Ocular tumours

• "Ocular tumour" is an umbrella for tumours of the eyelid, conjunctiva, uvea, retina and orbit. Some are common and locally invasive, others are rare but life-threatening.
• Uveal melanoma is the most common primary intraocular malignancy in adults, but retinoblastoma is the most common malignant intraocular tumour in children. ^[1]
• A pigmented ocular lesion, a new eyelid lump, leukocoria, or progressive proptosis can be the first sign. None of these should be assumed benign.
• Many tumours are treatable when found early, often with eye-preserving therapy, but delay reduces the chance of saving vision or life.
• Because these are rare, most patients are referred to an ocular oncology centre. Your job at first contact is to recognise the pattern and refer correctly.

By the end of this page you should be able to:

1. Explain the main categories of ocular tumour by site and age group.
2. Recognise the presentation of uveal melanoma, conjunctival melanoma, retinoblastoma, eyelid tumours and orbital tumours.
3. Choose appropriate first investigations and know what each result means.
4. Interpret the key staging and prognostic facts and apply them in a referral.
5. Outline treatment options and when eye preservation is and is not appropriate.
6. Describe red-flag features that require urgent specialist referral.

Ocular tumours are neoplasms arising from the ocular adnexa, ocular surface, intraocular tissues or orbit. They are usually classified by anatomical site, which predicts both the tumour type and the patient group:

• Eyelid tumours: basal cell carcinoma, squamous cell carcinoma, sebaceous gland carcinoma, melanoma, Merkel cell carcinoma and benign adnexal tumours. These arise from the skin and its adnexa.
• Conjunctival tumours: melanoma, squamous neoplasia, lymphoma and benign melanocytic or cystic lesions. Melanoma is the most dangerous of these.
• Uveal tumours: melanoma arising from melanocytes in the choroid, ciliary body or iris. This is the commonest primary intraocular malignancy in adults.
• Retinal tumours: retinoblastoma in children, and (rarely in adults) acquired retinal astrocytoma.
• Orbital tumours: lymphoproliferative disease, metastases, rhabdomyosarcoma in children, optic nerve sheath meningioma, lacrimal gland tumours, dermoid cysts and vascular lesions.

A parallel classification by behaviour is also useful: many eyelid and conjunctival tumours are treated locally, while intraocular and orbital malignancies need multidisciplinary oncology care. Uveal melanoma is often treated within eye-preserving radiotherapy programmes, and retinoblastoma within paediatric oncology services.

Uveal melanoma (UM) is the most common primary intraocular malignancy in adults.^[1] Its incidence follows a latitude gradient: at least 8.0 cases per million person-years in Northern and Western Europe and Oceania, between 2.0 and 7.9 in North America, Eastern and Southern Europe, and below 2.0 in South America, Asia and Africa.^[1] White patients are affected far more often than black or Asian patients.^[1] One percent of cases occur in children, and the incidence rises to over 20 per million by age 70.^[1] Between 2 and 5 percent are familial, mainly associated with germline BAP1 mutations.^[1]

Conjunctival melanoma (CoM) represents 2 to 5 percent of all ocular tumours and 5 to 7 percent of ocular melanomas.^[2] Its incidence in adults of European descent is 0.3 to 0.8 per million.^[2] It is much less common in black and Asian patients.^[2] The average age at presentation is 55 to 65 years, with a mean of 57.4 years at histopathological diagnosis, and cases under 20 years are exceedingly rare.^{[2] [3]}

Retinoblastoma (RB) is the most common malignant intraocular tumour in children, with about 8,000 new cases worldwide each year, or roughly 1 in 14,000 to 1 in 20,000 live births.^[3] About 40 percent of cases are heritable, the rest sporadic.^[3] Up to about 90 percent present before 3 years of age.^[3] Over half of cases occur in Asia and about a quarter in Africa, with marked differences in survival.^{[3] [1]}

Eyelid tumours account for about 5 to 10 percent of all skin cancers, and basal cell carcinoma makes up about 90 percent of malignancies in this group.^[5] Squamous cell carcinoma accounts for 5 to 10 percent, sebaceous gland carcinoma about 5 percent or fewer, and melanoma and Merkel cell carcinoma are rare.^[5] In Asian populations, sebaceous gland carcinoma is one of the commonest malignant eyelid tumours.^[5]

Orbital tumours include lymphoproliferative disease, metastases and vascular lesions in adults; dermoid cysts and rhabdomyosarcoma in children.^[4] Rhabdomyosarcoma accounts for about 40 percent of orbital tumours in children aged 5 to 10 years.^[4] Orbital metastases account for 1 to 3 percent of orbital tumours, with breast cancer the commonest source.^[4]

Eye-conserving treatment of childhood eye conditions is usually staged using the AAO Preferred Practice Pattern for pediatric eye evaluations (2023 edition), which lists retinoblastoma as a vision- and life-threatening malignancy detected by an abnormal red reflex.^[6]

• Uveal melanoma arises from melanocytes of the uveal tract. It is usually sporadic. Risk factors include light-coloured irides, oculo-dermal melanocytosis, and familial syndromes including BAP1 tumour predisposition syndrome, neurofibromatosis type 1, Li-Fraumeni syndrome and familial atypical mole and melanoma syndrome.^[1] UV exposure is not a major risk factor, except for iris melanoma.^[1]
• Conjunctival melanoma arises from melanocytes of the conjunctival epithelium. In about 70 percent of cases it arises from conjunctival melanocytic intraepithelial lesions, about 7 percent from pre-existing nevi, and 11 to 26 percent are de novo.^[2] Only about 2 percent of conjunctival nevi become melanoma.^[2] UV exposure is a probable risk factor: about 86 percent of bulbar tumours in sun-exposed sites show a UV mutational signature.^[2] Risk lesions include primary acquired melanosis with atypia, high-grade conjunctival melanocytic intraepithelial lesions and atypical conjunctival naevi.
• Retinoblastoma is caused by mutation of the RB1 gene on chromosome 13q14.2.^[3] Hereditary disease (about 40 percent) results from a germline RB1 mutation and is often bilateral; the remainder arise from somatic mutations in retinal cells. Most germline mutations are de novo, and only 10 to 15 percent of children have a family history.^[3]
• Eyelid tumours share risk factors with skin cancer: cumulative UV exposure, fair skin, increasing age and immunosuppression.^[5] Sebaceous gland carcinoma is more common in older patients and in Asian populations and can be associated with Muir-Torre syndrome.^[5]
• Orbital tumours in children (rhabdomyosarcoma, neuroblastoma metastasis) and adults (metastatic breast or prostate cancer, lymphoma) inherit the risk factors of the primary cancer.^[4]

In uveal melanoma, malignant melanocytes arise within the stroma of the uveal tract. Despite rarely spreading through lymphatics, haematogenous spread is common. Tumour diameter and thickness, ciliary body involvement and extraocular extension predict 10-year metastasis risk, from 5 percent in stage I to 40 percent in stage III.^[1] Up to 50 percent of patients eventually develop metastases, 90 percent of them in the liver.^[1]

In conjunctival melanoma, melanocytes of the basal conjunctival epithelium transform, with extension through lymphatics to regional nodes. Metastasis occurs in 20 to 30 percent of cases, regional nodal metastasis first in 45 to 60 percent, and overall mortality is about 30 percent.^[2]

In retinoblastoma, mutation of both RB1 alleles removes the cell-cycle brake in retinoblasts. Hereditary disease presents early and often bilaterally; metastatic disease occurs in 10 to 15 percent of cases, usually with post-laminar optic nerve or deep choroidal invasion, and mortality differs greatly between high- and low-resource settings.^[3]

In orbital disease, space-occupying lesions displace the globe producing proptosis, while infiltrative tumours may cause pain, ophthalmoplegia or optic neuropathy. Breast and prostate metastases, lymphoma and rhabdomyosarcoma all behave in these ways.^[4]

• Uveal melanoma: composed of spindle and epithelioid cells; epithelioid type, high mitotic count, ciliary body invasion, extravascular matrix patterns and extraocular extension carry poor prognosis. Choroid 90 percent, ciliary body 6 percent, iris 4 percent of cases.^[1]
• Conjunctival melanoma: variable flat, nodular or diffuse architecture with nevoid, epithelioid and spindle cells, with immunohistochemistry supporting diagnosis. Key mutations include BRAF (about one third of cases, V600E in most), NRAS (about 20 percent), KIT, NF1, PTEN, ATRX (20 to 60 percent) and TERT promoter (35 to 40 percent).^[2]
• Eyelid basal cell carcinoma: nodular (about 54 percent), infiltrative (about 15 percent), morpheaform and other subtypes; perineural invasion worsens prognosis.^[5]
• Squamous cell carcinoma, sebaceous gland carcinoma and melanoma of the eyelid show local invasion; SCC and SGC can spread to nodes; Merkel cell carcinoma is a neuroendocrine tumour linked to Merkel cell polyomavirus in about 80 percent of cases.^[5]
• Orbital rhabdomyosarcoma is an embryonal mesenchymal tumour, characteristically rapid, usually arising superiorly. Lacrimal gland adenoid cystic carcinoma accounts for about 29 percent of epithelial lacrimal gland tumours.^[4]
• Metastatic disease to the orbit most often arises from breast, prostate, lung and cutaneous melanoma.^[4]

• Blurred or changed vision, a new dark spot in the visual field or a pigmented patch that is enlarging or changing colour.
• A persistent eyelid lump, scab, ulcer, or one that bleeds or loses lashes.
• Leukocoria, the white pupillary reflex often noticed in a photograph, in a child.
• Strabismus in a young child, particularly if new.
• Eye redness, swelling or a sensation of pressure.
• Progressive, often painless proptosis, sometimes noticed first by a partner.
• Pain, double vision or visual loss from an orbital mass.

• Uveal melanoma: a pigmented or amelanotic dome- or mushroom-shaped choroidal mass; may show orange lipofuscin, subretinal fluid or retinal detachment. Thirteen to 30 percent are found on routine examination without symptoms.^[1]
• Conjunctival melanoma: a pigmented, nodular or flat lesion, most often on the bulbar conjunctiva (56 to 79 percent), but also in the fornices or palpebrae (9 to 29 percent) or caruncle (1 to 7 percent); may be amelanotic.^[2]
• Retinoblastoma: leukocoria in 60 percent of cases, strabismus, a red or congested eye, proptosis in advanced disease. Infants under 3 months may need examination under anaesthesia.^[3]
• Eyelid basal cell carcinoma: pearly papule with telangiectasia, often lower lid or medial canthus, locally invasive.^[5]
• Orbital lesions: proptosis, ophthalmoplegia, lid swelling, globe displacement, optic disc swelling or atrophy. Idiopathic orbital inflammation is painful and acute; lymphoma is slowly progressive and painless.^[4]

When you are the first clinician to see a suspected ocular tumour, four questions matter:

1. What is the age group? Eyelid and conjunctival tumours are adult diseases; retinoblastoma is a disease of young children; both benign and malignant orbital tumours occur at all ages.
2. Is vision threatened or is there a red flag? An abnormal red reflex, leukocoria or new strabismus in a child, proptosis with pain or rapid progression, an orbital mass with optic neuropathy or an ulcerated eyelid tumour all require prompt referral.
3. Has imaging already been done? Fundus photography, ultrasonography and optical coherence tomography support diagnosis of intraocular tumours; MRI is preferred for orbital soft-tissue disease. CT has a role for bone, calcification and trauma.^[4]
4. Where does this patient need to go? Ideally to an ocular oncology unit with ophthalmology, radiology, pathology and oncology input.

The table lists features that separate common pigmented or ocular lesions.

For uveal melanoma: diagnosis is clinical. Ophthalmoscopy, wide-field fundus photography and conventional ocular ultrasound are used, with high-frequency ultrasound biomicroscopy for suspected ciliary body involvement, and MRI for extraocular extension and orbit assessment.^[1] Biopsy is not mandatory for a typical lesion (grade II, D), but is recommended when the diagnosis is uncertain or for prognostication with fine-needle aspiration.^[1] Blood testing for HLA-A*02:01 is mandatory once metastatic disease exists, to establish eligibility for tebentafusp.^[1] Patients should be screened for distant metastases with liver ultrasound or MRI with diffusion-weighted sequences, plus CT or PET-CT for extrahepatic disease.^[1]

For conjunctival tumours: diagnosis rests on clinical appearance, slit-lamp photography and histopathology after excision or biopsy. Excisional biopsy is preferred where possible. BRAF, NRAS, KIT and TERT testing may guide adjuvant therapy in advanced disease.^[2]

For eyelid tumours: histopathological diagnosis is required before treatment. Imaging (MRI, CT) defines size, depth, bone or lacrimal system involvement and perineural spread. Enlarged or suspicious nodes are imaged by ultrasound or fine-needle aspiration cytology.^[5]

For orbital tumours: MRI is preferred for soft tissue and malignant disease; CT for bone, calcification, trauma and patients unable to have MRI; ultrasound for superficial anterior lesions; PET-CT for systemic staging of lymphoma and metastases.^[4] In a large series, vasculogenic lesions were the most common orbital problem (17 percent), followed by lymphoid and lacrimal gland lesions.^[4]

For retinoblastoma: diagnosis is by indirect ophthalmoscopy, usually under anaesthesia in young children; ultrasound shows characteristic calcification; MRI of the orbits and brain assesses optic nerve, extraocular and intracranial disease and trilateral disease. Molecular RB1 testing defines heritable versus sporadic disease and guides family screening.^[3]

Uveal melanoma is staged by the eighth edition of the AJCC Cancer Staging Manual. Stage predicts 10-year metastasis risk: about 5 percent in stage I and up to 40 percent in stage III.^[1] Three risk groups are defined on molecular grounds: low (normal chromosomes 3 and 8, gene expression profiling class 1, no PRAME expression), intermediate (SF3B1 mutation, late metastasis) and high (monosomy 3, 8q gain, BAP1 loss, class 2).^[1] The Liverpool Uveal Melanoma Prognosticator Online is a widely used prognostic tool.^{[1] [2]}

Conjunctival melanoma is staged with the eighth edition AJCC TNM system for conjunctiva. T categories, nodal involvement and M status correlate with recurrence and mortality.^[2]

Retinoblastoma is staged clinically under anaesthesia using the International Intraocular Retinoblastoma Classification (groups A to E) and the TNM system, and with the International Retinoblastoma Staging System for extraocular disease. Its key morphological high-risk features for metastasis include post-laminar optic nerve invasion, massive choroidal invasion and extraocular spread.^[3]

Orbital disease uses imaging-based TNM or AJCC systems for specific tumour types, for example lacrimal gland adenoid cystic carcinoma, which may be locally advanced at presentation.^[4]

Clinical and pathological staging of all these lesions should be done in an ocular oncology centre rather than by an independent generalist.

Local treatment aims for tumour control and, where possible, eye and vision preservation. Most uveal melanomas are localised at diagnosis (under 2 percent present with detectable metastases), yet up to 50 percent of patients develop metastases.^[1]

• Radiotherapy is the preferred eye-preserving treatment: plaque brachytherapy or proton beam radiotherapy for most tumours.^[1] A tumour under 2.5 mm thick with largest diameter between 5 and 18 mm is suitable for brachytherapy or proton beam therapy.^[1]
• Plaque brachytherapy has a median 5-year local control rate of 91 percent (ruthenium-106 86 percent, iodine-125 93 percent).^[1]
• Proton beam radiotherapy achieves a 5-year local control rate of 94 percent; recommended dose 60 Gy in four fractions or 50 to 70 Gy in five fractions.^[1]
• Stereotactic radiotherapy is an alternative where brachytherapy or proton therapy is not available (5-year local control about 90 percent fractionated, 89 percent radiosurgery).^[1]
• Enucleation is limited to large tumours, those with extensive extraocular extension (over 5 mm), or blind or painful eyes; about 30 percent of patients ultimately need this.^[1]
• Resection of iris or ciliary body melanoma may be used in selected cases.
• Transpupillary thermotherapy is used for small recurrences or as adjunct.
• Adjuvant therapy: patients with macroscopic extraocular extension over 5 mm may receive adjuvant radiotherapy; those with limited extraocular extension can be observed. No adjuvant systemic treatment has yet shown clear benefit, although trials of tebentafusp and others are underway.^[1]
• Metastatic disease: HLA-A02:01 testing in blood is mandatory in the metastatic setting. Tebentafusp improves outcomes in HLA-A02:01-positive disease; immune checkpoint inhibitors and other systemic therapies have limited activity.^[1]
• Follow-up: ophthalmological review every 6 months for years 2 to 5, then annually; monitor tumour stability, radiotherapy complications and vision. Intravitreal anti-VEGF therapy is used to prevent or treat irradiation-induced neovascularisation and maculopathy.^[1]

Management depends on location and extent.

• Localised disease: wide excision with a 2 to 4 mm tumour-free margin, no-touch technique, with fresh instruments at each stage; alcohol epithelialectomy if the limbus is involved; preserve the Bowman layer. Adjuvant treatment is strongly recommended: double freeze-thaw cryotherapy (recurrence 18 percent versus 52 percent with excision alone), topical mitomycin C 0.04 percent four times daily in cycles of 1 to 3 weeks with 1-week drug-free intervals, interferon alpha-2b 1,000,000 IU per mL four to five times daily for 6 to 12 weeks, or plaque brachytherapy (ruthenium-106, 100 Gy to a depth of 2 mm, recurrence 19 percent at 3 years and 21 percent at 5 years).^{[2] [5]}
• Surgery alone without adjuvant therapy is discouraged because of higher recurrence and mortality.^[2]
• Lymph node assessment: sentinel lymph node biopsy or regional lymph node imaging in at-risk cases is used in some centres.
• Metastatic disease: no standard protocol; treatments are adapted from cutaneous melanoma, including checkpoint inhibitors and targeted therapy. Metastasis occurs in 20 to 30 percent of cases, first to regional nodes in 45 to 60 percent.^[2]
• Follow-up should include slit-lamp photography, and careful inspection of the conjunctiva, lid margins and caruncle for recurrence.

Treatment is individualised by IIRC or TNM stage, heritable status, institutional resources and family wishes. The goals are survival, then globe salvage, then vision.

• Systemic chemotherapy: intravenous vincristine, etoposide and carboplatin for high-risk disease, including bilateral cases, mutation carriers and cases with optic nerve or choroidal invasion.^[3]
• Focal therapies: cryotherapy and laser; brachytherapy for small-to-medium tumours up to 16 mm in greatest dimension, with greatest basal diameter increased by 2 mm as a safety margin.^{[3] [1]}
• Intra-arterial and intravitreal chemotherapy for advanced or refractory disease are established in specialist centres.
• Enucleation remains essential for advanced Group D or E disease and cases resistant to conservative treatment, and provides the best safety margin.^[3]
• External beam radiotherapy is reserved for orbital recurrence or optic nerve involvement.^[3]
• Genetic counselling for families; long-term surveillance for heritable disease and second malignancies.

• Basal cell carcinoma: surgical excision with clear margins; Mohs micrographic surgery or frozen section-controlled excision achieve the lowest recurrence in high-risk, poorly defined or medial canthal tumours (about 2 percent 5-year recurrence, under 3 percent in pooled series).^[5] Recommended margins include at least 2 mm for nodular basal cell carcinoma and 3 mm for infiltrative forms.^[5]
• Squamous cell carcinoma: excision with about 5 mm margins and adjuvant radiotherapy when perineural or vascular invasion exists.^[5]
• Sebaceous gland carcinoma: full-thickness eyelid excision with margins up to 10 mm, conjunctival mapping and often cryotherapy or topical mitomycin C; recurrence about 20 percent within 2 years; imaging when the tumour exceeds 20 mm.^[5]
• Eyelid melanoma: excision with about 5 mm margins, with sentinel lymph node biopsy if Breslow thickness exceeds 0.8 mm; systemic therapy as for cutaneous melanoma in advanced disease.^[5]
• Merkel cell carcinoma: wide excision with radiotherapy; prognosis poor (about 50 percent 5-year survival) but checkpoint inhibitors have improved outcomes.^[5]

• Lymphoma: staged and treated as systemic lymphoma; radiotherapy for localised orbital disease.
• Metastases: treat the primary cancer; radiotherapy and systemic therapy.
• Lacrimal gland adenoid cystic carcinoma: eye-sparing surgery followed by radiotherapy in selected patients; about 87 percent 5-year disease-specific survival with eye-sparing surgery versus about 62 percent with exenteration.^[4]
• Rhabdomyosarcoma in children: systemic chemotherapy plus radiotherapy; surgery as part of combined treatment.
• Vascular and cystic lesions: observe, excise or treat with sclerotherapy or radiotherapy.
• Idiopathic orbital inflammation: corticosteroid therapy and exclusion of lymphoma or mimics.

Presentation is often later in low-resource settings, at least for retinoblastoma, and survival is worse (3 to 5 percent mortality in high-income countries versus about 40 percent elsewhere).^[3] Pragmatic steps include red-reflex and eye examination at routine child visits, point-of-care ultrasound for intraocular tumours, referral networks to ocular oncology centres, oral or intravenous chemotherapy rather than unavailable intra-arterial delivery and enucleation for life-threatening disease rather than attempts at heroic salvage.

• Loss of vision or the eye after treatment, particularly after enucleation, advanced exenteration or radiotherapy maculopathy.
• Metastasis, most commonly to liver in uveal melanoma, to regional nodes or brain, lung, liver, skin and bone in conjunctival melanoma, and to the CNS, bone, bone marrow and liver in retinoblastoma.^[1][2][3]
• Radiotherapy complications: cataract, dry eye, optic neuropathy, maculopathy (seen in up to 66.5 percent within 3 years after proton beam therapy), neovascular glaucoma.^[1]
• Local recurrence: despite treatment, 7.3 percent overall at 5 years after radiotherapy (range 0 to 55.6 percent), depending on treatment and site; 30 to 62 percent in conjunctival melanoma; about 20 percent in sebaceous gland carcinoma.^[1][2][5]
• Second malignancies, especially in children with heritable retinoblastoma.
• Treatment toxicity during chemotherapy and radiotherapy.

• Uveal melanoma: 5-year local recurrence 7.3 percent (0 to 55.6 percent across series). Despite local control, up to 50 percent develop metastases; metastatic survival is poor.^[1]
• Conjunctival melanoma: 5-year survival about 86.5 percent, 10-year survival 41 to 78 percent; metastasis 20 to 30 percent, overall mortality about 30 percent.^[2]
• Retinoblastoma: survival is excellent in high-income countries, with mortality of 3 to 5 percent there versus 40 to 70 percent in many African and Asian settings.^[3]
• Eyelid BCC: excellent, under 3 percent recurrence with margin-controlled surgery.^[5]
• Sebaceous gland carcinoma: recurrence about 20 percent within 2 years, with potential for node spread.^[5]
• Merkel cell carcinoma: about 50 percent 5-year survival, now improving with immunotherapy.^[5]
• Orbital rhabdomyosarcoma: good outcomes when treated promptly with combined therapy.^[4]

• Paediatric vision screening: red reflex testing of newborns and infants, with an absent, white, dull, opacified or asymmetric red reflex as a referral trigger; retinoblastoma is one of the vision- and life-threatening conditions detected.^[6] Incidence in children under 5 is about 0.0011 to 0.0013 percent per year.^[6]
• Fundus examination of children of affected families, from birth, for heritable retinoblastoma.
• UV protection for eyelid and conjunctival tumours: sunglasses, brimmed hats and avoidance of tanning beds. Evidence for UV in uveal melanoma is weak.
• Ocular oncology network and referral guidelines lower the chance of misdiagnosis of retinoblastoma and uveal melanoma.
• Periodic follow-up after treatment to detect recurrence early.

• Children: retinoblastoma dominates. Rule out retinoblastoma in children under 5 with strabismus or leukocoria.^[3]
• Older adults: sebaceous gland carcinoma and Merkel cell carcinoma occur in older people.^[5]
• Pregnancy: early detection still matters; treatment decisions involve obstetric and oncology teams.
• Immunocompromised patients: at higher risk of eyelid SCC and other skin tumours; standard excision with margin control remains central.^[5]
• Families with retinoblastoma: genetic counselling and examination of relatives under anaesthesia when appropriate.^[3]

Coverage of these conditions and service organisation follow national bodies such as the American Academy of Ophthalmology (AAO), the European Society for Medical Oncology (ESMO), the ESMO-EURACAN guideline group and, in the United Kingdom, the Royal College of Ophthalmologists (RCOphth).

Pragmatic care includes: red-reflex examination at routine visits and vaccinations; clinical diagnosis plus ultrasonography of intraocular masses; referral via established networks to ocular oncology centres; oral single-agent or low-cost systemic chemotherapy where intravenous combination protocols are unavailable; and enucleation for life-threatening disease. Ultrasound biomicroscopy, MRI, HLA typing and targeted agents are often not available; treatment plans then rest on accessible radiotherapy and surgery.

• Retinoblastoma management (Paediatrics)
• Cutaneous melanoma (Oncology / Dermatology)
• Orbit and ocular adnexal lymphoma
• Leukocoria (Ophthalmology)
• Proptosis (Neurology / Ophthalmology)
• Radiotherapy principles

• Ocular tumour covers eyelid, conjunctival, uveal, retinal and orbital neoplasms, with different typical patient groups.
• Uveal melanoma is the most common primary intraocular malignancy in adults and spreads haematogenously, mainly to liver.
• The choroid is involved in 90 percent of uveal melanomas, the ciliary body in 6 percent and the iris in 4 percent. ^[1]
• Stage predicts 10-year metastasis risk from 5 percent in stage I to 40 percent in stage III of the eighth AJCC system.
• Conjunctival melanoma incidence is 0.3 to 0.8 per million in adults of European descent; metastasis occurs in 20 to 30 percent of cases.
• Conjunctival melanoma needs wide excision plus adjuvant treatment; recurrence remains high after surgery alone.
• Retinoblastoma is the most common malignant intraocular tumour in children; about 40 percent of cases are heritable, and about 80 percent of those are bilateral. ^[3]
• Leukocoria in a child is retinoblastoma until proven otherwise.
• Eyelid basal cell carcinoma dominates eyelid malignancy and is usually locally invasive with low metastatic risk.
• Margins for eyelid surgery vary by tumour: at least 2 mm for nodular BCC, about 3 mm for infiltrative BCC and about 5 mm for SCC, with up to 10 mm for sebaceous or melanocytic lesions. ^[5]
• Mohs or frozen-section margin control gives low recurrence rates in periocular BCC, often under 3 percent. ^[5]
• Orbital metastases are most often from breast, prostate, lung and cutaneous melanoma; children usually have neuroblastoma.
• Red reflex and routine eye examination are crucial for early detection in children.
• Low-resource care centres on red reflex, ultrasound, referral networks, chemotherapy and, where life-threatening, enucleation.