Intrathoracic Malignant Peripheral Nerve Sheath Tumor: Histopathological and Immunohistochemical Features

Malignant peripheral nerve sheath tumor (MPNST) is a rare nerve sheath tumor derived from Schwann cells or pleuripotent cells of neural crest. Neurogenic tumors make about 10-20% of all mediastinal tumors. Incidence of MPNST is 0.001% in general population and 0.16% in patients with neurofibromatosis I (NF I). We report a case of 60 year female presenting with progressive cough and breathlessness for 2 years. The CECT revealed multiple focal enhancing lesions along inferior mediastinal pleural surface and along lateral pleural surface. A thoracotomy and tumor excision was done and MPNST was diagnosed on microscopy and immunohistochemistry. This case highlights that this unusual tumor may involve lung parenchyma. So this possibility should be kept in mind in patients with intrathoracic mass.