Malignant peripheral nerve sheath tumor

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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

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

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

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

Treatment

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

Prognosis

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

• Neurofibromatosis
• Schwannoma
• Sarcoma

References

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