Case of Malignant Peripheral Nerve Sheath Tumor (MPNST) of Lung

Malignant peripheral nerve sheath tumor (MPNST) is very unusual type of soft tissue sarcoma (STS) and occurs mostly in patients of neurofibromatosis. The occurrence of MPNST in lung is particularly rare. Only very rarely cases have been reported till date in absence of history of neurofibromatosis. We are reporting such case in a 34 year old female who presented with complains of chest pain, breathlessness and nonproductive cough since 3 months. Microscopic examination revealed a malignant spindle cell tumor of low grade histology. Immunohistochemistry (IHC) was performed which was positive for vimentin and actin and negative for desmin and S- 100. Final diagnosis of MPNST was made on the basis of morphological appearance and IHC. Patient was treated with neoadjuvant chemotherapy ifosfamide and adriamycin for four cycles, followed by surgical excision. At present patient is on regular follow up for one year post treatment.

Keywords: lung cancer; Malignant peripheral nerve sheath tumors.