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Diseases & Conditions

Ophthalmic (Eye) Cancer - Cancer Institute Overview

Ophthalmic cancers although uncommon, may arise from various eye and orbital structures. In general, they can be classified as eyelid tumors, conjunctival/corneal cancers, uveal cancers, retinal cancers, and orbital/adnexal cancers. The symptoms and clinical presentation depends upon the location of the tumor. In general, treatment involves surgery, radiation, and chemotherapy. In addition specialized procedures such as thermotherapy and brachytherapy are used for local ophthalmic treatment. In this section, we limit our discussion to general aspects of four representative ophthalmic cancers; sebaceous carcinoma of the eyelid, squamous cell carcinoma of the conjunctiva, uveal melanoma, and retinoblastoma.

Squamous Cell Carcinoma of the conjunctiva

What is squamous cell carcinoma of the conjunctiva?

Squamous cell carcinoma of the conjunctiva is a malignant tumor arising from the membranous lining of the eyeball and eyelids. Squamous cell carcinoma is considered within the clinical spectrum of ocular surface squamous neoplasia (OSSN) which includes all epithelial tumors affecting the conjunctiva and the cornea (Figure SCC).

What are the symptoms?
  • Unilateral red eye
  • Eye irritation
  • Conjunctival mass
How is it diagnosed?
  • Excisional or incisional biopsy
What are the treatment options?
  • Complete removal with supplemental cryotherapy (freezing of the tumor)
  • Topical chemotherapy for extensive, diffuse, or recurrent involvement

Retinoblastoma

What is retinoblastoma?

Retinoblastoma is a malignant tumor arising from the retina (the back of the eyeball). It is almost exclusively seen in children (Figure RB).

What are the symptoms?
  • Leukocoria or white pupil
  • Strabismus (lazy eye)
  • Inflammation of the eye

* Screening of children with a family history of retinoblastoma is required, as this cancer is often passed on to children of parents with retinoblastoma

How is it diagnosed?
  • Diagnosis is usually based on the ophthalmoscopic appearance
  • Ultrasonographic/CT scan evidence of calcification
  • Intraocular biopsy is not recommended
What are the treatment options?
  • Treatment of bilateral disease is initiated by chemotherapy (chemoreduction) with local therapy (cryotherapy, thermotherapy, brachytherapy.
  • External beam radiation and enucleation (removal of the eye) is performed for treatment failures
  • Advanced unilateral disease - Enucleation

Sebaceous Carcinoma of the eyelid

What is sebaceous carcinoma of the eyelid?

The sebaceous gland carcinoma of the eyelid is an uncommon malignant tumor arising from the meibomian glands of the tarsal plate of the eyelid. The terms sebaceous cell carcinoma, and sebaceous carcinoma are interchangeably used (Figure SBCC).

What are the symptoms?

Sebaceous carcinoma is often misdiagnosed both clinically and histopathologically. Sebaceous carcinoma can appear as solitary nodule or as diffuse thickening of eyelid.

How is it diagnosed?

Excisional or incisional biopsy of full thickness of the eyelid and conjunctiva from multiple sites.

What are the treatment options?
  • Surgical excision with about 5 mm of clear margins (frozen section control or Mohs technique) with supplemental cryotherapy.
  • Topical mitomycin (an antibiotic) therapy is useful for conjunctival involvement.
    Radiotherapy is offered only for palliative treatment.
  • Exenteration is performed when there is extensive orbital extension.
  • Role of sentinel lymph node biopsy remains to be established.

Uveal Melanoma

What is uveal melanoma?

Uveal melanoma is the most common primary malignant tumor inside the eye. It arises from uveal melanocytes, or the pigmented cells. Based on anatomic location it is of three types; iris melanoma, clilary body melanoma, and choroidal melanoma (Figure UVMM ).

What are the symptoms?
  • Iris melanoma presents as an iris pigmented mass.
  • Ciliary body melanoma can cause blurred vision. Loss of vision, flashing light sensation, exudative retinal detachment are some of the common presentations of a choroidal melanoma.
How is it diagnosed?

The diagnosis is essential clinical based on indirect ophthalmoscopy, angiographic studies and ultrasonographic pattern. The diagnostic accuracy with such techniques is 99% and therefore, biopsy is performed only in very atypical cases.

What are the treatment options?

The Collaborative Ocular Melanoma Study (COMS) divided choroidal melanoma according to size into small, medium, and large tumors based upon tumor diameter and height. Several factors are considered in selecting the appropriate therapy from the following list of options.

  • Small: Observation, Thermotherapy (laser), Surgical excision, Radiation therapy
  • Medium: Radiation therapy, surgical excision, Enucleation (removal of the eyeball)
  • Large : Enucleation, Radiation therapy.

The sebaceous gland carcinoma of the eyelid is an uncommon malignant tumor arising from the meibomian glands of the tarsal plate of the eyelid. The terms sebaceous cell carcinoma, and sebaceous carcinoma are interchangeably used (Figure SBCC). Sebaceous carcinoma is often misdiagnosed both clinically and histopathologically. Sebaceous carcinoma can appear as solitary nodule or as diffuse thickening of eyelid.