Doctors Revision

Common Eye Tumours: Recognition, Red Flags, Assessment and Emergency Referral

Common eye tumours: recognition, red flags, assessment and emergency referral

Ocular tumours may arise from the eyelid, conjunctiva, uvea, retina, lacrimal gland, orbit or optic nerve. They range from congenital and benign lesions to locally invasive cancer and life-threatening malignancy. For emergency clinicians, the essential tasks are to recognize a suspicious pattern, protect vision and life, avoid harmful procedures, and arrange timely specialist assessment. The appearance alone cannot reliably distinguish every benign lesion from cancer.

Emergency rule: A child with a white pupil (leukocoria), a newly abnormal red reflex, unexplained new squint, or a painful/proptotic eye needs urgent ophthalmic assessment. Treat leukocoria as retinoblastoma until a specialist excludes it. Do not biopsy or aspirate a suspected intraocular tumour. Do not delay referral while waiting for tests that can be arranged by the receiving specialist.

Learning outcomes

After studying this guide, learners should be able to: (1) localize an ocular or orbital mass; (2) identify features that raise concern for malignancy; (3) describe the emergency assessment without causing avoidable harm; (4) distinguish major tumour groups by age and location; (5) recognize leukocoria and suspected retinoblastoma; and (6) communicate an appropriate urgency and referral plan.

Source-slide coverage map

This post expands the complete 57-slide “Tumors of the eye” presentation supplied for this topic. Slides that were image-led are represented through the clinical anatomy, localization, lesion patterns and assessment sections below. Treatment schedules and statistics in older slides are not copied as current prescribing instructions; specialist protocols and current staging supersede them.

Deck slides Topics expanded here
1–6 What ocular tumours are; benign versus malignant; primary versus metastatic; why early recognition matters; six anatomic sites.
7–16 Orbital compartment localization; intraconal and extraconal patterns; dermoid; vascular and nerve-sheath lesions; optic nerve lesions; lymphoma; childhood rhabdomyosarcoma; lacrimal-gland tumours and pleomorphic adenoma.
17–24 Benign and premalignant eyelid lesions; basal-cell, squamous-cell and sebaceous carcinomas; clinical warning signs and specialist treatment principles.
25–27 Conjunctival choristomas and benign lesions; ocular-surface squamous neoplasia; melanocytic lesions and melanoma warning signs.
28–39 Iris, ciliary-body and choroidal tumours; uveal melanoma presentation, examination, imaging, risk assessment and treatment options.
40–41 Primary and secondary retinal tumours; retinoblastoma; retinal vascular hamartomas associated with inherited syndromes; metastatic disease.
42–57 Retinoblastoma epidemiology and heritability; leukocoria; specialist diagnostic pathway; classification concepts; chemotherapy and local treatment options; enucleation; advanced and extraocular disease.

1. Start by locating the lesion

The phrase “eye tumour” is broad. First decide whether the abnormality is on the surface, within the globe, or in the orbital tissues around the globe. Record the site and laterality precisely. A lesion’s position, direction of displacement, speed of growth, effect on vision and associated pain often guide the differential diagnosis more usefully than a long unranked list.

Compartment Examples Clues at first assessment
Eyelid and periocular skin Papilloma, nevus, vascular lesion, basal-cell carcinoma (BCC), squamous-cell carcinoma (SCC), sebaceous carcinoma Visible lesion; ulceration, bleeding, lash loss, lid-margin distortion or recurrence may be concerning.
Conjunctiva and corneal surface Dermoid/lipodermoid, nevus, papilloma, ocular-surface squamous neoplasia (OSSN), melanoma, lymphoma Visible surface mass, pigment change, limbal thickening, feeder vessels, gelatinous or leukoplakic surface.
Uvea (iris, ciliary body, choroid) Nevus, cyst, hemangioma, melanocytoma, melanoma; ciliary-body medulloepithelioma in children May be hidden inside the globe; possible abnormal pupil, visible iris mass, visual symptoms, cataract, secondary glaucoma or retinal detachment.
Retina Retinoblastoma, astrocytic hamartoma, retinal capillary haemangioblastoma, metastasis Leukocoria, strabismus, reduced vision or a fundal lesion; symptoms may be absent early.
Orbit and optic nerve Dermoid, vascular malformation, nerve-sheath tumour, lymphoma, rhabdomyosarcoma, metastasis, optic-nerve glioma/meningioma Proptosis, globe displacement, diplopia, restricted motility, pain, colour/vision loss or optic-nerve signs.
Lacrimal gland Pleomorphic adenoma, adenoid cystic carcinoma and other epithelial or secondary tumours Upper outer orbital fullness, superomedial globe displacement, palpable mass; pain or rapid progression increases concern.

Intraconal versus extraconal: The intraconal compartment lies within the cone formed by the extraocular muscles and contains the optic nerve and major vessels. Extraconal lesions arise outside that muscle cone, including in the lacrimal fossa and orbital rim. This distinction helps radiologists and ophthalmologists localize disease. Emergency clinicians should not try to assign a compartment by palpation alone: document the direction of globe displacement, motility and visual function, then use specialist imaging when indicated.

A slowly growing painless mass is more often benign than a rapidly progressive painful mass, but this is only a pattern, not a rule. A lesion that is fixed, recurrent, ulcerated, bleeding, associated with tissue destruction, reduced vision, proptosis, lymph nodes or systemic symptoms requires expedited investigation.

2. Emergency assessment and first decisions

Stabilize first

Use the usual ABC approach if the patient is unwell, has major trauma, severe infection or possible intracranial extension. In a child with a suspected tumour, provide calm, non-stigmatizing care and keep the caregiver with the child when possible. If painful proptosis and visual loss are acute, consider immediately vision-threatening orbital pressure or infection as well as malignancy; these are time-critical ophthalmic emergencies.

Focused history

  • Time course: first noticed, speed of growth, intermittent versus continuous change, recurrent lesion after treatment, and any prior “chalazion,” stye, conjunctivitis or trauma at the same site.
  • Visual symptoms: reduced or blurred vision, field defect, flashes, floaters, new squint, diplopia, photophobia, colour desaturation or transient obscurations. Ask about a white pupil noticed in person or in photographs.
  • Pain and orbital symptoms: spontaneous or movement-related pain, headache, numbness, fever, sinus symptoms, proptosis and restricted eye movement.
  • Systemic history: known cancer (especially breast, lung, skin melanoma, renal, gastrointestinal or haematological cancer), weight loss, night sweats, bone pain or other masses. An ocular lesion can be the first sign of metastatic or haematological disease.
  • Risk context: immunosuppression or HIV, long-term ultraviolet exposure, prior irradiation, chronic scars or wounds, family history of retinoblastoma, bilateral or multifocal childhood disease, and known genetic syndromes.

Examination: record function before appearance

  1. Measure visual acuity separately in each eye using an age-appropriate chart or fixation/behavioural response in a young child. Document whether pinhole improves acuity when appropriate. Do not assume that a child who appears comfortable can see normally.
  2. Inspect both eyelids, lashes, lid margins, conjunctiva, cornea, pupils, red reflexes, globe position and facial symmetry. Compare the fellow eye. Photograph only with consent and according to local policy.
  3. Check pupils and relative afferent pupillary defect, extraocular movements, diplopia, proptosis and pain on movement. Assess colour vision if the patient can cooperate.
  4. Examine the surface with a light or slit lamp if available. Fluorescein may help with a suspected epithelial defect, but it does not establish tumour histology.
  5. Assess the anterior chamber and red reflex, and perform fundoscopy when safe and feasible. A normal undilated direct examination does not exclude a posterior lesion; arrange a comprehensive dilated examination by an eye specialist.
  6. Palpate preauricular, submandibular and cervical nodes for suspicious eyelid or conjunctival cancers; check the orbit gently. Do not press a suspected open globe, painful proptosis or intraocular mass.

Investigations belong to the localization question

Do not order a generic “eye tumour panel.” Imaging is selected according to the suspected compartment and the receiving specialist’s question.

  • Ocular ultrasonography: commonly used by ophthalmology to characterize an intraocular mass, measure dimensions and assess associated retinal detachment. It should be performed by a clinician trained in ocular imaging; avoid pressure if open-globe injury is possible.
  • MRI of orbits and brain: gives soft-tissue detail and may assess optic nerve, orbital, intracranial or extraocular extension. For suspected retinoblastoma, ultrasound and MRI are used in specialist assessment. CT is generally avoided for routine retinoblastoma evaluation when suitable MRI is available because ionizing radiation is undesirable, particularly in children at inherited cancer risk.
  • CT orbit/sinuses: may be appropriate for selected orbital, bony, sinus or acute trauma questions, especially when MRI is unavailable or unsuitable. Choice should be discussed with radiology/ophthalmology if a childhood intraocular malignancy is possible.
  • Biopsy and histology: essential for many surface, eyelid, orbital and lacrimal lesions, but the site and technique are specialist decisions. Suspected retinoblastoma must not be sampled by routine intraocular needle biopsy. Uveal melanoma is also often diagnosed clinically with ocular imaging; biopsy is only used selectively by specialists.
  • Systemic work-up: blood tests, HIV testing with informed consent and counselling, chest or abdominal imaging, and investigations for a primary cancer are guided by the clinical picture and specialist team. Do not delay urgent eye referral to complete broad tests.

3. Orbital tumours

Orbital tumours are classified by tissue of origin, age, location and whether they are primary, secondary by direct extension, or metastatic. Important groups include developmental/dermoid lesions; vascular lesions; peripheral nerve and optic-nerve tumours; lymphoproliferative lesions; rhabdomyosarcoma and other mesenchymal tumours; lacrimal-gland lesions; and metastases. A rapidly enlarging mass in a child is not a “wait and see” diagnosis.

Dermoid and epidermoid cysts

Orbital dermoids are developmental choristomas, often presenting in childhood near a bony suture, classically in the superotemporal or superonasal region. They may be painless, slow-growing, and associated with globe displacement. Deep lesions can remodel bone or extend into the orbit. Infection or rupture can provoke marked inflammation. A typical superficial lesion is assessed by ophthalmology; deep, enlarging, atypical or symptomatic lesions may need imaging and planned excision. Do not squeeze or attempt bedside drainage of an orbital mass.

Vascular and nerve-related lesions

Vascular malformations and haemangiomas can cause proptosis, eyelid swelling, visual compromise or amblyopia in children depending on location and effect on the visual axis. Cavernous venous malformation (historically often called cavernous haemangioma) is a common benign adult orbital lesion and may present with slowly progressive, painless axial proptosis. A schwannoma can arise from an orbital cranial nerve. Optic-nerve glioma and optic-nerve-sheath meningioma are important compressive lesions. Optic neuritis is not a tumour; it is an inflammatory optic neuropathy and is a differential diagnosis for painful visual loss, not a neoplasm.

Lymphoma and metastatic disease

Orbital lymphoma may appear as a painless mass, eyelid fullness, proptosis or a salmon-pink conjunctival lesion. It can be localized or part of systemic lymphoma. Metastases may be the first manifestation of a cancer or occur in someone with a known malignancy. Breast and lung cancers are frequent sources of ocular metastases in adults; melanoma and renal, gastrointestinal and haematological cancers are also relevant. Ask about systemic history, but do not exclude metastasis because the patient has no known primary diagnosis.

Orbital rhabdomyosarcoma

Rhabdomyosarcoma is an aggressive childhood soft-tissue cancer and an important cause of rapidly progressive unilateral proptosis. Presentation may include a visible mass, lid swelling, globe displacement, pain or reduced motility over days to weeks. It may arise in the orbit or extend from nearby structures. The child needs urgent imaging and referral to ophthalmology, paediatric oncology and the appropriate head-and-neck team. Fever can suggest infection, but absence of fever or a well-looking child does not rule out cancer.

Orbital red flags

  • Rapidly increasing proptosis or globe displacement, especially in a child.
  • Reduced acuity, colour desaturation, afferent pupillary defect, visual-field loss or a tense orbit.
  • Restricted movements, new diplopia, severe pain, facial numbness or a fixed mass.
  • Bone destruction, sinus symptoms with an orbital mass, lymphadenopathy, unexplained systemic symptoms or a known cancer.

4. Lacrimal-gland tumours

The lacrimal gland lies in the superotemporal orbit. A mass often produces fullness above and lateral to the globe, with inferomedial displacement. Lesions can be epithelial, lymphoid, inflammatory, neural or metastatic. Clinical features guide urgency but cannot replace imaging and tissue diagnosis when indicated.

Pleomorphic adenoma

Pleomorphic adenoma is a benign mixed epithelial tumour and a common benign epithelial lacrimal-gland tumour. A classic pattern is a painless, firm, slowly enlarging superotemporal mass in an adult, with gradual inferomedial globe displacement. Imaging may show a well-defined lesion centred in the lacrimal fossa with smooth bony remodelling. Suspected pleomorphic adenoma requires ophthalmic/orbital specialist planning: incomplete or unplanned biopsy/excision can compromise definitive treatment and may increase recurrence risk. Do not perform a casual office biopsy of a suspected lacrimal epithelial tumour.

Malignant lacrimal tumours

Adenoid cystic carcinoma, carcinoma ex pleomorphic adenoma, adenocarcinoma and metastasis are among the malignant possibilities. Rapid growth, pain, sensory change from perineural involvement, irregular borders, bone destruction or invasion beyond the gland raise concern. Pain is not always present and benign lesions can be uncomfortable, so no single feature rules cancer in or out. Urgent orbital imaging and specialist referral are required. Treatment may involve surgery, radiotherapy and/or systemic therapy planned by an orbital oncology team.

5. Eyelid tumours

Common benign eyelid lesions include papilloma, nevus, cysts, vascular lesions, haemangioma, neurofibroma and sebaceous adenoma. Actinic (solar) keratosis, carcinoma in situ and inherited disorders such as xeroderma pigmentosum are risk contexts for malignancy. A small, stable, symmetric lesion with preserved lid architecture is more reassuring than an enlarging, destructive or recurrent one, but persistent uncertainty needs assessment.

Basal-cell carcinoma

BCC is a common periocular malignancy, often involving the lower lid or medial canthus after cumulative ultraviolet exposure. Typical features are a pearly or translucent papule with rolled edge, fine surface vessels, central ulceration or bleeding. It may slowly destroy local tissue and distort the lid while metastasis is uncommon. Medial canthal lesions can extend along tissues or into the orbit, so “slow-growing” does not mean harmless. Loss of lashes, a non-healing wound, recurrent bleeding or a lesion that erodes the lid margin is suspicious.

Squamous-cell carcinoma

Eyelid SCC may present as a scaly, indurated, crusted or ulcerated lesion with an irregular edge. It may be more locally invasive and has greater metastatic potential than BCC. Ultraviolet exposure, immunosuppression, chronic wounds/scars and previous radiation are relevant risks. Check for regional nodes and document any sensory symptoms, rapid growth or fixation. Specialist excision with margin assessment is a common treatment principle; suspected orbital or nodal spread needs coordinated cancer care.

Sebaceous carcinoma

Sebaceous carcinoma often arises from meibomian glands, commonly in the upper lid. It is a classic masquerader: a persistent or recurrent “chalazion,” a painless lid nodule, diffuse thickening, loss of lashes or chronic unilateral blepharoconjunctivitis that fails usual treatment should prompt reconsideration. Pagetoid spread across conjunctival epithelium can make the apparent surface disease broader than the visible nodule. Older adults are at increased risk, but age alone cannot exclude it. Refer suspicious lesions for specialist biopsy and histopathology; repeated incision and curettage without reassessment can delay diagnosis.

Practical eyelid lesion comparison

Pattern Features to look for Emergency-clinician action
Possible BCC Pearly rolled edge, ulcer/bleeding, telangiectasia, lower lid/medial canthus, lash loss Document size, site and lid-margin involvement; arrange expedited ophthalmology/oculoplastics assessment.
Possible SCC Scaly, crusted, indurated or ulcerated lesion; rapid enlargement; immunosuppression; nodes Urgent specialist review; assess regional nodes and visual/orbital function.
Possible sebaceous carcinoma Recurrent “chalazion,” upper-lid thickening, unilateral chronic blepharoconjunctivitis, madarosis Do not keep treating as a routine stye; refer for specialist examination and tissue diagnosis.
Likely benign nevus/papilloma Stable, symmetric, sharply defined, preserved lid architecture Record baseline and safety-net change; refer if atypical, changing, symptomatic or diagnostically uncertain.

Definitive treatment varies with histology, size and location. Excision with histological margin control or Mohs surgery may be selected for some eyelid skin cancers; reconstruction, radiotherapy or other oncology treatments are specialist decisions. Do not apply cautery, cryotherapy or topical cancer medicines to an undiagnosed periocular lesion in emergency care.

6. Conjunctival and ocular-surface tumours

Conjunctival lesions may be non-pigmented or pigmented. Benign and developmental lesions include dermoid/lipodermoid choristomas, papilloma, nevus, fibroma, vascular lesions and granuloma. Precancerous and malignant lesions include conjunctival/corneal intraepithelial neoplasia, invasive SCC (together within the OSSN spectrum), melanoma, basal-cell carcinoma and lymphoma. The limbus, where cornea meets conjunctiva, is a common site for OSSN.

Ocular-surface squamous neoplasia

OSSN describes dysplasia through invasive squamous carcinoma affecting conjunctival and/or corneal epithelium. It may appear as a gelatinous, papilliform, leukoplakic or nodular lesion, often at the limbus, with feeder vessels or an irregular transition from normal to abnormal epithelium. A lesion may resemble pterygium or chronic conjunctivitis. Risk is increased by ultraviolet exposure and immunosuppression; HIV is a major clinical context in East Africa, and OSSN may be the presenting sign of previously undiagnosed HIV. Discuss HIV testing sensitively and with consent according to local guidance. Do not assume that every conjunctival growth in a person with HIV is cancer, or that OSSN occurs only in HIV.

Ophthalmology may use slit-lamp assessment, anterior-segment imaging and biopsy/histopathology to define the lesion. Treatment may include excision with appropriate margin techniques and adjunctive cryotherapy, or specialist-selected topical agents for some epithelial disease. These treatments require ocular-oncology/ophthalmic supervision because toxicity, recurrence and invasion must be monitored.

Pigmented conjunctival lesions and melanoma

A conjunctival nevus is often stable and may contain small cysts; this feature can support a benign diagnosis but is not enough to self-diagnose. New or enlarging pigmentation, raised or nodular areas, prominent feeder vessels, fixation, atypical location or recurrence after excision warrant specialist evaluation. Primary acquired melanosis with atypia can be a precursor to melanoma. Conjunctival melanoma is uncommon but potentially life-threatening; changing pigmentation should not be dismissed as a cosmetic issue.

Conjunctival lymphoma

Lymphoma can form a painless salmon-pink patch or diffuse conjunctival thickening. It may be unilateral or bilateral and can be associated with systemic lymphoma. Tissue diagnosis and systemic assessment are specialist-led. A “pink eye” that is a persistent focal mass rather than diffuse inflammation should prompt review.

7. Uveal tumours: iris, ciliary body and choroid

The uvea is the iris, ciliary body and choroid. Uveal lesions include benign nevi and cysts, vascular lesions, melanocytoma, medulloepithelioma (particularly relevant in childhood ciliary body disease) and malignant melanoma. A lesion may remain asymptomatic until it affects the pupil, lens, retina, macula or intraocular pressure. A normal external eye does not exclude an intraocular tumour.

Iris lesions

An iris nevus may be flat or slightly elevated and stable. A cyst may be translucent. Iris melanoma may cause a new focal pigmented mass, pupil distortion, secondary glaucoma, inflammation or growth documented on serial examinations. New heterochromia or a changing iris lesion needs ophthalmology; do not attempt to biopsy or excise it in an emergency setting.

Ciliary-body tumours

The ciliary body is partly hidden behind the iris, so a tumour can remain occult until it grows. Clues include sentinel episcleral vessels, focal cataract or lens displacement, a shallow anterior chamber, secondary glaucoma, iris changes, or retinal detachment. Ultrasound biomicroscopy or other ocular imaging may be needed. Ciliary-body melanoma is a uveal melanoma; ciliary-body medulloepithelioma is a rare childhood tumour. An unexplained unilateral cataract or glaucoma with an abnormal mass or asymmetric signs deserves careful specialist evaluation.

Choroidal nevus versus melanoma

A choroidal nevus is a benign pigmented lesion found on dilated fundus examination. The ophthalmologist documents its basal dimensions, thickness, location, overlying retinal pigment changes, subretinal fluid and interval growth. Risk features such as increasing thickness, subretinal fluid, orange pigment, proximity to the optic disc, symptoms or documented growth increase concern for melanoma. No single feature is diagnostic. A new photopsia, floaters, field defect or reduced acuity can occur when a lesion causes retinal effects, but many lesions are incidental.

Uveal melanoma

Uveal melanoma arises from melanocytes of the iris, ciliary body or choroid; choroidal location is the most common. It may be pigmented or amelanotic and can be dome-shaped, mushroom-shaped after penetrating Bruch’s membrane, or more diffusely thickened. It may cause blurred vision, field loss, flashes/floaters, exudative retinal detachment, secondary glaucoma, cataract or uveitis; some are found before symptoms. Large or ciliary-body lesions may have prominent episcleral vessels. Metastatic surveillance is part of specialist care, with the liver an important site of spread.

Ocular ultrasound helps characterize and measure intraocular tumours; fundus photography, OCT and angiography may contribute. MRI can assess extraocular extension when indicated. Biopsy is rarely needed simply to make the diagnosis and is used selectively. Management depends on size, site, visual potential, fellow-eye status, patient health and evidence of spread. Specialist options include observation of selected small indeterminate lesions, plaque brachytherapy, charged-particle radiotherapy, selected local resection, or enucleation for large/high-risk disease. Laser or transpupillary thermotherapy has a limited, selected role. These are not interchangeable bedside interventions. Enucleation is removal of the globe, not orbital exenteration; exenteration removes orbital contents and is reserved for exceptional advanced situations.

8. Retinal tumours and syndromic associations

Primary retinal lesions include retinoblastoma, retinal astrocytic hamartoma and retinal capillary haemangioblastoma. Some retinal lesions signal a systemic genetic disorder. A retinal vascular lesion may prompt evaluation for von Hippel–Lindau disease; astrocytic hamartomas can occur with tuberous sclerosis; optic-pathway glioma can occur with neurofibromatosis type 1; and facial capillary malformation with ocular disease can occur in Sturge–Weber syndrome. These associations require specialist assessment and appropriate family counselling. Intraocular metastases can involve the choroid or retina and may appear as one or more pale/yellow masses, especially in a patient with known cancer.

9. Retinoblastoma: the childhood sight- and life-threatening emergency

Retinoblastoma is a malignant retinal tumour of early childhood caused by inactivation of the RB1 tumour-suppressor pathway. It may be heritable or non-heritable. Heritable disease is more likely to be bilateral, multifocal and diagnosed at a younger age, but unilateral disease does not exclude a germline mutation. Children who survive heritable retinoblastoma need specialist genetic counselling and long-term surveillance for additional tumours.

Recognize the presentation

  • Leukocoria: a white pupillary reflex, often noticed in flash photographs or by a caregiver. It is the classic warning sign, but not every child has it and not every white reflex is retinoblastoma.
  • Strabismus: a new squint can occur when central vision is reduced.
  • Other possible signs: reduced vision, red or painful eye, hyphema, pseudohypopyon, buphthalmos/glaucoma, proptosis or orbital cellulitis-like swelling in advanced disease.

Differentials for leukocoria include congenital cataract, persistent fetal vasculature, Coats disease, retinal detachment, retinopathy of prematurity, ocular toxocariasis and other retinal disorders. This differential is a reason for immediate examination, not a reason to reassure or delay.

Assessment and safety

Ask when the reflex or squint was first noticed, whether it appears in multiple photographs, whether one or both eyes are involved, and whether there is family history of retinoblastoma or childhood eye removal. Check each eye’s visual behaviour, pupils, red reflex and external appearance without delaying referral. The definitive examination usually requires a full dilated evaluation of both eyes by an experienced ophthalmologist, often under anaesthesia in young children. Ocular ultrasound and MRI are used to distinguish causes of leukocoria and evaluate extent. MRI is preferred over routine CT when suitable; avoid unnecessary ionizing radiation in children at risk for heritable disease.

Never needle-biopsy a suspected retinoblastoma. Sampling an intraocular tumour can damage the eye and may seed tumour outside the globe. Avoid pressure on a suspicious globe, unplanned intraocular surgery or empiric treatment. Contact ophthalmology/oncology urgently and transfer to the appropriate centre.

Classification and management principles

Specialists group intraocular retinoblastoma by extent and risk to the eye; the International Classification commonly uses groups A–E, from small localized tumours to extensive disease with poor visual potential and high-risk features. The purpose is to select a safe treatment plan, not to assign a group from an emergency-room examination.

Treatment aims to save life first, then the eye and useful vision where possible. A specialist multidisciplinary team may combine systemic, ophthalmic-artery or intravitreal chemotherapy with local laser, cryotherapy, thermotherapy or plaque brachytherapy. Enucleation may be recommended for a large advanced tumour, a blind painful eye, extensive involvement or when safe tumour control and survival take priority. It can be life-saving and does not mean care has failed. Extraocular or metastatic disease requires intensive oncology treatment. Management is individualized to each eye and to whether one or both eyes are involved.

The slide deck includes an older multi-drug chemotherapy schedule and historic radiation discussion. Those doses and regimens are deliberately not reproduced as a prescribing guide: treatment protocols change, vary by stage and resources, and require a paediatric oncology team. The current NCI approach describes risk-adapted options and recommends planning with clinicians experienced in childhood ocular cancer.

10. Metastatic ocular disease

Metastases to the eye may come from breast or lung cancer and, less often, kidney, gastrointestinal, prostate, thyroid, skin melanoma or haematological malignancy. Choroid is a frequent intraocular site because of its vascular supply. Patients may present with reduced vision, field defects, flashes/floaters or exudative retinal detachment. A prior cancer history is important, but absence of one does not exclude an occult primary. Conversely, not every choroidal lesion in a person with cancer is a metastasis; ophthalmology uses lesion appearance, imaging, systemic context and sometimes biopsy to distinguish the possibilities.

11. Emergency triage: how urgently should the patient be seen?

Presentation Action
Child with leukocoria, absent/abnormal red reflex or new unexplained squint Same-day contact with ophthalmology/paediatric eye service and urgent referral. If the child cannot be assessed locally, arrange transfer; do not wait for routine clinic or outpatient imaging.
Rapidly progressive proptosis, reduced vision, afferent pupillary defect, severe pain, restricted motility or suspected optic-nerve compression Immediate emergency ophthalmology assessment; evaluate for orbital compartment syndrome, infection and tumour. Stabilize, image as directed and transfer urgently if specialist care is unavailable.
Suspected intraocular melanoma, retinal mass, new ocular tumour with visual loss, secondary glaucoma or retinal detachment Urgent ophthalmology/ocular-oncology review. Same-day escalation if vision is acutely threatened or the patient is deteriorating.
Suspicious eyelid or conjunctival lesion with rapid growth, ulceration, bleeding, lash loss, fixation, nodes or immunosuppression Expedited specialist assessment; use an urgent cancer pathway where available. Document lesion and arrange biopsy only through the appropriate team.
Slow stable benign-appearing lesion without visual/orbital symptoms Non-emergency ophthalmic review may be reasonable, with baseline description and explicit return precautions. Refer sooner if it changes or uncertainty remains.

12. What to avoid in emergency care

  • Do not dismiss leukocoria as a photograph artifact, cataract or benign finding without urgent specialist examination.
  • Do not biopsy, aspirate or attempt to drain a suspected intraocular mass or orbital tumour at the bedside.
  • Do not repeatedly incise a recurrent “chalazion” or treat persistent unilateral conjunctivitis indefinitely without reconsidering malignancy.
  • Do not reassure based only on absent pain, normal external appearance, young age, or a normal direct ophthalmoscope view.
  • Do not order CT routinely for suspected childhood retinoblastoma if MRI and ocular ultrasound are appropriate and available; discuss imaging with the specialist team.
  • Do not promise that an eye can be saved. Explain that the specialist team balances survival, tumour control, eye preservation and useful vision.

13. Case-based learning

Case 1: the white reflex in a photograph

A caregiver brings a 20-month-old child after noticing a white reflex in one eye in several phone photographs. The child is otherwise well and has no pain. Examination shows an asymmetric red reflex.

Interpretation: Retinoblastoma is a must-not-miss cause of leukocoria. Congenital cataract and other retinal disorders are also possible, but no bedside finding safely excludes cancer.

Action: Contact ophthalmology the same day, arrange urgent specialist evaluation of both eyes, and avoid ocular biopsy or nonessential delay for imaging.

Case 2: the “chalazion” that returns

A 68-year-old patient has had three procedures for a painless upper-lid lump at the same location. The lid is diffusely thickened, lashes are missing over the lesion, and unilateral conjunctival irritation persists.

Interpretation: Sebaceous carcinoma is an important masquerader; BCC, SCC and chronic inflammatory disease remain possible.

Action: Do not repeat routine incision and curettage. Refer for oculoplastic/ophthalmic evaluation and appropriate biopsy with assessment for conjunctival spread.

Case 3: a growing limbal plaque

A 39-year-old outdoor worker with untreated HIV has a slowly enlarging, raised gelatinous lesion at the corneal edge with a feeder vessel. Vision is mildly blurred.

Interpretation: OSSN is a serious possibility; benign pterygium and other surface lesions are in the differential.

Action: Arrange expedited ophthalmology review, document the lesion, assess vision, and offer HIV testing/care respectfully if status is unknown. Do not use topical steroids as a substitute for diagnosis.

Case 4: painful proptosis in a child

An 8-year-old develops rapidly progressive unilateral proptosis over 2 weeks, with limited elevation but no fever. Vision is reduced compared with the other eye.

Interpretation: Orbital rhabdomyosarcoma is a key concern, alongside infection, inflammatory disease and other orbital masses.

Action: Immediate ophthalmology assessment, urgent imaging and referral to paediatric oncology. Absence of fever does not justify watchful waiting.

Case 5: incidental pigmented fundal lesion

A 57-year-old reports new floaters. Dilated examination by the eye service shows an elevated choroidal lesion with surrounding subretinal fluid.

Interpretation: Choroidal melanoma is one possibility, but a nevus and metastatic lesion must be considered in context.

Action: Urgent ocular-oncology evaluation and ultrasound/fundus imaging. Do not attempt biopsy or laser treatment outside specialist care.

14. Quick knowledge check

  1. What should be assumed until excluded in a child with leukocoria?
  2. Name three warning signs in a recurrent eyelid lesion that should prompt reconsideration of “chalazion.”
  3. Which tests are commonly used by specialists to assess suspected retinoblastoma, and what imaging is generally preferred over routine CT when suitable?
  4. Why should a suspected retinoblastoma not be needle-biopsied?
  5. What pattern suggests possible orbital rhabdomyosarcoma in a child?
  6. What is OSSN, and why is HIV relevant in East African clinical practice?
  7. Give two features that increase concern for a choroidal melanoma rather than a stable nevus.
  8. What is the immediate priority when an orbital mass is associated with declining vision and an afferent pupillary defect?

Answers

  1. Retinoblastoma, while urgently investigating other causes of leukocoria.
  2. Repeated recurrence at the same site, upper-lid thickening, lash loss, diffuse unilateral blepharoconjunctivitis, ulceration or progressive growth.
  3. A full dilated specialist eye examination, ocular ultrasonography and MRI as indicated. MRI is generally preferred to routine CT where appropriate and available.
  4. Sampling can injure the eye and may seed malignant cells outside the globe.
  5. Rapidly progressive proptosis, globe displacement or reduced motility/vision developing over days to weeks.
  6. Ocular-surface squamous neoplasia ranges from epithelial dysplasia to invasive SCC; immunosuppression, including HIV, is clinically relevant and OSSN may reveal previously unrecognized HIV.
  7. Documented growth, greater thickness, subretinal fluid, orange pigment, symptoms or proximity to the optic disc; a specialist weighs the complete pattern.
  8. Urgent ophthalmology evaluation to protect vision and assess for optic-nerve compression or other orbital emergency, while arranging appropriate imaging and transfer.

15. Documentation and referral handover

A useful referral states the patient’s age; laterality; exact lesion location; onset and growth rate; visual acuity in each eye; pupil and red-reflex findings; proptosis/motility; pain; relevant cancer, HIV/immunosuppression and family history; tests already completed; and the reason for urgency. Use closed-loop communication: identify the receiving eye service, confirm that the referral was received, and arrange transport or follow-up rather than giving a vague instruction to “see an eye doctor.” For children with possible retinoblastoma, include the caregiver’s observation or photograph history and make clear that the referral is urgent.

Key takeaways

  • Localize the lesion: lid, ocular surface, intraocular, retina, orbit, optic nerve or lacrimal gland.
  • Changing, ulcerated, bleeding, recurrent, fixed or rapidly enlarging lesions need specialist review.
  • Leukocoria in a child is an urgent cancer warning sign; arrange same-day contact with ophthalmology.
  • Do not biopsy a suspected retinoblastoma or perform unplanned procedures on an intraocular/orbital mass.
  • Rapid proptosis plus visual dysfunction is a vision-threatening emergency, regardless of whether the cause is tumour, infection or pressure.
  • Modern diagnosis and cancer treatment are team-based; emergency clinicians recognize, protect, refer and communicate.

References and further reading

  1. Tumors of the eye. Supplied 57-slide teaching presentation on SlideShare.
  2. National Cancer Institute. Retinoblastoma Treatment (PDQ), Health Professional Version.
  3. National Eye Institute. Retinoblastoma.
  4. National Cancer Institute. Intraocular (Uveal) Melanoma Treatment (PDQ), Health Professional Version.
  5. American Academy of Ophthalmology EyeWiki. Ocular Surface Squamous Neoplasia.
  6. American Academy of Ophthalmology EyeWiki. Basal Cell Carcinoma.
  7. American Academy of Ophthalmology. Diagnosis and Management of Sebaceous Carcinoma of the Eyelid.
  8. Clinical presentation and outcomes in children with retinoblastoma managed at the Uganda Cancer Institute.
  9. WHO/PAHO. Primary health care guidance for children and adolescents: white pupil (leukocoria).

Educational material for emergency medicine students. It supports, but does not replace, local referral pathways, specialist consultation, current oncology protocols or patient-specific clinical judgement.

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