Cancer Report

Cancer : Eye: Posterior uveal melanoma
Summary
Cancer Name Eye: Posterior uveal melanoma
Description melanomas derived from the pigmented uveal tract of the eye, which comprises the iris, ciliary body and the choriod choroid melanomas are the commonest (60-70%), and iris melanomas are particulary rare (5-8%)
 
Clinics and Pathology
Epidemiology annual incidence in the USA of 5-7 million population annually, similarities to cutaneous melanoma as the incidence is higher amongst fair skinned pale eyed individuals; median age for presentation 55 yrs, and a slightly higher incidence for males; the aetiology is unclear, but possibly sun exposure is a risk factor
Pathology classified into three categories depending on cell type; spindle cell melanoma has the best prognosis, epithelioid is most likey to spread, whilst mixed cell melanomas have an intermediate behaviour; (of note, the size and position of the tumour also affects the prognosis of individual melanomas); uveal melanomas can invade locally within the eye, and form deposits in other organs, but most commonly the liver
Treatment local resection, enucleation, photocoagulation, external beam irradiation, brachytherapy, and laser therapy
Prognosis spindle cell tumours, and those less than 10 mm in diameter have the best outcome; ciliary body melanomas and those where there is scleral invasion have a worse prognosis
 
Related Genes
Gene Symbol RBL2
Description "This oncosuppressor gene is involved in http://atlasgeneticsoncology.org/Tumors/breastID5018.html, Breast Cancer , http://atlasgeneticsoncology.org/Anomalies/BurkittID2077.html, Burkitt's lymphomas , http://atlasgeneticsoncology.org/Tumors/UvealmelanomID5047.html, Choroidal melanoma , http://atlasgeneticsoncology.org/Tumors/endometrID5045.html, Endometrial Cancer , http://atlasgeneticsoncology.org/Tumors/LungTumOverviewID5030.html, Lung Cancer , Malignant Lymphomas, Mesothelioma, http://atlasgeneticsoncology.org/Tumors/AstrocytID5007.html, Glioblastoma , http://atlasgeneticsoncology.org/Tumors/OvarianTumOverviewID5231.html, Ovarian Cancer , Oral malignant melanoma, Vulvar Carcinoma."
 
Gene Symbol PTENP1
Description From COSMIC:
 
Gene Symbol CDKN2A
Description From COSMIC:
 
Gene Symbol BRAF
Description From COSMIC:
 
Cytogenetics
Cytogenetics Morphological most analysis performed on ciliary body and choroid melanomas; usually relatively simple chromosome alterations, often with diploid karyotypes; the commonest alterations are those of chromosomes 1, 3, 6, 8 and the Y chromosome; monosomy 3 and isochromosome 8q occur in association in ciliary body melanomas, and are both independently predictive of poor prognosis, with the increase in the copy number of 8q also indicative of the disease free interval; chromosome 1 changes have been linked with large aggressive tumours and abnormalities of chromosome 6 are suggested to indicate a better prognosis
Cytogenetics Molecular studies are not as comprehensive as cytogenetic studies; comparative genomic hybridization has mainly confirmed cytogenetic data, but also identified les frequent alterations including those of chromsome 9p; no specific genes implicated, p16, p53, BRCA2 have been investigated but appear to be involved in only a small percentage of tumours; studies suggest that genes on chromosome 3 play a role, and several regions have been implicated; no association between MYCand poor prognosis has been made, and flow cytomeric analysis suggests that DNA index is not a reliable predictor of prognosis
 
Bibliography
Title Abnormalities of chromosomes 3 and 8 in posterior uveal melanoma correlate with prognosis.
Authors Sisley K, Rennie IG, Parsons MA, Jacques R, Hammond DW, Bell SM, Potter AM, Rees RC
Citations Genes Chromosomes Cancer. 1997 May;19(1):22-8
PubMedID 97281683
 
Title Chromosomal gains and losses in uveal melanomas detected by comparative genomic hybridization.
Authors Speicher MR, Prescher G, du Manoir S, Jauch A, Horsthemke B, Bornfeld N, Becher R, Cremer T
Citations Cancer Res. 1994 Jul 15;54(14):3817-23.
PubMedID 94306390
 
Title Acquired homozygosity (isodisomy) of chromosome 3 in uveal melanoma.
Authors White VA, McNeil BK, Horsman DE
Citations Cancer Genet Cytogenet. 1998 Apr 1;102(1):40-5
PubMedID 98191416
 
Title Correlation of cytogenetic abnormalities with the outcome of patients wi h uveal melanoma
Authors White VA, Chambers JD, Courtright PD, Chang WY, Horsman DE
Citations Cancer. 1998 Jul 15;83(2):354-9
PubMedID 98332482
 
Title Clinical applications of chromosome analysis, from fine needle aspiratio biopsies, of posterior uveal melanomas.
Authors Sisley K, Nichols C, Parsons MA, Farr R, Rees RC, Rennie IG
Citations Eye. 1998;12 ( Pt 2):203-7
PubMedID 98348563
 
Title Cytogenetics of iris melanomas: disparity with other uveal tract melanomas.
Authors Sisley K, Brand C, Parsons MA, Maltby E, Rees RC, Rennie IG
Citations Cancer Genet Cytogenet. 1998 Mar;101(2):128-33.
PubMedID 98155718
 
Title Cytogenetic findings in primary uveal melanoma.
Authors Singh AD, Boghosian-Sell L, Wary KK, Shields CL, De Potter P, Donoso LA, Shields JA, Cannizzaro LA
Citations Cancer Genet Cytogenet. 1994 Feb;72(2):109-15.
PubMedID 94192126
 
Title Loss of chromosome 3 alleles and multiplication of chromosome 8 alleles in uveal melanoma.
Authors Horsthemke B, Prescher G, Bornfeld N, Becher R
Citations Genes Chromosomes Cancer 1992 Apr;4(3):217-21
PubMedID 93002698
 
Title Loss of heterozygosity of the thyroid hormone receptor B in posterior uveal melanoma.
Authors Sisley K, Curtis D, Rennie IG, Rees RC
Citations Melanoma Res. 1993 Dec;3(6):457-61.
PubMedID 94214279
 
Title Cytogenetics of twelve cases of uveal melanoma and patterns of nonrandom anomalies and isochromosome formation
Authors Prescher G, Bornfeld N, Friedrichs W, Seeber S, Becher R
Citations Cancer Genet Cytogenet. 1995 Mar;80(1):40-6
PubMedID 95211746
 
Title DNA index and % S phase fraction in posterior uveal melanoma: a 5 year prospective study of fresh tissue using flow cytometry.
Authors Richardson RP, Lawry L, Rees RC, Rennie IG
Citations Eye. 1997;11 ( Pt 5):629-34.
PubMedID 98134649
 
Title Prognostic parameters in uveal melanoma: a review.
Authors Mooy CM, De Jong PT
Citations Surv Ophthalmol. 1996 Nov-Dec;41(3):215-28. Review.
PubMedID 97125149
 
Title The prognostic significance of c-myc oncogene expression in uveal melanoma
Authors Chana JS, Cree IA, Foss AJ, Hungerford JL, Wilson GD
Citations Melanoma Res. 1998 Apr;8(2):139-44
PubMedID 98272422
 
Title Non-random abnormalities of chromosomes 3, 6, and 8 associated with posterior uveal melanoma.
Authors Sisley K, Cottam DW, Rennie IG, Parsons MA, Potter AM, Potter CW, Rees RC
Citations Genes Chromosomes Cancer. 1992 Oct;5(3):197-200
PubMedID 93041424
 
Title Prognostic implications of monosomy 3 in uveal melanoma.
Authors Prescher G, Bornfeld N, Hirche H, Horsthemke B, Jockel KH, Becher R
Citations Lancet. 1996 May 4;347(9010):1222-5
PubMedID 96206419
 
Source and Citation
Source Atlas of Genetics and Cytogenetics in Oncology and Haematology
Citation Sisley K . Eye: Posterior uveal melanoma. Atlas Genet Cytogenet Oncol Haematol. June 1999 .
URL : http://AtlasGeneticsOncology.org/Tumors/UvealmelanomID5047.html
URL http://AtlasGeneticsOncology.org/Tumors/UvealmelanomID5047.html