title: Primary cerebral histiocytic sarcoma in childhood: a case report of protracted survival and review of the literature.
abstract: BACKGROUND
  Histiocytic sarcoma (HS) of the central nervous system (CNS) is exceptionally rare in pediatric patients, historically associated with an exceptionally poor prognosis. Here, the authors present a novel case of protracted progression-free survival following surgical excision, radiotherapy and temozolomide.
  
  
  METHODS
  A 15-year-old Caucasian girl presented with a two-month history of headache, diplopia, vomiting, lethargy, weight loss and neurocognitive deterioration without gross neurological deficit on physical examination. Magnetic resonance imaging (MRI) of the brain identified a 5.8 × 4.7 × 4.0 cm lesion in the right frontal lobe with associated mass effect and no dissemination. Following two surgical procedures, gross total resection was achieved. Histology and immunohistochemistry confirmed HS, with strong CD163 staining. After focal radiotherapy with concomitant temozolomide, and a further seven cycles of temozolomide, the patient made an excellent recovery and is recurrence free without neurological deficit, 23 months following presentation.
  
  
  CONCLUSIONS
  To the authors' knowledge, this is the first incidence of a prolonged, functionally preserved and recurrence-free outcome following a diagnosis of HS within the CNS of a pediatric patient. We suggest early diagnosis prior to dissemination and complete surgical resection as an essential treatment goal in this rare disease.
authors.affiliation: Salford Royal Hospital, Stott Lane, M6 8HD, Salford, England, UK.
authors.forename: Mitchell
authors.initials: M
authors.lastname: Foster
authors.affiliation: Children's Brain Tumour Research Network, Royal Manchester Children's Hospital, Oxford Road, M13 9WL, Manchester, England, UK.|The Centre for Paediatric, Teenage and Young Adult Cancer, Institute of Cancer Sciences, University of Manchester, Wilmslow Road, M20 4QL, Manchester, England, UK.
authors.forename: Ian
authors.initials: I
authors.lastname: Kamaly-Asl
authors.affiliation: Children's Brain Tumour Research Network, Royal Manchester Children's Hospital, Oxford Road, M13 9WL, Manchester, England, UK.|Academic Unit of Paediatric Radiology, Manchester Academic Health Science Centre, University of Manchester, M13 9WL, Manchester, England, UK.
authors.forename: Stavros
authors.initials: S
authors.lastname: Stivaros
authors.affiliation: Department of Histopathology Children's Brain Tumour Research Network, Royal Manchester Children's Hospital, Oxford Road, M13 9WL, Manchester, England, UK.
authors.forename: Anna
authors.initials: A
authors.lastname: Kelsey
authors.affiliation: Department of Clinical Oncology, Christie Hospital, Wilmslow Rd, Withington, England, UK.
authors.forename: Rao
authors.initials: R
authors.lastname: Gattamenini
authors.affiliation: Children's Brain Tumour Research Network, Royal Manchester Children's Hospital, Oxford Road, M13 9WL, Manchester, England, UK. John-Paul.Kilday@cmft.nhs.uk.|The Centre for Paediatric, Teenage and Young Adult Cancer, Institute of Cancer Sciences, University of Manchester, Wilmslow Road, M20 4QL, Manchester, England, UK. John-Paul.Kilday@cmft.nhs.uk.
authors.forename: John-Paul
authors.initials: JP
authors.lastname: Kilday
mesh_terms: D000293:Adolescent; D015703:Antigens, CD / Q000378:metabolism; D015214:Antigens, Differentiation, Myelomonocytic / Q000378:metabolism; D001932:Brain Neoplasms / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy; D005260:Female; D054747:Histiocytic Sarcoma / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy; D006801:Humans; D008279:Magnetic Resonance Imaging; D011956:Receptors, Cell Surface / Q000378:metabolism; D000098902:CD163 Antigen
chemical_list: D015703:Antigens, CD; D015214:Antigens, Differentiation, Myelomonocytic; D000098902:CD163 Antigen; D011956:Receptors, Cell Surface
keywords: Central nervous system; Histiocytic sarcoma; Neuro-oncology; Pediatric; Radiotherapy; Temozolomide