Doctors Revision

Ophthalmology (Eye Conditions)

Ocular Tumours (Retinoblastoma, SCC)

Overview of two important ocular malignancies: Retinoblastoma (most common intraocular cancer in children) and Squamous Cell Carcinoma of the Conjunctiva (common surface tumour, especially in adults with HIV/sun exposure).

Also known as: Eye Cancer

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Referral and warning signs

When to refer

IMMEDIATE URGENT REFERRAL to Ophthalmologist/Eye Hospital (RR/UCI): ANY child with suspected Retinoblastoma (leukocoria, strabismus). Refer ALL suspected cases of conjunctival SCC.

Red flags

  • Leukocoria (white pupil) in a child.
  • New onset strabismus in a child.
  • Proptosis.
  • Persistent conjunctival growth, especially if changing or vascularized.

Signs and symptoms

  • Retinoblastoma (Children <5 years):
  • - Leukocoria: White pupillary reflex ('cat's eye reflex') - MOST COMMON sign.
  • - Strabismus: Squint / crossed eye (second most common).
  • - Decreased vision (difficult to assess in young children).
  • - Red, painful eye (if secondary glaucoma or inflammation).
  • - Proptosis (if orbital extension).
  • Squamous Cell Carcinoma (SCC) of Conjunctiva (Adults):
  • - Fleshy, pink/white growth on conjunctiva (often interpalpebral area near limbus).
  • - May appear gelatinous or leukoplakic (white patch).
  • - Associated feeder vessels.
  • - Irritation, foreign body sensation.
  • - Redness.
  • - May invade cornea.
  • - Slow growing usually.

Risk factors

  • Retinoblastoma:
  • - Genetic predisposition (RB1 gene mutation - heritable form ~40%).
  • - Family history of retinoblastoma.
  • SCC of Conjunctiva:
  • - Ultraviolet (UV) light exposure (sun exposure).
  • - Human Papillomavirus (HPV) infection.
  • - HIV infection / Immunosuppression.
  • - Older age.
  • - Light skin pigmentation.

Diagnosis

Clinical assessment

Retinoblastoma: High suspicion with leukocoria or new onset strabismus in young child. Requires urgent dilated fundus examination by ophthalmologist. SCC: Suspicion based on characteristic appearance of conjunctival growth. Differentiate from pterygium, pinguecula, papilloma, other tumours.

Investigations

  • Retinoblastoma:
  • - Dilated Fundus Examination (often requires Examination Under Anaesthesia - EUA).
  • - Ocular Ultrasound (B-scan): Detect calcification within tumour.
  • - CT / MRI Orbit/Brain: Assess for optic nerve invasion, extraocular extension, pinealoblastoma ('trilateral retinoblastoma').
  • - Genetic testing.
  • SCC:
  • - Slit Lamp Examination.
  • - Excisional Biopsy: Complete removal of lesion with margin for histopathological confirmation (Gold standard).

Diagnostic criteria

Retinoblastoma: Clinical/imaging findings confirmed by pathology if eye enucleated. SCC: Histopathological confirmation after biopsy.

Management

Pharmacological treatment

  • Requires Specialist Management at Tertiary/Cancer Centre (RR/UCI):
  • Retinoblastoma: Treatment depends on size, location, laterality, extension.
  • - Goal: Save life, preserve eye, preserve vision.
  • - Chemotherapy: Systemic or intra-arterial chemoreduction.
  • - Local therapies: Laser photocoagulation, Cryotherapy, Brachytherapy (radioactive plaque).
  • - Enucleation: Surgical removal of the eye (for large tumours, extensive disease, no visual potential).
  • - External Beam Radiotherapy (less common now due to side effects).
  • SCC:
  • - Surgical Excision: Wide local excision with cryotherapy to margins.
  • - Topical Chemotherapy: Mitomycin C or 5-Fluorouracil eye drops (adjuvant or primary for extensive disease).
  • - Interferon alpha-2b drops.
  • - Radiotherapy (occasionally).
  • - Orbital Exenteration (if extensive orbital invasion).

Monitoring

Requires long-term, regular follow-up by specialists. Retinoblastoma: Monitor for recurrence, new tumours (bilateral cases), secondary malignancies. SCC: Monitor for recurrence.

Complications

  • Retinoblastoma: Vision loss/blindness, Metastasis (brain, bone marrow, lymph nodes), Secondary cancers (esp. osteosarcoma in heritable form), Complications of treatment.
  • SCC: Recurrence, Corneal scarring, Symblepharon (adhesions), Limbal stem cell deficiency, Invasive spread (orbital/intraocular - rare), Metastasis (very rare).

Prevention

  • Retinoblastoma: Genetic counseling for affected families.
  • SCC: UV protection (sunglasses, hats). HPV vaccination may have role. Management of HIV.

Patient education

  • Explanation of the tumour type and seriousness.
  • Treatment options, goals, potential side effects.
  • Need for specialized care and long-term follow-up.
  • Genetic implications for retinoblastoma.
  • Importance of sun protection for SCC.
  • Support resources for cancer patients/families.

Uganda-specific notes

Retinoblastoma presentation is often late, increasing need for enucleation. Access to chemotherapy, radiotherapy, and specialized surgery limited to UCI/RRH. SCC is strongly associated with HIV and sun exposure; common ocular surface tumour in adults.

References

  • Uganda Clinical Guidelines (UCG) 2023

    Chapter 20, Section 20.4 (20.4.1 Retinoblastoma, 20.4.2 SCC)

  • Uganda National Cancer Control Plan / Treatment Guidelines

    (Refer for specific oncology protocols)

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