Malignant Peripheral Nerve Sheath Tumor Arising from Small Bowel Mesentery: an Extremely Rare Case with Review of Literature.

J Gastrointest Cancer

Department of Radiotherapy, Postgraduate Institute of Medical Education and Research (PGIMER), Regional Cancer Centre, Chandigarh, India.

Published: March 2023

Purpose: 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).

Methods: 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.

Result: 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.

Conclusion: Surgery is the mainstay of treatment. Adjuvant treatment should be based on histopathological report.

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http://dx.doi.org/10.1007/s12029-021-00753-4DOI Listing

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