Successful Outcome Following Limb Conservation Surgery for a Recurrent Malignant Peripheral Nerve Sheath Tumour: A Case Report.

INTRODUCTION

A malignant peripheral nerve sheath tumor (MPNST) is a rare soft-tissue sarcoma with a high recurrence rate and poor prognosis. Early diagnosis and complete surgical excision are the fundamental principles of treatment. A benign presentation and low clinical suspicion often delay definitive diagnosis, and en bloc excision may not be feasible depending on the size and location of the tumor. We describe a rare case of a recurrent MPNST successfully treated by surgical excision.

CASE REPORT

A 35-year-old woman presented with a rapidly growing painful mass 3 months following incomplete removal of a MPNST from her forearm. Staging investigations showed no evidence of metastasis. The patient underwent en-bloc surgical excision, split skin grafting, and adjuvant radiation therapy. Histology and immunohistochemical analysis confirmed a MPNST. Five years after having surgery, the patient shows no evidence of recurrence and has excellent function.

CONCLUSION

MPNST are rare soft tissue sarcomas that can masquerade as benign lumps. There is a paucity of literature on the outcome of surgically-treated recurrent disease. Notwithstanding local recurrence of the tumor, complete surgical excision can yield excellent clinical results.