MPNST

Origins and Causes

MPNST is a type of soft tissue cancer that occurs in peripheral nerve tissues. MPNST is a rare soft tissue cancer that accounts for about 2% of all sarcomas. Patients with neurofibromatosis type 1 are at higher risk of developing MPNST compared to the general population.

 

Symptoms and Signs

MPNST can present as a progressively growing painful or painless mass. Large nerves such as the sciatic nerve, brachial plexus, and sacral plexus are the preferred sites for MPNST. Rapid growth of a known neurofibroma should raise suspicion of MPNST development.

 

Diagnosis

When a sarcoma is suspected, the initial evaluation includes either a CT scan or an MRI. The diagnosis is confirmed through biopsy of the suspected area. The investigation can be further completed with a chest CT scan and a PET scan.

 

Treatment

When a MPNST is localized, the treatment of choice is surgery to remove it completely. Regularly, radiation therapy before or after surgery is necessary. Adjuvant (preventative) chemotherapy may sometimes be offered.

When metastases are found, chemotherapy is offered with the aim of controlling symptoms and disease progression. The preferred agents are doxorubicin, ifosfamide, and pazopanib. Clinical trials may also be explored.

 

Follow-up

After surgery, patients with synovial sarcoma will be closely monitored due to the risk of recurrence (when cancer returns)