Bi TUMOURS 318 Irismelanoma 318 ‘isnaevi 320 pIRISCYSTS 320 Primary cysts 320 “Secondary cysts 321 CILIARY BODYTUMOURS — 322 Giary body melanoma 322 |CHOROIDALTUMOURS — 323 Choroidal melanoma 323 Choroidal naevus 327 Gircumscribed choroidal haemangioma 329 Difuse choroidal haemangioma 330 Metastatic tumours 330 Choroidal osseous choristoma 332 “Melanocytoma 333 raocular Tumours @ LYMPHOMA = (334 @ RETINAL AND OPTIC NERVE HEAD TUMOURS = 334 Retinoblastoma 334 Retinal astrocytoma 340 Retinal capillary haemangioma 344 Retinal cavernous haemangioma 343 Retinal racemose haemangioma 344 Retinal vascular proliferative tumour 344 © TUMOURS OF THE RETINAL PIGMENT EPITHELIUM — 345 Congenital hypertrophy of the retinal pigment epithelium — 345 ‘Combined hamartoma of the retinal pigment epithelium and retina 347 Hamartoma of the retinal pigment epithelium 347318 Clinical Ophthalmology > Iris tumours Iris melanoma In general, uveal melanomas are three times commoner in patients with blue/grey than brown irides. They are extremely rare in black people and there is no sexual pre- dilection. Conditions associated with or predisposed to early- onset uveal melanomas are: (3) ocular melanacytosis (b) naevus of Ota, (e) dysplastic cutaneous naevi, (d) familial melanoma and (e) neurofibromatosis-1. Iris melanoma makes up about 5% of uveal melanomas. The majority are composed of spindle B cells (see below) and are of low malignancy. A minority contain an epithelioid cell com- ponent and can be aggressive, ‘The tumour usually grows very slowly along the iris surface and may invade the angle and anterior ciliary body. The prognosis is very good and only about 5% of patients develop metastases, Clinical features 1. Presentation is in the fifth to sixth decades, a decade earlier than ciliary body and choroidal melanoma, with enlargement of a pre-existing iris lesion, 2. Signs a. Typical A pigmented or non-pigmented nodule at least 3 mm in diameter and 1 mm thickness located in the inferior half of the iris with a smooth or irregular surface (Fig, 11.1), Surface vascularity is also present and is easier to detect in a non-pigmented (Fig. 11.2) than a highly pigmented tumour, where it may be masked. © Papillary distortion, ectropion uveae and occasionally localized cataract may be seen (Pig. 11.3), * Features associated with a prominent epithelioid cell include prominent vascularity, rapid growth and heterogeneous pigmentation. Fig. 14.1 Pigmented iris melanoma Fig. 14.2 Non-pigmented iris melanoma with prominent surface vessels Fig. 11.3 Iris melanoma causing distortion of the pupil, ectropion weae and a localized lens opacity Fig. 11.4 Tapioca iris melanoma (Courtesy of 8 Damato) |b. Rare variants * Diffusely growing intrastromal melanoma may g rise Lo ipsilateral hyperchromic heterochromia. * "Tapioca melanoma’ is characterized by multiple les (Pig. 11.4), surface nod Treatment 1. Observation of suspicious lesions involves documenta: tion by slit-lamp examination, gonioscopy and photo: graphy, Follow-up should be lifelong because growth may ocur alter several years of apparent inactivity. Initially the patient is reviewed after 3-6 months, then 6-9 months and Tater annually 2. Iridectomy for small tumours with iris reconstruction to reduce postoperative photophobia. L.lridocyclectomy for tumours invading the angl (fig. 11.5), 4.Radiotherapy with local plaques (brachytherapy) or external irradiation with charged particles for non resectable tumours. 5, Enuclea fumours. yn _may be required for diffusely growing ig 115 Opic view of iris melanoma involving the angle 319 Intraocular Tumours Differential diagnosis L tris naevus, if large and distorting the pupil (see Pig, 11.11 2. Ciliary body melanoma with extension through the iris root (see Fig. 11.24} 3. Metastasis to the iris is rare and usually occurs in patients with a known systemic malignancy. Itis a pink or yellow fast-growing mass (Fig. 11.6) which may be assoctated with anterior uveitis and occasionally multiple deposits may also be seen (Fig 11.7). 4, Adenoma of the yment epithelium isa rare benign tumour characterized by a dark grey-black nodule with a smooth but sometimes multinodular surface, most phaema. Small quently in the peripheral iris (Fig, 11.8). The lesion causes anterior displacement an thinning of the iis stroma, which eventually erodes. disclosing the tumour on slit-lamp biomicroscopy 5. Leiomyoma is an extremely rare benign tumour which arises from smooth muscle, The appearance is similar 10 that of an amelanotic melanoma except that it is not rly confined to the inferior hall of the iris (Fig. 11.9) the diagnosis can be established only histologically 6. Primary iris cyst (sv below}. Fig. 11.7 Multiple iris metastases (Courtesy of 8. Damato) Fig. 11.8 ‘Adenoma of the iris pigment epithelium