Nerve sheath myxoma

Nerve Sheath Myxoma is a rare, benign neoplasm that originates from the Schwann cells of the peripheral nerves. It is also known as neurothekeoma, a term coined by Gallager and Helwig in 1980.

Epidemiology

Nerve sheath myxoma is a rare tumor, with no specific age, sex, or racial predilection. It can occur at any age but is most commonly seen in adults.

Pathogenesis

The exact pathogenesis of nerve sheath myxoma is not well understood. It is believed to originate from the Schwann cells, which are the principal glia of the peripheral nervous system. Schwann cells play a crucial role in the maintenance of the myelin sheath, a fatty layer that covers the nerve fibers and aids in the rapid transmission of nerve impulses.

Clinical Features

Nerve sheath myxomas typically present as a slow-growing, painless mass. The most common locations are the upper and lower extremities, followed by the head and neck region. However, they can occur anywhere in the body where peripheral nerves are present.

Diagnosis

The diagnosis of nerve sheath myxoma is primarily based on histopathological examination. Immunohistochemistry is often used to confirm the diagnosis. The tumor cells are typically positive for S-100 protein, a marker for cells derived from the neural crest, including Schwann cells.

Treatment and Prognosis

The treatment of choice for nerve sheath myxoma is surgical excision. The prognosis is generally good, as these tumors are benign and have a low risk of recurrence if completely excised.

See Also

• Neoplasm
• Schwann cell
• S-100 protein