Primary intrapulmonary malignant peripheral nerve sheath tumor: A rare case

Abstract

A rare case of malignant peripheral nerve sheath tumor (MPNST) was found in the lungs of a 68-year-old male patient. He was referred to our hospital because of visual disturbances, confusion, and headaches. Upon admission, a chest-Abdomen-Pelvis CT scan (CT scan of CAP) showed a right posterobasal pulmonary lesion process associated with liver metastases and a lytic lesion of the vertebral body of D3. Brain MRI (Magnetic Resonance Imaging) revealed secondary brain lesions above and below the tentorium. Image guided liver biopsy showed a suspicious site of malignancy. In the histopathological examination, a diagnosis of MPNST was established. A biological agent, Imatinib, was administered and the patient underwent cerebral radiotherapy. CT scan of CAP showed a partial tumor response according to Chun's criteria. Pre-existing literature indicates that MPNSTs with metastases to the brain are very rare and have a poor prognosis – survival after brain metastases would be about 10 months. Our case report agrees with the literature, as the disease stabilized with treatment up to 7 months, after which the patient was unreachable and our team lost track of him. Our case report appears to be one of the first cases of primary MPNST in the lungs with brain metastases.

Keywords

• Chemotherapy
• Lung
• Malignant peripheral nerve sheath tumor
• Malignant schwannoma
• Metastases