ASPN - Weakness At Presentation Does Not Affect Outcome In Motor Nerve Malignant Peripheral Nerve Sheath Tumors

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Weakness At Presentation Does Not Affect Outcome In Motor Nerve Malignant Peripheral Nerve Sheath Tumors
Ross Puffer, MD; Robert J. Spinner, MD
Mayo Clinic, Rochester, MN

Introduction: Malignant peripheral nerve sheath tumors of major motor nerves often present with pain and/or weakness. It is not known whether weakness as a presenting symptom confers any change in outcome compared to patients without weakness at presentation

Methods: A retrospective search involving MPNST cases at our institution since 1994 was performed. Cases not involving a major motor nerve were excluded. Outcomes and other factors were compared between patients presenting with documented weakness and those that did not

Results: A total of 181 cases of MPNST were reviewed. Of these, 65 involved a major motor nerve. Thirty-six patients (55%), presented with weakness, while 45% had only pain, sensory abnormality or no symptoms at all. MPNST location included 25 (38%) in the upper extremity, and 41 (62%) in the lower extremity. Complete excision (negative histologic margins) was performed in 51 (78%) patients. Adjuvant chemotherapy/radiation was performed in 52 (80%) patients. At the time of this abstract, 23 (35%) patients had died, and the mean time to death or last follow-up for the entire cohort was 35.9 +/- 4.9 weeks. There was no statistically significant difference between age, histologic grade, presence of metastases at presentation, ability to perform complete excision or outcome between patients presenting with weakness and those that did not.

Conclusion: In cases of MPNST involving a major motor nerve of the upper or lower extremity, weakness at presentation may not affect long term outcome.

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