title: Giant malignant peripheral nerve sheath tumor of the scalp.
abstract: OBJECTIVE
  Malignant peripheral nerve sheath tumors (MPNST) constitute a group of soft tissue neoplasms with neurogenic origin. Association of MPNST with neurofibromatosis type 1 (NF1) has been frequently reported as a result of malignant transformation of a pre-existing plexiform neurofibroma. However, most of the cases are small and few have been described with giant dimensions. We describe a case of a giant MPNST in an unusual location (scalp), in a NF1 patient.
  
  
  METHODS
  A fifty-six years old woman with a history of NF1 since childhood was admitted due to a rapid growing of a scalp tumor in occipital region. The mass had 15 cm in the largest dimension and a central ulceration with purulent secretion. Computed tomography revealed no bone invasion. The tumor was surgically removed and the histopathological examination revealed a neurofibrosarcoma. The patient had a good post operatory evolution and notwithstanding the tumor size, no metastasis was found and she was discharged to oncologic follow-up.
  
  
  CONCLUSIONS
  Given the poor prognosis of MPNST, patients with NF1 should be aware that neurofibromas can undergo malignant transformation and report signs such as rapid growth of a pre-existing lesion.
authors.affiliation: Department of Internal Medicine, Santa Casa de Misericordia de Maceio Hospital, Alagoas, Brazil. flavioteles@hotmail.com
authors.forename: Teles
authors.initials: T
authors.lastname: F
authors.forename: Ataíde
authors.initials: A
authors.lastname: A M M
authors.forename: De Lima
authors.initials: de L
authors.lastname: B A
authors.forename: Costa
authors.initials: C
authors.lastname: T C C
authors.forename: Lins
authors.initials: L
authors.lastname: R C
authors.forename: Barbosa
authors.initials: B
authors.lastname: G H T S
authors.forename: Costa
authors.initials: C
authors.lastname: H
mesh_terms: D005260:Female; D006801:Humans; D008875:Middle Aged; D018317:Nerve Sheath Neoplasms / Q000150:complications* / Q000601:surgery; D009456:Neurofibromatosis 1 / Q000150:complications* / Q000473:pathology / Q000601:surgery; D009418:S100 Proteins / Q000378:metabolism; D012535:Scalp / Q000473:pathology*
chemical_list: D009418:S100 Proteins