Guo Xiang, Wu Wei-Ming, Wang Lei, Yang Yi
Department of Thoracic Surgery, Shanghai Jiao Tong University Affiliated Sixth People's Hospital, Shanghai 200233, China.
World J Clin Cases. 2021 Aug 26;9(24):7117-7122. doi: 10.12998/wjcc.v9.i24.7117.
Malignant peripheral nerve sheath tumors (MPNSTs) are a group of rare and aggressive sarcomas that often arise from major peripheral nerves and represent a notable challenge to efficacious treatment. MPNSTs can occur in any body surface and visceral organs with nerve fiber distribution. The treatment options for MPNSTs include surgery, chemotherapy, and adjuvant radiotherapy.
A 26-year-old female cellist presented with chest pain on her left side when she squatted to lift the cello. One week later, a chest X-ray was performed and revealed fracture of the fourth rib on the left side. Three months later, the patient inadvertently touched a mass on the left side of the chest wall. Chest computed tomography (CT) three-dimensional reconstruction of the ribs revealed bone destruction of the fourth rib on the left side with a soft tissue mass shadow measuring 5.7 cm × 3.7 cm. CT-guided puncture biopsy of the tumor showed that heterotypic cells (spindle cells) tended to be nonepithelial tumor lesions. PET-CT demonstrated bone destruction and a soft tissue mass with avid 18F-fluorodeoxyglucose activity (SUV7.5) in the left fourth rib. The tumor of the left chest wall was resected under general anesthesia, and reconstruction of the chest wall was performed. The postoperative pathological report exhibited an MPNST.
MPNSTs are relatively chemo-insensitive tumors. The mainstay of treatment for MPNSTs remains resection with tumor-free margins.
恶性外周神经鞘瘤（MPNSTs）是一组罕见且侵袭性强的肉瘤，常起源于主要外周神经，对有效治疗构成显著挑战。MPNSTs可发生于任何有神经纤维分布的体表和内脏器官。MPNSTs的治疗选择包括手术、化疗和辅助放疗。
一名26岁的女性大提琴演奏者在蹲下搬大提琴时出现左侧胸痛。一周后进行胸部X线检查，显示左侧第四肋骨骨折。三个月后，患者无意中摸到胸壁左侧有一肿块。胸部肋骨计算机断层扫描（CT）三维重建显示左侧第四肋骨骨质破坏，伴有一个大小为5.7 cm×3.7 cm的软组织肿块阴影。对肿瘤进行CT引导下穿刺活检，结果显示异型细胞（梭形细胞）倾向于非上皮性肿瘤病变。PET-CT显示左侧第四肋骨骨质破坏及一个18F-氟脱氧葡萄糖摄取活跃（SUV 7.5）的软组织肿块。在全身麻醉下切除左侧胸壁肿瘤，并进行胸壁重建。术后病理报告显示为MPNST。
MPNSTs是相对化疗不敏感的肿瘤。MPNSTs的主要治疗方法仍然是进行切缘无肿瘤的切除。
