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Washington University Experience | NEOPLASMS - CRANIAL AND PARASPINAL NERVEs | Malignant Peripheral Nerve Sheath Tumor (MPNST) | 18A1 MPNST, plex NF in NF1, epitheloid (Case 18) - Copy

Case 18 History ---- The patient is a 50-year-old man, with personal and family history of neurofibromatosis type I, who has had increasing difficulty with initiating micturition and maintaining bowel control. He also developed a sore in the gluteal cleft and has noticed that his entire buttocks have become insensate. There are numerous subcutaneous, deep, and nerve-root associated neurofibromas, MRI and PET imaging studies identified an FDG avid soft tissue mass that fills the central canal from L5 to S4, invades and remodels the S2 and S3 vertebral bodies, has destroyed the posterior elements from L5 to S2, and extends into the paraspinal musculature. Radiological impression: Suspicious for malignant degeneration of a plexiform neurofibroma. A needle biopsy of the sacral mass yielded the diagnosis 'pleomorphic sarcoma,' with a comment that the lesion is suggestive of malignant peripheral nerve sheath tumor. Operative procedure: Excision of sacral mass. ---- This T2-weighted scan shows innumerable large and small nerve sheath tumors.

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