title: Langerhans cell histiocytosis of skull: a retrospective study of 18 cases.
abstract: BACKGROUND
  The present study presents 18 cases of Chinese patients harboring a Langerhans cell histiocytosis (LCH) of the skull.
  
  
  METHODS
  Eighteen consecutive patients were diagnosed as LCH of the skull and confirmed pathologically between March 2002 and February 2014. In the present study, the patients of LCH without skull involvement were excluded. According to disease extent at diagnosis, the 18 LCH patients with skull involvement were divided into three groups: (I) unifocal-monosystem group, including ten cases with solitary skull lesion; (II) multifocal-monosystem group, including two cases with multiple bone lesions and no extra-skeletal involvement; (III) multisystem group, including six cases with LCH lesions involving both skeletal and extra-skeletal system. In unifocal-monosystem group, excision of the skull lesion was performed in eight of ten cases, a low dosage of local radiotherapy and a purposeful observation was accept by the remaining two cases of this group after biopsy respectively. In multifocal-monosystem group, both of the two cases were received chemotherapy. In multi-system group, all the six cases were managed with systemic chemotherapy, after their diagnoses of LCH were confirmed.
  
  
  RESULTS
  The mean age at the time of diagnosis was 9.4 years. There was a male predominance in this disease male/female ratio was 3.5:1. In our cases, a skull mass with or without tenderness was the most common chief complaint (13 cases, 72.2%), and frontal bone was the most frequent affected locations of skull (6 cases, 33.3%). In unifocal-monosystem group, nine of ten remained free from LCH, the remain one lesion recurred 22 months after his surgical excision. In multifocal-monosystem group, a complete response (CR) was obtained in one of them, and a stable disease (SD) of multiple osseous lesions was obtained in another one. In the multi-system group, a CR in four cases and a partial response (PR) in one case were obtained, and a progressive disease (PD) was observed in the remaining one.
  
  
  CONCLUSIONS
  The unifocal-monosystem of LCH of the skull is a clinicopathological entity with a good outcome, and resection, irradiation or purposeful observation are also can be been utilized as the choice of treatment. For the multifocal bone lesions and multisystem lesions of LCH, chemotherapy is an effective treatment as a systemic therapy. There is no enough publication literature to determine guidelines or indications for managing this disease.
authors.affiliation: Department of Neurosurgery, Sun Yat-sen University Cancer Center, Guangzhou 510060, China.
authors.forename: Xiang-Heng
authors.initials: XH
authors.lastname: Zhang
authors.affiliation: Department of Neurosurgery, Sun Yat-sen University Cancer Center, Guangzhou 510060, China.
authors.forename: Ji
authors.initials: J
authors.lastname: Zhang
authors.affiliation: Department of Neurosurgery, Sun Yat-sen University Cancer Center, Guangzhou 510060, China.
authors.forename: Zheng-He
authors.initials: ZH
authors.lastname: Chen
authors.affiliation: Department of Neurosurgery, Sun Yat-sen University Cancer Center, Guangzhou 510060, China.
authors.forename: Ke
authors.initials: K
authors.lastname: Sai
authors.affiliation: Department of Neurosurgery, Sun Yat-sen University Cancer Center, Guangzhou 510060, China.
authors.forename: Yin-Sheng
authors.initials: YS
authors.lastname: Chen
authors.affiliation: Department of Neurosurgery, Sun Yat-sen University Cancer Center, Guangzhou 510060, China.
authors.forename: Jian
authors.initials: J
authors.lastname: Wang
authors.affiliation: Department of Neurosurgery, Sun Yat-sen University Cancer Center, Guangzhou 510060, China.
authors.forename: Chao
authors.initials: C
authors.lastname: Ke
authors.affiliation: Department of Neurosurgery, Sun Yat-sen University Cancer Center, Guangzhou 510060, China.
authors.forename: Chen-Chen
authors.initials: CC
authors.lastname: Guo
authors.affiliation: Department of Neurosurgery, Sun Yat-sen University Cancer Center, Guangzhou 510060, China.
authors.forename: Zhong-Ping
authors.initials: ZP
authors.lastname: Chen
authors.affiliation: Department of Neurosurgery, Sun Yat-sen University Cancer Center, Guangzhou 510060, China. mouyg@sysucc.org.cn.
authors.forename: Yong-Gao
authors.initials: YG
authors.lastname: Mou
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000970:Antineoplastic Agents / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D002681:China / Q000453:epidemiology; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000188:drug therapy / Q000453:epidemiology* / Q000473:pathology; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012737:Sex Factors; D012886:Skull
chemical_list: D000970:Antineoplastic Agents
keywords: Langerhans cell histiocytosis (LCH); skull; treatment