Segmental Schwannomatosis in the Upper Extremity: A Case Report and Review of Literature

Schwannomas, the most frequently occurring benign tumors of the peripheral nerve sheath, generally remain as painless swellings for several years before diagnosis. Multiple schwannomas involving different nerves within the same extremity are rare. We report a rare case of a 61-year-old female who presented with multiple schwannomas in the palmar common and proper digital nerves, 15 years after the resection of a median nerve schwannoma within the same upper extremity. Using preestablished diagnostic criteria, she was diagnosed with segmental schwannomatosis. After careful surgical resection, biopsy confirmed the diagnosis and she recovered without neurological symptoms or limitations in the range of motion. Literature review revealed only four case series on segmental schwannomatosis, indicating its rarity. Postoperative sensory deficits are more likely in cases with multiple schwannomas in the common and proper digital nerves. We demonstrate that such complications can be avoided by meticulous dissection and separation of the tumors from the nerve fibers.