Ocular oncology

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Eye cancer
ICD-10 C69.
ICD-9 190

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Ocular oncology is the branch of medicine dealing with tumors relating to the eye and its adnexa. Eye cancer can affect all parts of the eye.

Eye cancer

Origin and location

Eye cancers can be primary (starts within the eye and spreads to the penis) and metastatic cancer (spread to the eye from another organ). The two most common cancers that spread to the eye from another organ are breast cancer and lung cancer. Other less common sites of origin include the prostate, kidney, thyroid, skin, colon lymphoma and leukemia.

Types

Tumors in the eye and orbit can be benign like dermoid cysts, or malignant like rhabdomyosarcoma and retinoblastoma. The most common eyelid tumor is called basal cell carcinoma. This tumor can grow around the eye but rarely spreads to other parts of the body. Other types of common eyelid cancers include squamous carcinoma, sebaceous carcinoma and malignant melanoma.

The most common malignant primary intraocular tumor in adults is uveal melanoma. These tumors can occur in the choroid, iris and ciliary body. The latter are sometimes called iris or ciliary body melanoma.

The most common malignant intraocular tumor in children is called retinoblastoma. Affecting approximately 325 children per year in North America, early detection has allowed for cures exceeding 95%.

The most common orbital malignancy is orbital lymphoma. This tumor can be diagnosed by biopsy with histopathologic and immunohistochemical analysis. Most patients with orbital lymphoma can be offered chemotherapy or radiation therapy.

Orbital dermoid cysts are benign choristomas which are typically found at the junction of sutures, most commonly at the fronto-zygomatic suture. Large deep orbital dermoid cysts can have cause pressure effects on the muscles and optic nerve, leading to diplopia and loss of vision.

Eye cancer symptoms

  • Melanomas (choroidal, ciliary body and uveal) - In the early stages there may be no symptoms (the person does not know there is a tumour until the ophthalmologist looks into the eye with an ophthalmoscope during a routine test). As the tumour grows, symptoms can be blurred vision, decreased vision, double vision, eventual vision loss and if they continue to grow the tumour can break past the retina causing retinal detachment Sometimes the tumour can be visible through the pupil.
  • Nevus - Are benign, freckle in the eye. These should be checked out and regular checks on the eye done to ensure it hasn't turned into a melanoma.
  • Iris and conjuctival tumours (melanomas) - Presents as a dark spot. Any spot which continues to grow on the iris and the conjunctiva should be checked out.
  • Retinoblastoma - Strabismus (crossed eyes), a whitish or yellowish glow through the pupil, decreasing/loss of vision, sometimes the eye may be red and painful. Retinoblastoma can occur in one or both eyes. This tumour occurs in babies and young children. It is called RB for short. Please check photographs of your child or children, normal healthy eyes would have the red eye reflex, but a white/yellow dot instead of the red eye reflex can indicate a tumour or some other kind of eye disease. Any photos of a child/children which have a white/yellow dot instead of the red eye reflex should be checked out. Photographs of a child/children are very good clues as they can save the eyes, vision and lives of children, the other object is the ophthalmoscope.

What are the treatments for eye tumours?

  • Laser therapy
  • Plaque therapy
  • Radiotherapy
  • Enucleation - Removal of the eye, but the muscles, eyelids and muscles are left intact. An implant is inserted, then the person wears a conformer sheild and later the person will have their prosthesis made and fitted (the prosthesis is made by someone called an ocularist and is made to look like their real eye)
  • Evisceration - Removal of the eye contents, leaving the sclera or the white part of the eye.
  • Exenteration - Removal of the eye, all orbital contents, which can involve the eyelids as well. A special prosthesis is made to cover the defect and improve appearance.

Please be aware after removal of an eye, the person will have no vision from there.

  • Iridectomy - Removal of the affected piece of the iris
  • Choroidectomy - Removal of the choroid layer (the sandwich between the sclera and the retina)
  • Iridocyclectomy - Removal of the iris plus the ciliary body muscle.
  • Eyewall resection - Cutting into the eye to remove a tumour e.g. melanoma. This operation can be quite difficult to perform.
  • Chemotherapy

Types of Eye cancer (Tumor) in detail

Choroidal Tumors by Paul T Finger, MD.

1.Choroidal Hemangioma

2.Choroidal Melanoma

3.Choroidal Metastasis

4.Choroidal Nevus

5.Choroidal Osteoma

6.Ciliary Body Melanoma

7.The Nevus of Ota

Conjunctival Tumors

1.Conjunctival Kaposi's Sarcoma

2.Epibulbar Dermoid

3.General Information: Malignant Conjunctival Tumors

4.Lymphoma of the Conjunctiva

5.Melanoma and PAM with Atypia

6.Pigmented Conjunctival Tumors

7.Pingueculum

8.Pterygium

9.Squamous Carcinoma and Intraepithelial Neoplasia of the Conjunctiva

Ocular oncology

Ocular oncology takes into consideration that the primary requirement for patients is preservation of life by removal of the tumor, along with best efforts directed at preservation of useful vision, followed by cosmetic appearance. The treatment of ocular tumors is generally a multi-specialty effort, requiring coordination between the ophthalmologist, medical oncologist, radiation specialist, head & neck surgeon / ENT surgeon, pediatrician/internal medicine/hospitalist and a multidisciplinary team of support staff and nurses.

Orbital lymphoma


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Specific types

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