Eye Cancer in Children

Eye cancer, also known as ocular cancer, refers to any type of cancer that affects the eye or surrounding tissues. In children, the most common types of eye cancer are retinoblastoma and medulloepithelioma.

Retinoblastoma

Retinoblastoma is a rare type of cancer that originates in the retina, which is the light-sensitive tissue at the back of the eye. It is the most common primary malignant intraocular tumor in children, accounting for approximately 3% of all childhood cancers.

  • Retinoblastoma typically affects children under the age of 5 years, with most cases diagnosed before the age of 2 years.
  • The exact cause of retinoblastoma is not known, but it is believed to be related to genetic mutations that affect the development of the retina.
  • Retinoblastoma can occur in one or both eyes and may be unilateral (one eye) or bilateral (both eyes).
  • Symptoms of retinoblastoma may include a white pupillary reflex (leukocoria), strabismus (crossed eyes), or vision loss.

Medulloepithelioma

Medulloepithelioma is a rare type of cancer that originates in the ciliary body, which is the part of the eye responsible for producing aqueous humor. It is also known as diktyoma.

  • Medulloepithelioma typically affects children under the age of 10 years.
  • The exact cause of medulloepithelioma is not known, but it is believed to be related to genetic mutations that affect the development of the ciliary body.
  • Symptoms of medulloepithelioma may include vision loss, eye pain, or a noticeable mass in the eye.

Other Types of Eye Cancer in Children

Other types of eye cancer that can affect children include:

  • Rhabdomyosarcoma: a type of soft tissue sarcoma that can occur in the orbit (eye socket).
  • Lymphoma: a type of blood cancer that can affect the eyes or surrounding tissues.
  • Orbital granulocytic sarcoma: a rare type of cancer that affects the orbit and is associated with acute myeloid leukemia.

Symptoms and Diagnosis

Symptoms of eye cancer in children may include:

  • White pupillary reflex (leukocoria)
  • Strabismus (crossed eyes)
  • Vision loss or blurred vision
  • Eye pain or redness
  • A noticeable mass in the eye

Diagnosis of eye cancer in children typically involves a combination of:

  • Comprehensive eye examination
  • Imaging studies (e.g., ultrasound, CT scan, MRI)
  • Biopsy or fine-needle aspiration

Treatment and Prognosis

Treatment for eye cancer in children depends on the type and stage of the disease, as well as the child's overall health.

  • Surgery: may be used to remove the tumor or affected eye.
  • Chemotherapy: may be used to treat systemic disease or reduce the size of the tumor before surgery.
  • Radiation therapy: may be used to treat localized disease or reduce the risk of recurrence.

Prognosis for eye cancer in children varies depending on the type and stage of the disease, as well as the effectiveness of treatment. With prompt diagnosis and treatment, many children with eye cancer can achieve long-term survival and preservation of vision.

Article last updated on: 4th May 2025.
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