title: Head and neck manifestation and prognosis of Langerhans' cell histiocytosis in children.
abstract: OBJECTIVE
  To appreciate the several head and neck manifestations of Langherans' cell histiocytosis (LCH) in children and their multidisciplinary management and outcome.
  
  
  METHODS
  Retrospective study.
  
  
  METHODS
  Clinical reports of 42 patients with LCH treated in the Departments of Paediatric Haematology, Paediatric Oncology and Paediatric Otorhinolaryngology of a tertiary care center were analyzed. Only cases where the disease was localized to the head and neck were considered. The age at diagnosis, gender, clinical presentation, extension of disease as well as response to treatment and outcome were recorded from the charts of each of these patients.
  
  
  RESULTS
  Of the 42 patient charts reviewed, 31 (73.8%) presented with head and neck localization. 10 of these had an exclusive head and neck presentation. Multisystem LCH was mostly found in infants under 3-year-old (mean age: 2-year-old), and bony manifestations in older. All treatments delivered to patients were well-tolerated and the evolution good.
  
  
  CONCLUSIONS
  Head and neck involvement is known to be very frequent in LCH. There is no consensus about treatment but authors highlight that all teams in charge of patients presenting with LCH agree to remain as conservative as possible. For solitary large lesions looking like a tumor which resection could result in functional or cosmetic morbidity, it would be important to get first a biopsy. For multisystemic LHC, therapeutic trials with chemotherapy agents still in process should increase the rate of success.
authors.affiliation: Department of Paediatric Otorhinolaryngology Head and Neck Surgery, La Timone Children's Hospital, 264 rue St Pierre, 13385 Marseille cedex 5, France. richard.nicollas@ap-hm.fr
authors.forename: R
authors.initials: R
authors.lastname: Nicollas
authors.forename: A
authors.initials: A
authors.lastname: Rome
authors.forename: H
authors.initials: H
authors.lastname: Belaïch
authors.forename: S
authors.initials: S
authors.lastname: Roman
authors.forename: M
authors.initials: M
authors.lastname: Volk
authors.forename: J C
authors.initials: JC
authors.lastname: Gentet
authors.forename: G
authors.initials: G
authors.lastname: Michel
authors.forename: J M
authors.initials: JM
authors.lastname: Triglia
mesh_terms: D000293:Adolescent; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D000972:Antineoplastic Agents, Phytogenic / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001847:Bone Diseases / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D004359:Drug Therapy, Combination; D005047:Etoposide / Q000627:therapeutic use; D005260:Female; D005624:Frontal Bone / Q000000981:diagnostic imaging*; D006646:Histiocytosis, Langerhans-Cell / Q000000981:diagnostic imaging* / Q000188:drug therapy; D006801:Humans; D007223:Infant; D033183:Interdisciplinary Communication; D008297:Male; D009333:Neck; D009915:Orbit / Q000000981:diagnostic imaging*; D010296:Parietal Lobe / Q000473:pathology*; D010348:Patient Care Team; D011241:Prednisone / Q000627:therapeutic use*; D011379:Prognosis; D012189:Retrospective Studies; D013701:Temporal Bone / Q000000981:diagnostic imaging*; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D014747:Vinblastine / Q000627:therapeutic use*
chemical_list: D000893:Anti-Inflammatory Agents; D000972:Antineoplastic Agents, Phytogenic; D014747:Vinblastine; D005047:Etoposide; D011241:Prednisone