Malignant peripheral nerve sheath tumor

A malignant peripheral nerve sheath tumor (MPNST) is a tumor that develops in the protective lining that covers nerves.^[1][2][3] The first symptom of MPNST is often a lump or mass that increases in size, sometimes causing pain or a tingling sensation.^[1] MPNST is considered an aggressive tumor because there is up to a 65% chance of the tumor regrowing after surgery (a recurrence),^[1][4] and approximately 40% chance of spreading to distant parts of the body (a metastasis), most commonly to the lung.^[5][6][7] Treatment of MPNST begins with surgery to remove as much of the tumor as possible. Radiation therapy may be used to decrease the chance of a recurrence. Chemotherapy might be used if the whole tumor cannot be removed during surgery, or to treat a metastasis.^[1][2] MPNSTs are quite rare, occurring in 0.001% of the general population.^[8]  Approximately 25-50% of MPNSTs are associated with a genetic condition known as neurofibromatosis type 1.^[9]

Related diseases are conditions that have similar signs and symptoms. A health care provider may consider these conditions in the table below when making a diagnosis. Please note that the table may not include all the possible conditions related to this disease.

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• I was diagnosed with a malignant peripheral nerve sheath tumor in my right thigh.  The tumor was taken out surgically.  Surgery was followed by 30 sittings of radiation therapy.  Several months later, a lump was felt on top of the scar from the first surgery and tests confirmed the lump was the same kind of tumor.  Again, the tumor has been removed surgically and chemotherapy is needed.  Other tests suggest the tumor has spread to the lungs.  What is the prognosis for this type of tumor? See answer