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Segmental Schwannomatosis of the Upper Extremity
Lauren Wright, DO; Thomas W. Bauer MD, PhD; William H. Seitz, MD
Cleveland Clinic - South Pointe Hospital
2017-01-31
Presenter: Lauren Wright
Affidavit:
Writing and editing of manuscript were performed by resident under supervision of Dr. Seitz and Dr. Bauer.
Director Name: Mark Foglietti
Author Category: Resident Plastic Surgery
Presentation Category: Clinical
Abstract Category: Hand
Schwannomatosis, characterized by the development of multiple schwannomas without evidence of vestibular tumors, represents one of three major forms of neurofibromatosis. A rare variant of schwannomatosis is segmental schwannomatosis, defined as being limited to one limb or five or fewer contiguous segments of the spine. Nineteen cases of segmental schwannomatosis of the upper extremity have since been reported to date in the English literature, making this case the twentieth. We report a case of a 38 year old male who presented with multiple enlarging masses of the right upper extremity. The masses were progressively painful and incapacitating with associated numbness and paresthesia in the ulnar and radial nerve distribution. An open, staged surgical approach provided definitive diagnosis and successful treatment through excision of tumors with the aid of collagen neural conduits to provide a protective environment during healing. We additionally review literature regarding pathogenesis, diagnosis, and treatment of this uncommon tumor.
