North Texas GME Research Forum 2023



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North Texas


Medical City Arlington


Internal Medicine

Document Type


Publication Date



Neurofibromatosis 1, nerve sheath neoplasms


Congenital, Hereditary, and Neonatal Diseases and Abnormalities | Internal Medicine | Neoplasms | Nervous System Diseases


Malignant peripheral nerve sheath tumors (MPNSTs) are rare sarcomas, most commonly seen in patients with Neurofibromatosis type 1 (NF1), that are characterized as aggressive with high rate of local recurrence. Among NF1 patients, the risk of developing MNPSTs is approximately 8-13% over a lifetime. Primary MPNST is exceedingly rare, of which the vast majority are concentrated in the head and neck region. Here, we present a case of a 40-year-old male with NF1 who presented with a giant MPNST that originated in the right proximal femur. The mass was treated with complete surgical resection with right hip disarticulation. In a matter of 4 months, the patient had a rapid progression with metastatic disease to the brain and lungs. We will use this case as an opportunity to explore the current guidelines for post-surgical malignant sarcomas especially in those with neurofibromatosis and the need for more aggressive and active adjuvant treatments.

Original Publisher

HCA Healthcare Graduate Medical Education

Rare Presentation of Primary Malignant Peripheral Nerve Sheath Tumor of the Femur in Neurofibromatosis-1



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