Abstract
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.
Publication types
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Case Reports
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Research Support, Non-U.S. Gov't
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Research Support, U.S. Gov't, P.H.S.
MeSH terms
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Bone Marrow / pathology
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Brain Neoplasms / etiology
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Brain Neoplasms / pathology
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Child
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Female
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Genes, p53
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Humans
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Leukemia, Myeloid, Acute / etiology*
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Leukemia, Myeloid, Acute / genetics
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Leukemia-Lymphoma, Adult T-Cell / etiology*
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Leukemia-Lymphoma, Adult T-Cell / genetics
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Medulloblastoma / etiology*
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Medulloblastoma / genetics
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Neoplasms, Multiple Primary / etiology*
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Neoplasms, Multiple Primary / genetics
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Neurofibromatosis 1 / complications*
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Neurofibromatosis 1 / genetics
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Wilms Tumor / etiology*
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Wilms Tumor / genetics