Intraosseous malignant peripheral nerve sheath tumor in a patient with neurofibromatosis
β Scribed by D. G. Terry; Donald D. Sauser; Melissa D. Gordon
- Publisher
- Springer
- Year
- 1998
- Tongue
- English
- Weight
- 360 KB
- Volume
- 27
- Category
- Article
- ISSN
- 0364-2348
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Neurofibromatosis type 1 (NF1) patients have 10% of lifetime risk for developing malignant peripheral nerve sheath tumors (MPNST), one of the most aggressive cancers. We examined the spectrum of constitutional NF1 mutations among 24 NF1 patients with MPNST. We found mutations in 18 patients: four me
Malignant peripheral nerve sheath tumor (MPNST) is an uncommon sarcoma in the pediatric population; however, its presence should be considered in a child with an enlarging or painful soft-tissue mass. Diagnosis of this neoplasm depends on either the demonstration of its origin within a peripheral ne