Recurrent Schwannomatosis of the Hand

Abstract

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.

Segmental schwannomatosis in the upper extremity: a case report and review of the literature

“…Although our patient did not undergo genetic assessment, mutations of either the SMARCB1 or the LZTR1 tumor suppressor genes have been identified in 86% of the patients with a familial history of schwannomatosis [2].…”

“…Schwannomas (or neurilemmomas) are the most frequently occurring benign tumors of the peripheral nerve sheath [1,2]. Commonly occurring in the fourthsixth decades of life without any sex predilection [3,4], they are mostly observed in the volar aspect of the forearm and hand.…”

“…Commonly occurring in the fourthsixth decades of life without any sex predilection [3,4], they are mostly observed in the volar aspect of the forearm and hand. They reportedly comprise 12% of all soft-tissue neoplasms of the hand [1][2][3].…”

“…Because schwannomas are generally manifested as painless swellings with less common neurological deficits, the time to their diagnosis can vary from a few months to several years [4]. Magnetic resonance imaging (MRI) and ultrasonography are helpful in their diagnosis and localization [1,2]. Schwannomas are usually solitary lesions, and multiple schwannomas of different nerves within the same extremity are rare [3].…”

Recurrent schwannoma of digital nerve on both hands: A very rare case report

“…In the case of the upper extremity schwannoma, the ulnar nerve is the most common nerve involved, and the median nerve is the second most commonly involved. The occurrence of this lesion on the digital nerve is extremely rare and only reported in several literatures: Troy et al [6] , Pertea et al [7] , Han KJ et al [8] , Aleksić et al [9] , and Nicolescu et al [10] .…”