Abstract
Cervical vagal schwannoma is a rare entity among lesions presenting as a neck mass. They are usually slow-growing benign lesions closely associated with the vagus nerve. They are usually solitary and asymptomatic. Multiple schwannomas occurring in patients without neurofibromatosis (NF) are rare and have recently been referred to as schwannomatosis. Here, we present a case of a neck mass that had imaging features suggestive of vagal schwannoma and was operated upon. Intraoperatively, it was discovered to be a case of multiple vagal cervical schwannoma, all directly related to the right vagus nerve, and could be resected from the nerve in toto preserving the function of the vagus nerve. Final HPR confirmed our pre-op suspicion of vagal schwannomatosis.
Highlights
Schwannoma is defined as a benign tumor of neural origin derived from the benign nerve sheath composed of Schwann cells, which normally produce the insulating myelin sheath covering the peripheral nerves [1]
Schwannomatosis is a term relatively recently introduced that defines a condition of multiple schwannomas related to a cranial nerve, spinal nerve root, or a peripheral nerve [3]
Multiple schwannomas are usually seen in cases of neurofibromatosis 2 but rarely seen otherwise
Summary
Schwannoma is defined as a benign tumor of neural origin derived from the benign nerve sheath composed of Schwann cells, which normally produce the insulating myelin sheath covering the peripheral nerves [1]. Schwannoma of the vagus nerve is a rare entity, seen in only a handful of cases [2]. Most have been seen among middle aged men as an asymptomatic neck swelling [1]. They have been seen as a solitary swelling arising from the cervical vagus nerve, even though mediastinal origin has been seen. Multiple schwannomas are usually seen in cases of neurofibromatosis 2 but rarely seen otherwise. We hereby present a rare case of schwannomatosis of the cervical vagus nerve not otherwise associated with neurofibromatosis 2 (NF-2) [5]
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