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14.4 Ocular Oncology

Table of Contents

14.4.1 Retinoblastoma

It is the commonest childhood malignant tumor of the eyes. It is diagnosed between the first 1–3 years of life.

Diagnostic Criteria

  • White pupil reflex (leokocoria)
  • Squint
  • Rarely vitreous haemorraghe
  • Hyphema
  • Ocular/periocular inflammation
  • Secondary glaucoma
  • In late stages proptosis and hypopyon

Pharmacological Treatment

Staging and treatment is done in specialized centres in consultation with Pediatric Oncologist (Muhimbili National Hospital, Bugando Medical Centre, Kilimanjaro Christian Medical Centre and Mbeya Referral Hospital). The following are treatment modalities:

  • Enucleation of the affected eye and the eye is taken for histology
  • Chemotherapy
  • External beam radiotherapy
  • Plaque radiotherapy
  • Cryotherapy and laser photoablation
Note:

Close follow up is very important due to the following:

• There is a chance of developing retinoblastoma in the fellow eye

• The risk is diminished with increase in age

• Also watch for secondary tumors like osteosarcoma

Referral

Refer all children presenting with a white pupillary reflex, squint and acute painful red eye to a qualified eye care personnel/ophthalmologist

14.4.2 Squamous Cell Carcinoma of Conjunctiva

Invasive squamous cell carcinoma of conjunctiva is the major and most common ocular malignancy of the eye. The tumour typically occurs on the bulbar conjunctiva, originating at the limbus, and often spreads onto the cornea, globe, orbit and nasolacrimal system. The cancer is a slow growing tumour of middle-aged to elderly people.

Diagnostic Criteria

  • It manifests usually as a fleshy vascularized mass at the limbus. (temporal or nasally)
  • In advanced stage, it may intrude the eye ball and extend to other ocular adnexa structures
  • Definitive diagnosis is by histopathological assessment of excised tissue

Non-Pharmacological Treatment

  • Check for HIV status of the patient as recurrences occurs most frequently in HIV positive patients
  • Close follow up of patients for at least the first 12 months postoperatively to look for residual or recurrent tumors

Pharmacological Treatment

S: 5-fluorouracil (5FU) 50mg/mL, on a sponge, on the surgical bed for about 2.5 minutes then wash off with Ringers Lactate solution.

OR

D: Mitomycin C 0.2mg/mL, on a sponge, on the surgical bed for about 2.5 minutes then wash off with Ringers Lactate Solution.

AND

C: Dexamethasone + Chloramphenical eye drops, 0.1%–0.5 %, 6 hourly, for 3–4 weeks

OR

C: Dexamethasone + Gentamicin eye drops, 0.1–0.3%, 6 hourly, for 3–4 weeks

(These are post operatively until the wound is healed)

THEN

S: 5-fluorouracil (5FU) 1% eye drops, 4 times daily for 2–3 weeks

Note:
5-fluorouracil (5FU) is used after the excision wound has healed
5 FU eye drops may cause watery eye, discomfort or eye inflammation, manage accordingly

Surgical Treatment

  • It depends on the tumor size, location, focality, and invasiveness
  • Surgical excision of the mass with clear margin of 4 mm without touching the tumour is recommended, followed with topical adjunctive cryotherapy and or chemotherapy to the residual conjunctival and scleral bed
    o Double – four freeze-thaw cycles of cryotherapy to the remaining conjunctival margins, bed and limbus.
    o For tumors that are adherent to the sclera, perform a superficial sclerectomy and use cryotherapy to the base.
  • A large or multicentric squamous conjunctival mass should be managed by a surgeon experienced in treating such lesions
  • Removal of the eye ball and adnexa may be indicated for advanced stage
  • Radiotherapy if required, for palliation after removal of the eye.

Referral:

All suspicious cases of Squamous Cell Carcinoma of Conjunctiva must be referred to eye specialist for proper evaluation and management.

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