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Ocular melanocytosis

MedGen UID:
507939
•Concept ID:
C0025210
•
Congenital Abnormality
Synonyms: Melanosis oculi; Melanosis Oculi; Ocular Melanocytosis; Ocular melanosis
SNOMED CT: Ocular melanosis (45795007); Ocular melanocytosis (45795007); Melanosis oculi (45795007)
 
HPO: HP:0025534

Definition

A congenital lesion of the sclera characterized by unilateral patchy but extensive slate-gray or bluish discoloration of the sclera . The conjunctiva are spared. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Ocular melanocytosis

Professional guidelines

PubMed

Iris melanoma: Review of clinical features, risks, management, and outcomes.
Cherkas E, Kalafatis NE, Marous MR, Shields CL
Clin Dermatol 2024 Jan-Feb;42(1):62-70. Epub 2023 Oct 21 doi: 10.1016/j.clindermatol.2023.10.009. PMID: 37865279
Ocular manifestations in phakomatosis pigmentovascularis: Current concepts on pathogenesis, diagnosis, and management.
Abdolrahimzadeh S, Pugi DM, de Paula A, Scuderi G
Surv Ophthalmol 2021 May-Jun;66(3):482-492. Epub 2020 Oct 13 doi: 10.1016/j.survophthal.2020.10.002. PMID: 33058925
Pathophysiology and management of glaucoma associated with phakomatoses.
Thavikulwat AT, Edward DP, AlDarrab A, Vajaranant TS
J Neurosci Res 2019 Jan;97(1):57-69. Epub 2018 Apr 1 doi: 10.1002/jnr.24241. PMID: 29607552

Recent clinical studies

Etiology

Pediatric ocular melanoma: a collaborative multicenter study and meta-analysis.
Masoomian B, Dalvin LA, Riazi-Esfahani H, Ghassemi F, Azizkhani M, Mirghorbani M, Khorrami-Nejad M, Sajjadi Z, Kaliki S, Sagoo MS, Al Harby L, Al-Jamal RT, Kivelä TT, Giblin M, Lim LS, Shields CL
J AAPOS 2023 Dec;27(6):316-324. Epub 2023 Nov 8 doi: 10.1016/j.jaapos.2023.08.021. PMID: 37949393
Uveal melanoma.
Jager MJ, Shields CL, Cebulla CM, Abdel-Rahman MH, Grossniklaus HE, Stern MH, Carvajal RD, Belfort RN, Jia R, Shields JA, Damato BE
Nat Rev Dis Primers 2020 Apr 9;6(1):24. doi: 10.1038/s41572-020-0158-0. PMID: 32273508
Tumors of the conjunctiva and cornea.
Shields CL, Shields JA
Indian J Ophthalmol 2019 Dec;67(12):1930-1948. doi: 10.4103/ijo.IJO_2040_19. PMID: 31755426Free PMC Article
Primary orbital melanoma: a case series and literature review.
Figueira E, Rajak S, McKelvie P, Kalantzis G, Ismail A, Gonzales M, James C, McNab A, Selva D
Orbit 2018 Oct;37(5):352-357. Epub 2018 Feb 1 doi: 10.1080/01676830.2017.1423354. PMID: 29388848
Bilateral uveal melanoma in an arc welder.
Turaka K, Shields CL, Shah CP, Say EA, Shields JA
Graefes Arch Clin Exp Ophthalmol 2011 Jan;249(1):141-4. Epub 2010 Sep 19 doi: 10.1007/s00417-010-1516-5. PMID: 20853114

Diagnosis

Ocular Melanocytosis with Uveal Melanoma.
Begaj T, Capone A Jr
Ophthalmology 2024 Jan;131(1):65. Epub 2023 May 22 doi: 10.1016/j.ophtha.2023.04.017. PMID: 37212765
Ocular Melanocytosis and Peripheral Retinal Avascularity in a Case of Cutis Marmorata Telangiectatica Congenita.
Rosignoli L, Wood EH, Geloneck MM, Diaz LZ, Levy ML, Harper CA
Ophthalmic Surg Lasers Imaging Retina 2022 Nov;53(11):647. Epub 2022 Nov 1 doi: 10.3928/23258160-20221011-01. PMID: 36378608
Uveal melanoma.
Jager MJ, Shields CL, Cebulla CM, Abdel-Rahman MH, Grossniklaus HE, Stern MH, Carvajal RD, Belfort RN, Jia R, Shields JA, Damato BE
Nat Rev Dis Primers 2020 Apr 9;6(1):24. doi: 10.1038/s41572-020-0158-0. PMID: 32273508
Tumors of the conjunctiva and cornea.
Shields CL, Shields JA
Indian J Ophthalmol 2019 Dec;67(12):1930-1948. doi: 10.4103/ijo.IJO_2040_19. PMID: 31755426Free PMC Article
Primary orbital melanoma: a case series and literature review.
Figueira E, Rajak S, McKelvie P, Kalantzis G, Ismail A, Gonzales M, James C, McNab A, Selva D
Orbit 2018 Oct;37(5):352-357. Epub 2018 Feb 1 doi: 10.1080/01676830.2017.1423354. PMID: 29388848

Therapy

Bilateral uveal melanoma in an arc welder.
Turaka K, Shields CL, Shah CP, Say EA, Shields JA
Graefes Arch Clin Exp Ophthalmol 2011 Jan;249(1):141-4. Epub 2010 Sep 19 doi: 10.1007/s00417-010-1516-5. PMID: 20853114
Bilateral primary choroidal melanoma treated with bilateral plaque radiotherapy: a report of three cases.
Furuta M, Shields CL, Uysal Y, Shields JA
Eur J Ophthalmol 2006 Nov-Dec;16(6):879-82. doi: 10.1177/112067210601600619. PMID: 17191200
Two discrete choroidal melanomas in an eye with ocular melanocytosis.
Honavar SG, Shields CL, Singh AD, Demirci H, Rutledge BK, Shields JA, Eagle RC Jr
Surv Ophthalmol 2002 Jan-Feb;47(1):36-41. doi: 10.1016/s0039-6257(01)00281-8. PMID: 11801268
Melanocytic glaucoma in a child associated with ocular melanocytosis.
Kiratli H, Irkeç M
J Pediatr Ophthalmol Strabismus 1997 Nov-Dec;34(6):380-1. doi: 10.3928/0191-3913-19971101-12. PMID: 9430066
Bilateral primary uveal melanoma. Bad luck or bad genes?
Singh AD, Shields CL, Shields JA, De Potter P
Ophthalmology 1996 Feb;103(2):256-62. doi: 10.1016/s0161-6420(96)30707-0. PMID: 8594511

Prognosis

Pediatric ocular melanoma: a collaborative multicenter study and meta-analysis.
Masoomian B, Dalvin LA, Riazi-Esfahani H, Ghassemi F, Azizkhani M, Mirghorbani M, Khorrami-Nejad M, Sajjadi Z, Kaliki S, Sagoo MS, Al Harby L, Al-Jamal RT, Kivelä TT, Giblin M, Lim LS, Shields CL
J AAPOS 2023 Dec;27(6):316-324. Epub 2023 Nov 8 doi: 10.1016/j.jaapos.2023.08.021. PMID: 37949393
Uveal melanoma.
Jager MJ, Shields CL, Cebulla CM, Abdel-Rahman MH, Grossniklaus HE, Stern MH, Carvajal RD, Belfort RN, Jia R, Shields JA, Damato BE
Nat Rev Dis Primers 2020 Apr 9;6(1):24. doi: 10.1038/s41572-020-0158-0. PMID: 32273508
Primary orbital melanoma: a case series and literature review.
Figueira E, Rajak S, McKelvie P, Kalantzis G, Ismail A, Gonzales M, James C, McNab A, Selva D
Orbit 2018 Oct;37(5):352-357. Epub 2018 Feb 1 doi: 10.1080/01676830.2017.1423354. PMID: 29388848
Iridociliary melanoma associated with ocular melanocytosis in a 6-year-old boy.
Malik Rahman A, Augsburger JJ, Corrêa ZM
J AAPOS 2008 Jun;12(3):312-3. Epub 2008 Mar 24 doi: 10.1016/j.jaapos.2008.01.012. PMID: 18359649
Posterior uveal melanoma. The Swedish perspective.
Seregard S
Acta Ophthalmol Scand 1996 Aug;74(4):315-29. doi: 10.1111/j.1600-0420.1996.tb00701.x. PMID: 8883543

Clinical prediction guides

CHOROIDAL MELANOMA IN PHAKOMATOSIS PIGMENTOVASCULARIS WITH OVERLAPPING STURGE-WEBER SYNDROME AND KLIPPEL-TRENAUNAY SYNDROME.
Fry MV, Williams BK Jr, Kim HJ, Di Nicola M
Retin Cases Brief Rep 2023 Mar 1;17(2):130-133. doi: 10.1097/ICB.0000000000001154. PMID: 33907078
CHOROIDAL MELANOMA, SECTOR MELANOCYTOSIS, AND RETINAL PIGMENT EPITHELIAL MICRODETACHMENTS IN BIRT-HOGG-DUBÉ SYNDROME.
Marous CL, Marous MR, Welch RJ, Shields JA, Shields CL
Retin Cases Brief Rep 2019 Summer;13(3):202-206. doi: 10.1097/ICB.0000000000000595. PMID: 28541267
PARANEOPLASTIC VITELLIFORM MACULOPATHY IN THE SETTING OF CHOROIDAL MELANOMA: EVOLUTION OVER ONE YEAR.
Nagiel A, Rootman DB, McCannel TA
Retin Cases Brief Rep 2017 Winter;11 Suppl 1:S7-S10. doi: 10.1097/ICB.0000000000000383. PMID: 27533643
Bilateral uveal melanoma in an arc welder.
Turaka K, Shields CL, Shah CP, Say EA, Shields JA
Graefes Arch Clin Exp Ophthalmol 2011 Jan;249(1):141-4. Epub 2010 Sep 19 doi: 10.1007/s00417-010-1516-5. PMID: 20853114
Genetic aspects of uveal melanoma: a brief review.
Singh AD, Wang MX, Donoso LA, Shields CL, De Potter P, Shields JA
Semin Oncol 1996 Dec;23(6):768-72. PMID: 8970601

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