title: Association of ocular and oculodermal melanocytosis with the rate of uveal melanoma metastasis: analysis of 7872 consecutive eyes.
abstract: OBJECTIVE
  Ocular/oculodermal (oculo[dermal]) melanocytosis is a congenital periocular pigmentary condition that can lead to the development of uveal melanoma, estimated at 1 in 400 affected patients. In this study, patients with melanocytosis who developed uveal melanoma were found to have double the risk for metastasis compared with those without melanocytosis.
  
  
  OBJECTIVE
  To determine the relationship of oculo(dermal) melanocytosis to the prognosis of patients with uveal melanoma.
  
  
  METHODS
  Retrospective chart review of 7872 patients with uveal melanoma treated at the Ocular Oncology Service, Wills Eye Institute, from August 25, 1970, through August 27, 2008.
  
  
  METHODS
  Enucleation, plaque radiotherapy, local resection, or thermotherapy.
  
  
  METHODS
  Metastasis and death.
  
  
  RESULTS
  Of 7872 patients with uveal melanoma, oculo(dermal) melanocytosis was present in 230 (3%). The melanocytosis involved the sclera (92%), iris (17%), choroid (12%), eyelid (8%), and temporal fossa (1%). Eyes with melanoma and oculo(dermal) melanocytosis had a relative risk for metastasis 1.6 times greater compared with those with no melanocytosis (P < .001). Metastasis of uveal melanoma was 2.8 times higher in patients with iris melanocytosis (P < .001), 2.6 times higher with choroidal melanocytosis (P = .02), and 1.9 times higher with scleral melanocytosis (P < .001). By Kaplan-Meier estimates, metastasis in patients with oculo(dermal) melanocytosis vs no melanocytosis was 2% vs 1.8% at 1 year, 27% vs 15% at 5 years, and 48% vs 24% at 10 years (P < .001). By multivariable analysis, the factors predictive of metastasis in patients harboring uveal melanoma associated with oculo(dermal) melanocytosis were increased tumor thickness (P = .001) and the presence of subretinal fluid (P = .05), and the only factor predictive of death was increased tumor thickness (P = .009). CONCLUSIONS AND RELEVANCE Patients with uveal melanoma associated with oculo(dermal) melanocytosis have double the risk for metastasis compared with those with no melanocytosis. All patients with oculo(dermal) melanocytosis should undergo ophthalmic examination and imaging on a twice-yearly basis because this could help with the early detection of melanoma.
authors.affiliation: Ocular Oncology Service, Wills Eye Institute, Thomas Jefferson University, Philadelphia, Pennsylvania 19107, USA. carol.shields@shieldsoncology.com
authors.forename: Carol L
authors.initials: CL
authors.lastname: Shields
authors.forename: Swathi
authors.initials: S
authors.lastname: Kaliki
authors.forename: Michael
authors.initials: M
authors.lastname: Livesey
authors.forename: Brianna
authors.initials: B
authors.lastname: Walker
authors.forename: Robert
authors.initials: R
authors.lastname: Garoon
authors.forename: Marissa
authors.initials: M
authors.lastname: Bucci
authors.forename: Eric
authors.initials: E
authors.lastname: Feinstein
authors.forename: Aline
authors.initials: A
authors.lastname: Pesch
authors.forename: Cristina
authors.initials: C
authors.lastname: Gonzalez
authors.forename: Sara E
authors.initials: SE
authors.lastname: Lally
authors.forename: Arman
authors.initials: A
authors.lastname: Mashayekhi
authors.forename: Jerry A
authors.initials: JA
authors.lastname: Shields
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001918:Brachytherapy; D002471:Cell Transformation, Neoplastic / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D015353:Eye Enucleation; D005134:Eye Neoplasms / Q000473:pathology*; D005260:Female; D006801:Humans; D006979:Hyperthermia, Induced; D008297:Male; D008545:Melanoma / Q000401:mortality / Q000556:secondary* / Q000628:therapy; D008548:Melanosis / Q000473:pathology; D008875:Middle Aged; D009507:Nevus of Ota / Q000473:pathology*; D012189:Retrospective Studies; D012307:Risk Factors; D012878:Skin Neoplasms / Q000473:pathology*; D014604:Uveal Neoplasms / Q000401:mortality / Q000556:secondary* / Q000628:therapy; D055815:Young Adult