:Neoplasms, Multiple Primary / Q000209:etiology* / Q000235:genetics; D009456:Neurofibromatosis 1 / Q000150:complications* / Q000235:genetics; D009396:Wilms Tumor / Q000209:etiology* / Q000235:genetics