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.
Keywords: Malignant peripheral nerve sheath tumor; en bloc excision; radiation therapy; recurrence; soft-tissue sarcoma.
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