title: Primary intracranial leiomyosarcoma of the torcular Herophili associated with Fanconi anemia and allogenic stem cell transplantation.
abstract: OBJECTIVE
  Fanconi anemia is associated with a high risk for developing malignant tumors. The occurrence of primary intracranial leiomyosarcoma, however, which in general has a poor prognosis, has not been described thus far. The purpose of this study is to report on management and outcome of leiomyosarcoma of the torcular Herophili associated with Fanconi anemia in a pediatric patient.
  
  
  METHODS
  A 12-year-old girl with Fanconi anemia presented with a primary intracranial leiomyosarcoma arising from the torcular Herophili and infiltrating the adjacent venous sinuses after previous allogenic hematopoietic stem cell transplantation. Radical tumor resection followed by radiotherapy resulted in tumor-free survival and good outcome at a 2-year follow-up.
  
  
  CONCLUSIONS
  Despite occurrence of leiomyosarcoma in a site thought unfavorable for surgery, combined tumor resection and radiosurgery may yield excellent outcome.
authors.affiliation: Department of Neurosurgery, Medical School, MHH, Carl-Neuberg-Straße 1, 30625, Hannover, Germany.
authors.forename: Michèle
authors.initials: M
authors.lastname: Aumüller
authors.forename: Karl-Walter
authors.initials: KW
authors.lastname: Sykora
authors.forename: Christian
authors.initials: C
authors.lastname: Hartmann
authors.forename: Elvis J
authors.initials: EJ
authors.lastname: Hermann
authors.forename: Joachim K
authors.initials: JK
authors.lastname: Krauss
mesh_terms: D002148:Calmodulin-Binding Proteins / Q000378:metabolism; D002648:Child; D003392:Cranial Sinuses / Q000473:pathology*; D003893:Desmin / Q000378:metabolism; D005199:Fanconi Anemia / Q000150:complications* / Q000601:surgery*; D005260:Female; D005682:Gadolinium; D006801:Humans; D007890:Leiomyosarcoma / Q000150:complications* / Q000601:surgery*; D008279:Magnetic Resonance Imaging; D033581:Stem Cell Transplantation / Q000379:methods*
chemical_list: D002148:Calmodulin-Binding Proteins; D003893:Desmin; D005682:Gadolinium
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