Recurrent Schwannomatosis of the Hand

Background: Peripheral nerve sheath tumors (PNSTs) are neoplastic soft tissue masses generated from the abnormal proliferation of Schwann cells. Often, these tumors occur in isolation and are known as schwannomas or neurilemmomas. The presence of multiple schwannomas is known as schwannomatosis. The purpose of this article is 2-fold: (1) to review the relevant literature and describe a unique case of this rare condition; and (2) to emphasize salient clinical considerations in the diagnosis and treatment of schwannomatosis. Methods: In this report, we describe the case of a 52-year-old white man who presented with multiple recurrent soft tissue masses of the right hand. On initial presentation, he described pain across his right hand and index finger, which persisted despite numerous prior operations. The index finger had a flexion contracture around the location of the proximal interphalangeal joint, and there were multiple tender masses along the length of the finger and palm. Results: Segmental excision of the affected radial digital nerve was performed. A pulp flap based on contralateral neurovascular bundle resulted in a sensate, pain-free digit. Tissue pathology confirmed the diagnosis of multiple neurilemmomas. Conclusions: We report the success of a radial digital neurectomy in a patient with widespread neurilemmomas, who had previously excluded that painful digit from use. It was through careful consideration of the preoperative differential diagnosis, by valuing the preoperative imaging, and by considering all surgical options with specific attention paid to skin flap design that this good outcome of a fully sensate, pain-free, mobile index finger was achieved.