title: Uveal melanoma in young patients.
abstract: OBJECTIVE
  To study the clinical profile of young patients with uveal melanoma.
  
  
  METHODS
  Retrospective case-control series.
  
  
  METHODS
  Tertiary referral center.
  
  
  METHODS
  Data on 63 patients aged 20 years or younger with uveal melanoma were reviewed for clinical profile and association with oculo(dermal) melanocytosis, familial uveal melanoma, dysplastic nevus syndrome, cutaneous melanoma, and other second malignant neoplasms.
  
  
  RESULTS
  Of 8000 patients with uveal melanoma, 63 (0.8%) were found in patients who were 20 years of age or younger. The median age at diagnosis was 16 years, and the youngest patient was 3 years old. Sixty-two patients (98%) were white, and uveal melanoma was unilateral in all cases. Seven patients (11%) had oculo(dermal) melanocytosis. Two patients (3%) had dysplastic nevi syndrome, and personal history of cutaneous melanoma was observed in 1 patient (2%). No other second cancers were present in any patient. The 5- and 15-year posttreatment survival estimates were 0.95 (95% confidence interval, 0.87-1.00) and 0.77 (95% confidence interval, 0.52-1.00), respectively.
  
  
  CONCLUSIONS
  Uveal melanoma is rare in children or teenagers. It occurs in a heterogeneous group displaying various associations, especially with oculo(dermal) melanocytosis. Oculo(dermal) melanocytosis is 9 times (95% confidence interval, 3.6-22.8) more common in young patients with uveal melanoma than in the general population with uveal melanoma. Young patients with uveal melanoma have short-term (5-year) survival better than that of adults, but the long-term (15-year) survival is similar to that of adults. Arch Ophthalmol. 2000;118:918-923
authors.affiliation: Oncology Service, Wills Eye Hospital, 900 Walnut St, Philadelphia, PA 19107, USA. arunsingh@eyetumors.com
authors.forename: A D
authors.initials: AD
authors.lastname: Singh
authors.forename: C L
authors.initials: CL
authors.lastname: Shields
authors.forename: J A
authors.initials: JA
authors.lastname: Shields
authors.forename: T
authors.initials: T
authors.lastname: Sato
mesh_terms: D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D002648:Child; D002675:Child, Preschool; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D008297:Male; D008545:Melanoma / Q000235:genetics / Q000401:mortality / Q000473:pathology*; D010375:Pedigree; D010414:Pennsylvania / Q000453:epidemiology; D012189:Retrospective Studies; D015996:Survival Rate; D014604:Uveal Neoplasms / Q000235:genetics / Q000401:mortality / Q000473:pathology*