Ocular melanoma

Abstract

Ocular melanomas comprise uveal and conjunctival sub-types, which are very different from each other. A large majority of uveal melanomas involve the choroid, with less than 10% being confined to the ciliary body and iris. They tend to metastasize haematogenously, almost always involving the liver. Therapeutic methods include various forms of radiotherapy, surgical resection and phototherapy, which are often used in combination. Conjunctival melanomas show many similarities to their cutaneous counterparts, often metastasizing by lymphatic spread. Treatment consists of excision of invasive melanoma with adjunctive radiotherapy and/or cryotherapy and topical chemotherapy for intra-epithelial disease. The management of patients with ocular melanomas demands a good understanding of the pathology of these tumours. Pathological examination of the tumour indicates the prognosis and hence the need for further investigation and treatment. The scope of the pathologist is enhanced thanks to advances in molecular biology.

Keywords: Conjunctiva; Eye; Melanoma; Pathology; Uvea.

Figure 1

Histopathology of uveal melanoma (a) haematoxylin and eosin slide showing epithelioid cells^∗; (b) staining for HSP-27, which is associated with a good prognosis; (c) mitoses, stained with Ser-10 (PHH3)^∗; and (d) “closed” loops^∗. ^∗From Damato BE et al., Progress in Retinal and Eye Research (2011).

Figure 2

Fundus photographs of choroidal melanomas showing: (a) a small tumour with overlying lipofuscin pigment; (b) a large, dome-shaped tumour with serous retinal detachment; (c) an amelanotic collar-stud melanoma; and (d) a diffuse melanoma.

Figure 3

Slit-lamp appearances of anterior uveal melanomas, showing: (a) a ciliary body melanoma extending into anterior chamber; (b) a pigmented, nodular iris melanoma; (c) an amelanotic, nodular iris melanoma^∗; and (d) a diffuse iris melanoma with seeding. ^∗From Ocular Tumours, B Damato. Butterworth Heinemann (2000).

Figure 4

Kaplan–Meier survival curves showing survival according to the presence or absence of chromosome 3 loss, determined by multiplex ligation-dependent probe amplification.

Figure 5

Personalised prognostication integrating pathological findings with genetic results and clinical tumour stage. The metastatic risk is estimated by subtracting the all-cause mortality of the general population (upper curve) from that of the patient population (lower curve).

Figure 6

Histological grading of conjunctival melanocytic intra-epithelial neoplasia (C-MIN). From Damato B and Coupland SE. Management of conjunctival melanoma. Expert Rev. Anticancer Ther. 2009;9:1227–39.

Figure 7

Slit-lamp photographs showing: (a) conjunctival melanocytic intraepithelial neoplasia; (b) diffuse, invasive conjunctival melanoma; (c) bulbar invasive conjunctival melanoma; and (d) caruncular invasive conjunctival melanoma. From Damato B and Coupland SE. Management of conjunctival melanoma. Expert Rev. Anticancer Ther. 2009;9:1227–39.