Giant intrapulmonary malignant peripheral nerve sheath tumour

Pulmonary malignant peripheral nerve sheath tumours (MPNSTs) are extremely rare soft tissue sarcomas that develop from the cell constituting the nerve sheaths, approximately 5-10% of all soft tissue sarcomas. We present a rare case of primary lung MPNST in an adult female non-smoker patient, in whom surgical thoracotomy approach has obtained a good control of the disease. Low-grade MPNST was established from excisional biopsy followed by immunohistochemistry.