title: Treatment outcome of rhabdomyosarcoma in Hong Kong Chinese children.
abstract: OBJECTIVE
  To review the treatment outcome of rhabdomyosarcoma in Hong Kong Chinese children.
  
  
  METHODS
  Retrospective review.
  
  
  METHODS
  University teaching hospital, Hong Kong.
  
  
  METHODS
  Consecutive cases of rhabdomyosarcoma diagnosed and treated by the Department of Paediatrics and Adolescent Medicine of Queen Mary Hospital between 1989 and 2005. Each patient was staged and treated according to the Intergroup Rhabdomyosarcoma Study guidelines.
  
  
  METHODS
  Overall and event-free survival rates, and toxicity data.
  
  
  RESULTS
  Of 19 patients (8 males and 11 females), 14 (74%) were younger than 10 years old. The median age at diagnosis was 6 (range, 0.5-17) years. Primary sites of rhabdomyosarcoma included: the head and neck (n=8; 6 classified as cranial parameningeal), genitourinary (3), extremity (3), pelvis (3), and trunk (2). Thirteen (68%) had embryonal and six (32%) had alveolar histology. Two, 2, 9, and 6 were classified as belonging to Intergroup Rhabdomyosarcoma Study groups 1, 2, 3, and 4, respectively. Respective 5-year overall and event-free survival rates of the entire cohort were 49% (95% confidence interval, 26-73%) and 32% (10-55%), with a median follow-up of 3.4 (range, 0.2-16.7) years. In non-metastatic cases (Intergroup Rhabdomyosarcoma Study groups 1-3), the 5-year overall survival rate was 66% (95% confidence interval, 39-93%) and in metastatic cases (group 4) it was 17% (0-46%). The 5-year overall survival rate for patients aged less than 10 years was 60% (95% confidence interval, 33-87%) compared to 20% (0-55%) in those aged 10 years and over. Significant treatment-related toxicities including myelosuppression, infections, peripheral neuropathy, and second cancers were encountered.
  
  
  CONCLUSIONS
  Treatment outcome of rhabdomyosarcoma in this cohort of Chinese children was less favourable than that reported in international studies. Whilst the main reason could have been related to the high proportion of metastatic cases, also non-metastatic cases faired worse. Improved outcomes may be achieved by advances in multidisciplinary (paediatric oncology, pathology, radiotherapy, and surgery) management and supportive care.
authors.affiliation: Department of Paediatrics and Adolescent Medicine, Li Ka Shing Faculty of Medicine, The University of Hong Kong, Queen Mary Hospital, Pokfulam, Hong Kong.
authors.forename: X J
authors.initials: XJ
authors.lastname: Yuan
authors.forename: Godfrey C F
authors.initials: GC
authors.lastname: Chan
authors.forename: S K
authors.initials: SK
authors.lastname: Chan
authors.forename: Tony W H
authors.initials: TW
authors.lastname: Shek
authors.forename: Dora L W
authors.initials: DL
authors.lastname: Kwong
authors.forename: William I
authors.initials: WI
authors.lastname: Wei
authors.forename: S Y
authors.initials: SY
authors.lastname: Ha
authors.forename: Alan K S
authors.initials: AK
authors.lastname: Chiang
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D018450:Disease Progression; D018572:Disease-Free Survival; D005260:Female; D006723:Hong Kong; D006801:Humans; D007223:Infant; D008297:Male; D012189:Retrospective Studies; D012208:Rhabdomyosarcoma / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D012983:Soft Tissue Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D016896:Treatment Outcome