title: Clinical characteristics and outcome of pediatric patients with stage IV Hodgkin lymphoma.
abstract: OBJECTIVE
  While treatment outcomes for patients with Hodgkin lymphoma (HL) have improved remarkably, patients with disseminated disease still have a poorer outcome. Stage IV HL is often reported with other 'advanced stage' categories, confusing the specific contribution of disease dissemination to the outcome. This single-institution report looks at characteristics and outcomes of this specific category.
  
  
  METHODS
  The medical records of pediatric HL patients (< 14 years) from 1975 through 2003 were retrospectively reviewed and the data analyzed.
  
  
  RESULTS
  Stage IV patients (n = 67) had more poor-risk characteristics than patients in stages I-III (n = 300) (B symptoms 86.6% vs. 19.3%, bulky disease 57.6% vs. 45.5% and mediastinal mass 77.6% vs. 29.7%; P < .001 for all characteristics). The liver was the most common extralymphatic site (in 51.5% of patients with stage IV disease. Stage IV patients received chemotherapy (CT) alone (n = 55) or combined modality therapy (CMT) (n = 12). Fifty-four patients (80.6%) achieved complete remission, 2 (3%) partial remission, 10 (14.9%) had progressive disease and 1 was lost to follow up. Overall survival was 79.4% and event-free survival (EFS) was 63.9% at 5 years. There was a non-significant benefit for CMT (OS = 91.7% v. 77.1%, P = .3; EFS = 70.7% v. 62.7%, P = .3). Ten of 12 relapsed and only 1 of 10 progressive disease patients were salvaged. On multivariate analysis, failure to achieve complete remission with CT was associated with a poorer outcome.
  
  
  CONCLUSIONS
  Stage IV disease is associated with poor risk features and confers a worse outcome than stage I-III disease. Achievement of complete remission with CT is an important prognostic feature. Slow responders may require novel and/or aggressive therapy to achieve complete remission.
authors.affiliation: Department of Pediatric Hematology/Oncology, King Faisal Specialist Hospital & Research Centre, Riyadh, Saudi Arabia. belgaumi@kfshrc.edu.sa
authors.forename: Asim
authors.initials: A
authors.lastname: Belgaumi
authors.forename: Amani A
authors.initials: AA
authors.lastname: Al-Kofide
authors.forename: Yasser
authors.initials: Y
authors.lastname: Khafaga
authors.forename: Nicey
authors.initials: N
authors.lastname: Joseph
authors.forename: Rubina
authors.initials: R
authors.lastname: Jamil-Malik
authors.forename: Khawar S
authors.initials: KS
authors.lastname: Siddiqui
authors.forename: Rajeh S
authors.initials: RS
authors.lastname: Sabbah
mesh_terms: D000293:Adolescent; D000970:Antineoplastic Agents / Q000627:therapeutic use; D002648:Child; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D005260:Female; D006689:Hodgkin Disease / Q000401:mortality / Q000473:pathology* / Q000628:therapy*; D006801:Humans; D008297:Male; D009367:Neoplasm Staging; D011878:Radiotherapy; D012189:Retrospective Studies; D012307:Risk Factors; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents