Open AccessMALIGNANT PERIPHERAL NERVE SHEATH TUMOUR AT GASTROESOPHAGEAL JUNCTIONA RARE CASE
Author(s) -
Praveen babu j,
Sankar Sambandam,
Sandhya Sundaram,
Suresh Kumar P,
Chinni Vikram Asokan
Publication year - 2021
Publication title -
paripex indian journal of research
Language(s) - English
DOI - 10.36106/paripex/9011894
Subject(s) - medicine , neurofibromatosis , surgery , dissection (medical) , gastroesophageal junction , lymph node , soft tissue , malignant peripheral nerve sheath tumor , radiology , cancer , pathology , adenocarcinoma
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.