Malignant peripheral nerve sheath tumor pathophysiology

Malignant peripheral nerve sheath tumor Microchapters
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shanshan Cen, M.D. [2]

Pathogenesis

• Malignant peripheral nerve sheath tumors are a rare type of cancer that arise from the soft tissue that surrounds nerves. They are a type of sarcoma. Most malignant peripheral nerve sheath tumors arise from the nerve plexuses that distribute nerves into the limbs—the brachial and lumbar plexuses—or from nerves as they arise from the trunk.

Genetics

• About half of the cases of malignant peripheral nerve sheath tumor (MPNST) occur along with NF1. The lifetime risk of having both of these conditions is at 8–13% while those with only MPNST have a 0.001% in the general population.

• The NF1 gene locus is on chromosome 17q11.2 and the gene product is neurofibromin, acts as a tumour suppressor; inactivation of the gene thus predisposes to tumour development.

Microscopic Pathology

• Cellular

• Usually spindle cells
• Rarely epithelioid

• Nuclear atypia
• Mitoses
• Herring bone pattern