Iris/Ciliary Tumour Overview

Iris Tumours

Small Benign Iris Lesions:

• Freckles: Smaller than naevi and occurs at the surface layer only, not stroma

• Bruschfield Spots: Pale lesions peripheral iris stroma, often patients with Trisomy 21

• Mamillations: Unilateral tiny villiform lesions; uncommon but in patients with congenital ocular melanocytosis, Axenfeld and Peter’s anomaly

• Lisch Nodules: Small bilateral nodules after ~16yrs in most Px with NF1

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Benign Iris Naevus and Melanocytoma:

• Flat (<3mm diameter, <0.5mm thick)

• Mostly pigmented, usually no growth → surface proliferation of melanocyte

• Typically, melanocytomas are benign lesions formed by melanocytes with dense pigments

• May disrupt normal iris structure or distort pupil (ectropion uveae)

Iris and CIliary Body Melanoma:

• Uveal melanomas ~3% (cf. ciliary body 10%, choroid 85%)

• Often asymptomatic and seen around age ~50-60 years.

• Can be diffuse, nodular or sometimes a ring affecting the whole iris

• Usually affects the inferior iris, with prominent feeder vessels

  • Details of the iris are lost, such as the crypts

  • Can also distort the pupil

  • Generally, the prognosis is good with only 5% developing metastasis in 10 years

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Non-melanocytic Iris Tumours:

• Iris vascular tumours like haemangiomas more obvious under OCT-A which reveals tortuous vessels

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• Iris juvenile xanthogranuloma

• Iris lymphoid tumour

• Iris metastasis from breast or remote cancer

Iris Pigment Epithelium Adenoma

• Rare benign tumour, grey-black nodule with smooth surface, often peripherally, causing anterior displacement and stromal thinning

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Ciliary Body Tumours

Ciliary Body Melanoma:

• Rare, ~8-10% of uveal melanomas and can often extend to choroid

• Very aggressive cf. iris

  • Good to look for the scleral sentinal vessels or external tumour

    

  • Asymptomatic until complications arise, such as glaucoma and VF loss

  • Detection is fairly late, and prognosis typically poor due to metastatic risk

Other Ciliary Body Tumours:

• Leiomyoma = very rare smooth muscle tumour, affecting ciliary body, iris and anterior chamber

  • Minimal intra/extraocular extension, low mitotic rate

  • More common in younger women and asymptomatic until it affects nearby structures

  • Blurred vision, floaters and sometimes pain

• Medulloepithelioma = very rare, unilateral embryonic tumour that occurs in first decade