Abstract
BACKGROUND: 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. CASE SUMMARY: 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 (SUV(max)7.5) 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. CONCLUSION: MPNSTs are relatively chemo-insensitive tumors. The mainstay of treatment for MPNSTs remains resection with tumor-free margins.