Case ReportBrain Metastasis From Malignant Peripheral Nerve Sheath Tumors
Introduction
Malignant peripheral nerve sheath tumors (MPNSTs) are rare soft tissue sarcomas that occur most commonly in patients with neurofibromatosis type 1, but also can occur spontaneously in otherwise healthy patients (∼0.001% incidence)1 and after radiation. MPNSTs are usually found arising from peripheral nerves, but from cranial nerves as well.2 These tumors often have a poor outcome despite wide local surgical resection followed by radiotherapy with or without chemotherapy.1, 3, 4 Despite these treatments, local recurrence and distant metastases are common. We present a patient who developed a metastatic lesion to the brain after initially being treated for an MPNST of the sciatic nerve. In addition, we searched our surgical records to identify any other cases of pathology-proven brain metastases after initial diagnosis and treatment of an MPNST.
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Case Report
A 56-year-old man presented with symptoms of right thigh pain as well as weakness of the lower extremity that was progressive in nature, ultimately requiring him to use a cane for ambulation. He had no pertinent past medical history, only reporting a remote history of trauma to the right leg approximately 25 years prior (fall resulting in a femur and tibia/fibula fracture requiring fixation). On initial physical examination, he had an area of tenderness to percussion on the distal aspect of the
Review of Records at our Institution
Approval for this study was obtained from our Institutional Review Board. A search of our surgical records at our institution was performed using an institutionally created tool that searches all clinical notes for a particular search term or string. It identifies any patients whose clinical data contain the searched term. For our search, we used the term MPNST as well as malignant+peripheral+nerve+sheath+tumor. After patients had been identified, we excluded any patient who did not have a
Discussion
Although MPNSTs are a very rare soft tissue sarcoma, metastatic lesions to the brain are even less common, with at present only 21 cases reported in the literature.1, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25 Recent data from a large series of MPNSTs suggest that median time to distant recurrence was 1.4 years, with the most common location being the lungs, followed by the paraspinal region.26 With the addition of the case at our institution to the
Conclusion
Brain metastases from MPNSTs are very rare and represent a poor prognosis, with survival after brain metastasis reported to be approximately 10 months. Early and effective initial diagnosis and treatment of MPNSTs likely represent the best opportunity for increased overall survival.
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Conflict of interest statement: The authors declare that the article content was composed in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.