Jejunal Malignant Peripheral Nerve Sheath Tumor with Preoperative Suspicion of Gastrointestinal Stromal Tumor: Report of a Korean Case and Literature Review

Background: Malignant Peripheral Nerve Sheath Tumors (MPNSTs) are defined as malignant tumors arising from a peripheral nerve or displaying sheath differentiation. We report the first Korean case of MPNST ileum. Case Presentation: A 33-year-old female without family history neurofibromatosis type I complained lower abdominal pain. An abdomen computed tomography and enhanced magnetic resonance imaging pelvis confirmed 5.6 cm heterogenous lobulated lesion in Laparoscopic exploration revealed severe adhesion main ileal mass to sigmoid colon. adhesiolysis segmental resection with anastomosis ileum was performed. On gross examination, resected specimen 6.5 tumor located 10 proximal 15 distal margins. In microscopic nucleus cells oval elongated shape, showing moderate pleomorphism. Immunohistochemical staining showed that were positive for S100 about 10% Ki-67. During 12-month follow-up period, she presented no symptoms CT scans evidence distant metastasis thorax recurrence. Conclusion: rare young jejunum, suspected having GIST before surgery an extensive review literature intestinal tract.