MALIGNANT PERIPHERAL NERVE SHEATH TUMOUR AT GASTROESOPHAGEAL JUNCTIONA RARE CASE

Malignant peripheral nerve sheath tumors (MPNSTs) are very rare and , represents 3 - 10% of all soft tissue sarcomas.Approximately half of all such tumors are diagnosed in patients with neurofibromatosis type 1 (NF1). The extremities areinvolved most commonly, and the patient's age is usually 20 - 50 years. We herein describe a case of 63 year old lady withgastroesophageal junction MPNST, which was diagnosed histopathologically after surgery. The patient underwentcurative esophagogastrectomy with D2 lymph node dissection and Roux-en-Y esophagojejunostomy. Thepostoperative recovery was uneventful. The ideal adjuvant treatment protocol is yet to be decided due to the relativelylimited number of cases of these tumours previously reported.