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Malignant peripheral nerve sheath tumors (MPNSTs) are sarcomas on peripheral nerves or nerve sheath. These MPNSTs can occur on any cell type and differs from one to other. A sarcoma is regarded as MPNST only when it is raised on the peripheral nerve or it exists on neurofibroma. MPNSTs usually present as an enlarging palpable mass. Pain is a variable complaint.
Rapid enlargement occurs more often in the setting of NF1 and should raise concern for malignant degeneration of a neurofibroma.In Poland Malignant peripheral nerve sheath tumor (MPNST) accounts for 5-10% of all malignant soft tissue tumors. It often affects the extremities and abdominal cavity and very rarely develops in the thoracic cavity.
The incidence in the general population is 0.001%. In our paper we would like to present a case report of a 74-year-old female patient with primary MPNST of the pleural cavity which was previously misdiagnosed as a non-small cell lung cancer. Despite the combined treatment, after four months the patient developed a secondary tumor in the contralateral pleural cavity which was also treated operatively.