• World Neurosurg · Oct 2019

    Case Reports

    Malignant peripheral nerve sheath tumor arising within a lumbar spinal plexiform neurofibroma.

    • Sérgio Ferreira Alves Júnior, João Antônio Pessoa Corrêa, and Edson Marchiori.
    • Department of Radiology, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil.
    • World Neurosurg. 2019 Oct 1; 130: 264-266.

    AbstractA 41-year-old woman presented with a 16-year history of lumbar pain and right leg paresthesia. Twenty-four years previously, she had been diagnosed with neurofibromatosis type 1 syndrome. Magnetic resonance imaging showed a heterogeneous mass extending through widened lumbar neural foramina to the psoas muscles bilaterally, with a dumbbell-like appearance. Biopsy led to the diagnosis of a malignant peripheral nerve sheath tumor, an uncommon soft tissue sarcoma of neural origin that may occur in the context of neurofibromatosis type 1 syndrome. Given the aggressive behavior of these tumors, prompt and accurate diagnosis is critical to improve patient outcomes.Copyright © 2019 Elsevier Inc. All rights reserved.

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