Mulimani PN, Kadam SS
Abstract: Malignant Peripheral Nerve sheath tumour (MPNST) is defined as a malignant tumour arising from a peripheral nerve or showing nerve sheath differentiation, with the exception of tumours originating from epineurium or the peripheral nerve vasculature. We present an extremely rare case of (MPNST) in a 50-year-old woman without associated neurofibromatosis arising from thorax. Patient had come to the Emergency Department with complaints of shortness of breath since15 days, dry cough since 15 days and with an incidental history of chest trauma 1 month ago. Chest x ray showed a mass in the left hemi thorax, CECT of chest showed a large enhancing solid tumour measuring 11.5cm x 8cm x11cm.USG guided biopsy revealed low grade spindle cell lesion with possible diagnosis of Myxofibroma, Schwannoma with focal degenerative changes and Reactive Myxofibromatous degenerative lesion. The patient underwent excision of mediastinal mass through left postero lateral thoracotomy under general anaesthesia. Histology of resected mass showed a biphasic neoplasm with a spindle cell component admixed with glandular elements, suggestive of Synovial sarcoma, malignant peripheral nerve sheath tumour in possible pre-existing Schwannoma.IHC report was suggestive of malignant peripheral nerve sheath tumour (MPNST).
Keywords: MPNST, Solid tumour, Spindle cell, Biphasic Neoplasm, tumour size