title: A Case of Occipital Malignant Peripheral Nerve Sheath Tumor with Neurofibromatosis Type 1.
abstract: BACKGROUND
  The prognosis of malignant peripheral nerve sheath tumor (MPNST) with neurofibromatosis type 1 (NF-1) is worse than that of a solitary MPNST, because of the tumor size and location difficult to resect completely. We experienced a case of MPNST in the occipital region with NF-1.
  
  
  METHODS
  A 59-year-old woman presented with NF-1 and an MPNST of the occipital region. We performed wide excision involving the occipital bone, and reconstructed with a titanium plate and a free latissimus dorsi muscle flap. Despite three operations and postoperative radiotherapy, the tumor locally recurred after each surgery; further complicated by lung and mediastinal metastasis. Adjuvant therapy was insufficient to control local recurrence, which was observed intracranially, resulting in sagittal sinus invasion. Thirty months after the initial surgery, the patient died of respiratory failure due to lung metastasis.
  
  
  CONCLUSIONS
  MPNST with NF-1 has poorer prognosis than that of a solitary lesion. Recently, it is reported in several literature that combination therapy with surgery and radiotherapy improve survival rates. But as we presented, when the complete local resection with free margin could not achieved due to the size and anatomical location of the tumor, the effectiveness of radiotherapy is not sufficient to control local recurrence.
authors.affiliation: Department of Plastic Surgery, Tokai University School of Medicine, 143 Shimokasuya, Isehara, Kanagawa 259-1193, Japan. hanaiushio@icloud.com.
authors.forename: Ushio
authors.initials: U
authors.lastname: Hanai
authors.forename: Tadashi
authors.initials: T
authors.lastname: Akamatsu
authors.forename: Megumi
authors.initials: M
authors.lastname: Kobayashi
authors.forename: Yotaro
authors.initials: Y
authors.lastname: Tsunoda
authors.forename: Kenichi
authors.initials: K
authors.lastname: Hirabayashi
authors.forename: Tanehumi
authors.initials: T
authors.lastname: Baba
authors.forename: Hideki
authors.initials: H
authors.lastname: Atsumi
authors.forename: Mitsunori
authors.initials: M
authors.lastname: Matsumae
mesh_terms: D001932:Brain Neoplasms / Q000601:surgery*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008175:Lung Neoplasms / Q000556:secondary; D008479:Mediastinal Neoplasms / Q000556:secondary; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local; D009378:Neoplasms, Multiple Primary; D009442:Neurilemmoma / Q000601:surgery*; D009456:Neurofibromatosis 1 / Q000601:surgery*; D019635:Neurosurgical Procedures / Q000379:methods; D009778:Occipital Lobe; D018714:Radiotherapy, Adjuvant; D019651:Plastic Surgery Procedures / Q000379:methods