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Intrathoracic peripheral nerve sheath tumors in patients with neurofibromatosis type 1 (von Recklinghausen disease)

โœ Scribed by Vijay S. Patel; James D. St Louis; Olusola Oduntan; Kevin P. Landolfo

Book ID
113760642
Publisher
CTSNet
Year
2006
Tongue
English
Weight
112 KB
Volume
131
Category
Article
ISSN
1097-685X

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The two main peripheral nerve sheath tumors found in patients with neurofibromatosis, type 1 (NF1), are neurofibroma, a benign tumor, and malignant peripheral nerve sheath tumor (MPNST). The tumors are related in that most MPNSTs are thought to arise by malignant transformation of neurofibromas. Suc

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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