title: Treatment of a malignant peripheral nerve sheath tumor and its complications through a multidisciplinary approach.
abstract: The authors report the case of a 14-year-old girl with a residual malignant peripheral nerve sheath tumor after thoracotomy, chemotherapy, and radiation therapy. The residual tumor, which involved the intercostal muscles, aorta, and neural foramina of T4-10, was completely resected through a costotransversectomy and multiple hemilaminotomies with the patient in the prone position and was stabilized using a T1-12 pedicle screw fusion. Postoperatively, the patient developed several infections requiring multiple washouts and prolonged antibiotics. Thirty months after surgery, she developed a bronchocutaneous fistula. The hardware was removed, and a vascularized latissimus dorsi free flap was placed over the lung. She continued to have an air leak and presented 3 weeks later with a 40° left thoracic curve. She returned to the operating room for a T2-L2 fusion with a vascularized fibular graft. On postoperative Day 1, she underwent a bronchoscopy and had her left lower lobe airways occluded with multiple novel one-way endobronchial valves. She is now 5 years out from her tumor resection and 3 years out from her definitive fusion. She has no evidence of residual tumor, infection, or pseudarthrosis and continues to remain asymptomatic.
authors.affiliation: Division of Neurosurgery, Children’s Hospital of Philadelphia, University of Pennsylvania Medical Center, Philadelphia, Pennsylvania 19104, USA. eamonmcl@mail.med.upenn.edu
authors.forename: Eamon J
authors.initials: EJ
authors.lastname: McLaughlin
authors.forename: Gregory G
authors.initials: GG
authors.lastname: Heuer
authors.forename: Robert G
authors.initials: RG
authors.lastname: Whitmore
authors.forename: John K
authors.initials: JK
authors.lastname: Birknes
authors.forename: Jean
authors.initials: J
authors.lastname: Belasco
authors.forename: Daniel
authors.initials: D
authors.lastname: Sterman
authors.forename: David W
authors.initials: DW
authors.lastname: Low
authors.forename: Phillip B
authors.initials: PB
authors.lastname: Storm
mesh_terms: D001706:Biopsy; D003131:Combined Modality Therapy; D003299:Cooperative Behavior; D005500:Follow-Up Studies; D006801:Humans; D007091:Image Processing, Computer-Assisted; D033183:Interdisciplinary Communication; D008279:Magnetic Resonance Imaging; D020360:Neoadjuvant Therapy; D018365:Neoplasm, Residual / Q000150:complications / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery; D018317:Nerve Sheath Neoplasms / Q000150:complications* / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery*; D009460:Neurologic Examination; D010348:Patient Care Team; D011183:Postoperative Complications / Q000175:diagnosis / Q000209:etiology / Q000601:surgery; D011788:Quality of Life; D050397:Radiotherapy, Intensity-Modulated; D012086:Reoperation; D013123:Spinal Fusion; D013125:Spinal Neoplasms / Q000150:complications / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery*; D013899:Thoracic Neoplasms / Q000150:complications / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery*; D013904:Thoracic Vertebrae / Q000473:pathology / Q000601:surgery; D013906:Thoracoscopy; D013908:Thoracotomy; D014057:Tomography, X-Ray Computed; D047368:Tumor Burden