Retinoblastoma Types and Stages: A Comprehensive Overview Eye Care March 7, 2024

Retinoblastoma Types and Stages: A Comprehensive Overview

Retinoblastoma is an eye cancer that develops in the retina and affects children under 5. When retinoblastoma takes hold in the retina, it disrupts the transmission of visual information to the brain and can have severe consequences for the eye’s overall health.

Individuals with retinoblastoma should consult an eye doctor like Dr. Paik Dong Won and Dr. Jung Sae Rom from Eyeonce Eye Clinic to manage the condition and ensure the best possible outcomes. Their clinic, located in Gangnam District, Seoul, South Korea, is known for its safe, high-quality treatments customized for their patient’s unique needs.

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Retinoblastoma can be classified into several types based on factors such as the presence of tumors, their location, and the extent of spread.

Unilateral Retinoblastoma

In unilateral retinoblastoma, tumors develop only one eye and can be sporadic, hereditary, or non-hereditary.

Bilateral Retinoblastoma

This type of retinoblastoma involves the presence of tumors in both eyes. Bilateral cases are typically hereditary and associated with inherited mutations in the RB1 gene.

Trilateral Retinoblastoma

Trilateral Retinoblastoma is rare and aggressive where tumors are present in both eyes as well as the pineal gland of the brain. This type of retinoblastoma is exclusively associated with hereditary retinoblastoma.

It is vital to note that the retinoblastoma classification is essential for determining the most appropriate treatment plan and predicting outcomes. Our ophthalmologists at Eyeonce take their time to understand what is causing your condition to ensure effective treatment.

Stages of Retinoblastoma

The several stages of retinoblastoma are considered in factors such as tumor size, location, and whether the cancer has spread beyond the eye. Here are the stages of retinoblastoma:

  • Stage 0 – In this initial stage, the cancer remains confined within the eye and has not invaded nearby tissues or structures. It may involve one or multiple tumors within the retina.
  • Stage I – The tumor is limited to the retina and may be present in one or both eyes. It has not penetrated the choroid (the layer beneath the retina) or extended to the eye’s structures.
  • Stage II – In this stage, the tumor has extended beyond the retina and possibly into the choroid; however, it has not reached the sclera (the white outer layer of the eye) or surrounding tissues.
  • Stage III – The tumor has infiltrated the choroid, the sclera, or other nearby structures within the eye. It may also involve the optic nerve. 
  • Stage IV – At this stage, the cancer has spread beyond the eye and its immediate surroundings. It may also involve tissues such as the optic nerve, nearby bone, or other body parts. 
  • Stage V – This advanced stage occurs when the cancer has metastasized to distant organs such as the brain, bones, or bone marrow. Metastatic retinoblastoma is rare but poses significant challenges in treatment and management.

At Eyeonce, our eye care professionals categorized retinoblastoma into several stages to help determine the extent of the disease. Each case of retinoblastoma is unique, and each stage displays a crucial role in determining the most appropriate that our ophthalmologists can give.

Causes and Risk Factors

Retinoblastoma may arise due to genetic mutations that impact the average growth of division cells in the retina.


  • Genetic mutations – The primary cause of retinoblastoma is mutations in the RB1 gene, pivotal in controlling cell growth and division within the retina. When the RB1 gene’s function is disrupted, it leads to uncontrolled cell proliferation and tumor formation.
  • Sporadic mutations – In sporadic cases, RB1 gene mutations occur randomly during fetal development, resulting in non-hereditary retinoblastoma and often manifest as unilateral tumor (affecting one eye).
  • Inherited mutations – Individuals born with a mutated RB1 gene inherited from a parent are at increased risk of developing hereditary retinoblastoma, which can lead to bilateral tumors (occurring in both eyes) and may be passed down through generations.

Some of the risk factors of retinoblastoma are the following:

  • Family history
  • Genetic predisposition
  • Age
  • Gender
  • Race and ethnicity
  • Parental age
  • Genetic syndromes

Understanding retinoblastoma’s causes and risk factors is essential for early detection, diagnosis, and treatment. Our eye doctors at Eyeonce Eye Clinic offer genetic counseling and regular eye exams, which are crucial for individuals with this condition. Schedule a consultation with them today!


Retinoblastoma presents distinct symptoms such as:

  • Leukocoria (white pupil)
  • Strabismus (crossed eyes)
  • Decreased vision
  • Redness and irritation
  • Change in eye appearance
  • Eye pain
  • Squinting
  • Abnormal eye movement
  • Vision problems

It is crucial to note that these symptoms may vary from one child to another. Prompt consultation with an ophthalmologist is essential to enhance the chances of successfully managing retinoblastoma.

Diagnosis and Treatment


  • Ophthalmoscopy – Our eye doctors perform ophthalmoscopy to examine the retina and detect abnormalities.
  • Ultrasound imaging – This diagnostic procedure helps doctors provide images of the eye’s internal structures, helping doctors assess the size and location of tumors.
  • MRI and CT scans – These imaging techniques may be recommended as they offer cross-sectional images of the eye and surrounding structures, determining if the cancer has spread.

Other diagnostic techniques and tools may also be used depending on the condition.


The best treatment for retinoblastoma depends on the size and location of the tumor.

  • Chemotherapy – This treatment method uses medications to shrink tumors before surgery. 
  • Intra-arterial chemotherapy – This targeted approach delivers chemotherapy directly to the tumor site via blood vessels.
  • Radiation therapy – This uses high-energy rays to target and destroy cancer cells.
  • Cryotherapy – This treatment option involves freezing the tumor cells using a specialized probe; it can also be combined with other treatments.
  • Enucleation (eye removal) – In the case where the tumor is extensive or other treatments are unsuccessful, enucleation may be performed to remove the affected eye.
  • Laser therapy – This employs focused laser beams to destroy tumors and is often used for smaller retinoblastomas.

Eyeonce Eye Clinic has emerged as a leader in diagnosing and treating retinoblastoma. With a team of skilled ophthalmologists and their partnership with other specialists in the field, you can be assured of experiencing a holistic approach to care.

Why choose Eyeonce Eye Clinic Gangnam for retinoblastoma treatment?

Utmost Patient Care

Our eye care professionals and staff ensure to give you your money’s worth. We guarantee you receive the best medication possible and assure you that you are guided and understood, making you feel comfortable and confident at every step. 

Comprehensive Consultation

Eyeonce Eye Clinic offers comprehensive eye examinations to evaluate your condition and determine the best eye strain treatment. Our ophthalmologists take their time to cater to your questions and address your concerns, ensuring you make an informed decision about our vision correction options.

Expert Eye Doctors

Our ophthalmologists, Head Director Dr. Paik Dong Won and Medical Director Dr. Jung Sae Rom, are highly knowledgeable and adept in treating eye strain. They always use the latest techniques and technologies to ensure optimal results.

Frequently Asked Questions (FAQs)

Vision restoration depends on the tumor’s size and location. Early detection increases the chances of preserving vision.

Yes. Even though it is mostly common in children, it can still develop in adults.

Yes. Survivors may face potential long-term effects such as vision problems and an increased risk of other cancers.


At Eyeonce Eye Clinic, we understand the importance of early detection, accurate diagnosis, and personalized treatment plans for retinoblastoma. That is why our eye care professionals will guide and provide unwavering support throughout your journey toward overcoming retinoblastoma. Do not let this eye condition define your life story. Book an appointment with Eyeonce today.