Zaheer Samreen, Khosla Divya, Gupta Vikas, Nada Ritambhra, Kumar Divyesh, Kapoor Rakesh
Department of Radiotherapy, Postgraduate Institute of Medical Education and Research (PGIMER), Regional Cancer Centre, Chandigarh, India.
Department of Surgical Gastroenterology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
J Gastrointest Cancer. 2023 Mar;54(1):259-263. doi: 10.1007/s12029-021-00753-4. Epub 2021 Nov 18.
Malignant peripheral nerve sheath tumor (MPNST) of small bowel mesentery is a rare tumor. We report a rare case of MPNST of small bowel mesentery in a patient without neurofibromatosis (NF).
A 50-year-old male, with no features suggestive of NF1, presented to us with complaints of pain abdomen. Contrast-enhanced computed tomography (CECT) of the abdomen revealed a mass in the infrarenal region. On laparotomy, mass was seen to be arising from the mesentery of the jejunum. En-bloc resection of the tumor was done, and histopathological examination was suggestive of malignant peripheral nerve sheath tumor of the small bowel mesentery.
Patient received adjuvant external beam radiotherapy to a dose of 50.4 Gy to the tumor bed. The patient was planned for chemotherapy but absconded and later came with recurrence. The patient finally succumbed to disease.
Surgery is the mainstay of treatment. Adjuvant treatment should be based on histopathological report.
小肠系膜恶性外周神经鞘瘤（MPNST）是一种罕见肿瘤。我们报告一例无神经纤维瘤病（NF）患者的小肠系膜MPNST罕见病例。
一名50岁男性，无提示NF1的特征，因腹痛主诉前来就诊。腹部增强计算机断层扫描（CECT）显示肾下区域有一肿块。剖腹手术时，可见肿块起源于空肠系膜。对肿瘤进行了整块切除，并进行了组织病理学检查，提示为小肠系膜恶性外周神经鞘瘤。
患者接受了肿瘤床50.4 Gy的辅助外照射放疗。患者计划接受化疗，但潜逃，后来复发前来。患者最终死于该疾病。
手术是主要治疗方法。辅助治疗应基于组织病理学报告。
