Reconstruction using a Free Lateral Leg Perforator Flap after Excision of a Malignant Peripheral Nerve Sheath Tumor of the Hand - A Case Report and Literature Review

Background: Malignant peripheral nerve sheath tumor (MPNST) is a rare form of soft tissue sarcoma with high recurrence rates and poor survival outcomes. Primary MPNST arising from the hand is extremely rare. Only isolated case reports regarding hand MPNSTs have appeared in the literature. Aim and Objectives: In this report we present our experience managing a patient with MPNST of the hand, and review the relevant literature. Materials and Methods: A 62-year-old man with no clinical signs or family history of neurofibromatosis type 1 presented with an asymptomatic soft tissue mass increasing in size over the first volar web of his left index finger. The tumor was attached to the radial proper palmar digital nerve of the index finger during initial en bloc excision. The histopathologic analysis revealed MPNST with a positive margin. The imaging studies showed no distant metastasis. One month later, the patient underwent wide amputation of the index and long fingers that achieved a negative margin status. A free proximal lateral leg perforator flap was used for defect reconstruction. Results: The flap survived completely, without any sequelae. The patient was disease free at nearly 2 years of follow-up. Excellent hand function was retained, and the esthetic result of the reconstructed hand was satisfactory. Conclusions: The nature of MPNST is highly aggressive. To date, surgical excision with tumor -free margins remains the mainstay of treatment. For hand MPNSTs, the surgeon performing oncologic resection should avoid either incomplete or unnecessary resection. When feasible, wide-margin and function-sparing surgical excision is the cornerstone of effective treatment for hand MPNSTs.