Malignant peripheral nerve sheath tumor

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Malignant peripheral nerve sheath tumor
High magnification micrograph of a malignant peripheral nerve sheath tumor
Synonyms MPNST, neurofibrosarcoma, malignant schwannoma
Pronounce N/A
Specialty N/A
Symptoms Pain, neurological deficits, palpable mass
Complications Metastasis, local recurrence
Onset Typically in adulthood
Duration Variable
Types N/A
Causes Often associated with neurofibromatosis type I
Risks Neurofibromatosis, prior radiation therapy
Diagnosis Biopsy, imaging studies
Differential diagnosis Schwannoma, neurofibroma, fibrosarcoma
Prevention N/A
Treatment Surgery, radiation therapy, chemotherapy
Medication N/A
Prognosis Generally poor, depends on stage and resectability
Frequency Rare, <0.1% of all cancers
Deaths N/A


Malignant peripheral nerve sheath tumor
Malignant peripheral nerve sheath tumor
Autosomal dominant inheritance pattern

Malignant peripheral nerve sheath tumor (MPNST) is a rare form of cancer that originates from the peripheral nerve sheath, the protective layer that surrounds nerves. These tumors are often aggressive and can spread to other parts of the body.

Symptoms[edit]

The symptoms of MPNST can vary depending on the location of the tumor. Common symptoms include a growing lump or mass, pain or discomfort, and neurological problems such as numbness or weakness.

Causes[edit]

The exact cause of MPNST is unknown, but it is often associated with certain genetic conditions such as Neurofibromatosis type 1 (NF1) and Schwannomatosis.

Diagnosis[edit]

Diagnosis of MPNST typically involves a combination of physical examination, imaging tests such as MRI or CT scan, and biopsy.

Treatment[edit]

Treatment for MPNST often involves surgery to remove the tumor, followed by radiation therapy or chemotherapy to kill any remaining cancer cells.

Prognosis[edit]

The prognosis for MPNST varies depending on factors such as the size and location of the tumor, the patient's overall health, and the success of treatment.

See also[edit]

References[edit]

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