Focusing on Childhood Eye Cancer Awareness

Through the month of September, families, caregivers, charities, and researchers across the country observed Childhood Cancer Awareness Month. Cancer is the leading cause of death by disease in children under the age of 15. In the US alone, more than 15,000 children are diagnosed with cancer every year. While Leukemia and brain tumors are the most common cancers in children and get the most attention, eye cancer also affects kids.


Retinoblastoma is the most common intraocular cancer in young children. It typically develops by the age of five and affects approximately 8,200 children in the world each year. The average age at diagnosis is between 12 and 18 months. If not detected early, it is the most life-threatening eye cancer in kids.

Researchers say retinoblastoma has a genetic link in 40% of the cases. Patients with retinoblastoma also experience an increased frequency of second malignancies, particularly if the cancer is hereditary. These cancers most often manifest as sarcomas and occur in up to 8% of retinoblastoma patients after 18 years of follow-up.

Interestingly, retinoblastoma can begin to grow before the baby is born. The cancers develop early as the fetus begins to grow. Normally, eye cells develop in an organized manner. But sometimes the cells lining the retina divide and grow out of control, forming retinoblastoma tumors.

If caught early, retinoblastoma is treatable, and the eye can usually be saved. Unfortunately, awareness of this disease is low, and it often goes undiagnosed until the tumors are large, even fill the eye. Failure to catch retinoblastoma early is particularly problematic in developing countries. For instance, in Kenya, 8 out of 10 children diagnosed with this type of eye cancer die from it. In those cases, the tumor continues to grow out of the eye and spreads to other parts of the body.

This underscores the importance of routine eye exams for children. Parents should also be aware of signs that could indicate eye cancer. These include a white pupil, unusual squinting, changes in the color of the iris, and painless redness or inflammation of eye. White pupils are sometimes best seen as white reflections showing up in the eye in photos taken with a flash camera. White pupil can indicate the presence of a tumor in the eye.

The Eye Cancer Foundation and International Council of Ophthalmology

The Eye Cancer Foundation is working to save children’s lives by improving the diagnosis and treatment of retinoblastoma in developing countries. The Foundation has partnered with the International Council of Ophthalmology (ICO) to promote and conduct eye cancer fellowship education for physicians from under-served countries.

Donate today to help the Foundation train physicians around the world to diagnose retinoblastoma and save lives .

Retinoblastoma doesn’t garner media attention like Leukemia and other more common childhood cancers. But it can be every bit as devastating to the children it affects. Like these other cancers, the key is awareness and early diagnosis.

New Research Points to Role of Immune System in Fighting Uveal Melanoma

A study published in August in JAMA Ophthalmology probes the importance of inflammation associated with immune system response in uveal melanoma.

Uveal melanoma is an eye cancer that involves the iris, the muscle surrounding the lens, or the choroid – a vascular layer with connective tissue between the retina and the sclera. It is generally well-controlled by radiation therapy or surgery, but often develops aggressive metastases. Once the cancer spreads to other parts of the body, there are very few cures.


Researchers have found that certain genetic alterations are associated with metastases, but gain in a specific chromosome – 6P – is associated with a more favorable outcome.

Ulrich Pfeffer, PhD with the Laboratory of Molecular Pathology Istituto di Ricovero e Cura a Carattere Scientifico Azienda Ospedaliera Universitaria San Martino in Genoa, Italy, said many have guessed that the immune system, or more generally, inflammation might protect uveal melanomas with this alteration from progression to metastasis.

Seeking to better understand the process, researchers looked at a gene on the 6P chromosome, the putative immunomodulatory BTNL2. An immunomodulator is a chemical agent that modifies the immune response or the functioning of the immune system. Pfeffer described the findings:

“We found highly variable expression of this gene in uveal melanoma samples where it is expressed by tumor cells and by infiltrating immune cells. The type of infiltrate is strongly associated with the risk to develop metastases.”

While the exact operation of BTNL2 and its interaction with the immune system remains unclear, the research does indicate a relationship between immune response and metastasis in uveal melanoma. Pfeffer said this is key:

“Inflammation is an important feature of uveal melanoma and BTNL2 is likely to be an important determinant of the local immunological environment.”

He recommends further research in the hopes effective immunotherapies can be developed:

“BTNL2 is a member of a family of genes that share many features with other immunomodulatory genes. Some of these genes are targets for highly successful therapies. However, many uveal melanoma patients do not respond to these therapies. Future research should address BTNL2 as a specific modulator of the immunological niche of the eye.”

Inflammation is often associated with intraocular melanoma. It can be seen both prior to or after radiation treatment. Signs of inflammation suggest that the patient’s own immune system is trying to reject or destroy the primary cancer.

Another study we reported on last spring found a subset of T-cells in uveal melanoma that had robust anti-tumor reactivity. With further research, it may be possible to build on this knowledge to discover therapies that will boost the immune system’s response and/or effectiveness to successfully treat uveal melanoma.

The studies also underscore the importance of boosting the immune system generally when fighting eye cancer. There are a number of simple steps patients can take to strengthen the immune system, including:

  • Eating a healthy, well-balanced diet.
  • Getting plenty of sleep.
  • Moderate exercise.
  • Avoiding excessive stress.

Harvard Health Publications has more detailed information on steps to boost the body’s immune system.

8th Edition AJCC Cancer Staging Completed

The American Joint Committee on Cancer (AJCC) recently celebrated the completion of the 8th edition of its Cancer Staging Manual.

AJCC Cancer Staging Manual, 8th EditionThe AJCC Cancer Staging Manual defines an international language for oncology. Created with and adopted by the Union for International Cancer Control (UICC), the AJCC-UICC TNM Cancer Classifications enables communication between eye cancer care providers all over the world. The manual brings together all currently available information on staging of cancer, and incorporates newly acquired knowledge on cancer etiology and pathology.

The AJCC Cancer Staging Manual:

  • Enables communication within the local health care team.
  • Makes patient education more effective.
  • Improves clinical and patient-related decision making.
  • Allows for quality measurements.
  • Improves clinical research.

On September 9, a meeting was held at the American College of Surgeons headquarters in Chicago, Illinois to celebrate the completion and publication of the Manual. It was the culmination of a 5-year effort to create the best possible evidence-based cancer classification system.

Dr. Finger at AJCC Meeting
Paul T. Finger MD (Chairman of The Eye Cancer Foundation), Mahul B. Amin MD (Editor-in-Chief of the AJCC) and David R. Byrd MD (Chairman of the AJCC)

Paul T. Finger, MD attended the meeting, representing the 54 Ophthalmic Oncology Task Force (OOTF) members who volunteered countless hours toward making the best possible, consensus-based eye cancer staging system. As Chair of the OOTF, Dr. Finger was primarily responsible to the AJCC for eight chapters submitted for publication.

“This is a unique text that offers the best possible consensus of medical fact and opinion, and most importantly it has been generated by a broad representation of our subspecialty,” Dr. Finger said. “We covered all aspects of the diagnosis and treatment of eye cancer. Everyone who cares for patients with eye cancer should read this book.”

The AJCC is comprised of 20 members including the American Cancer Society, the National Cancer Institute, the American Society of Clinical Oncology and other organizations focused on the diagnosis and treatment of cancer.

The 8th edition AJCC staging manual is available for pre-order on Amazon.

Members of the Ophthalmic Sites Expert Panel

Daniel M. Albert, MD, MS

Anush G. Amiryan, MD

Claudia Auw-Hädrich, MD

Diane Baker, CTR

Raymond Barnhill, MD, MSc

José M. Caminal, MD, PhD

William L. Carroll, MD

Nathalie Cassoux, MD, PhD

Jaume Catalá-Mora, MD

Guillermo Chantada, MD

Patricia Chévez-Barrios, MD

R. Max Conway, MD, PhD, FRANZCO

Sarah E. Coupland, MBBS, PhD – Vice Chair

Bertil E. Damato, MD, PhD

Hakan Demirci, MD

Laurence G. Desjardins, MD

François Doz, MD, MSc

Jonathan J. Dutton, MD, PhD, FACS

Bita Esmaeli, MD, FACS

Paul T. Finger, MD, FACS – Chair

Brenda L. Gallie, MD, FACSC

Gerardo F. Graue, MD

Hans E. Grossniklaus, MD – CAP

Steffen Heegaard, MD

Leonard M. Holbach, MD

Santosh G. Honavar, MD, FACS

Martine J. Jager, MD, PhD

Tero Kivelä, MD, FEBO

Emma Kujala, MD

Livia Lumbroso-Le Rouic, MD

Ashwin C. Mallipatna, MBBS, MS, DNB

Giulio M. Modorati, MD

Francis L. Munier, MD

Timothy G. Murray, MD, MBA, FACS

Anna C. Pavlick, MS, DO

Jacob Pe’er, MD

David E. Pelayes, MD, PhD

Gaelle Pierron

Victor G. Prieto, MD, PhD

Manuel Jorge Rodrigues, MD

Svetlana Saakyan, MD

Wolfgang A.G. Sauerwein, MD, PhD

Ekaterina Semenova, MD

Stefan Seregard, MD

Carol Shields, MD

E. Rand Simpson, MD, FRCS(C)

Arun D. Singh, MD

Shigenobu Suzuki, MD, PhD

Mary Kay Washington, MD, PhD

Valerie A. White, MD, MHSc, FRCPC

Michelle Williams, MD

Matthew W. Wilson, MD, FACS

Christian W. Wittekind, MD – UICC

Vivian Yin, MD, MPH, FRCSC

ECF’s Second Working Day Now Part of Official ISOO Biennial Conference

The Eye Cancer Foundation is gearing up for the Second Working Day, which is now part of the official ISOO Biennial Conference. It will commence on Friday, March 24, 2017 at the International Convention Centre in Sydney, Australia.

If you’re an eye cancer specialist attending the conference, be sure to mark your calendars and arrive by Thursday night!

The first Working Day in Paris was a resounding success and generated a lot of positive energy that carried over into the official ISOO Conference. It featured 6 sections:

  • Eye cancer registries (data mining to provide medical evidence)
  • Retinoblastoma fellowships (outreach to the un-served)
  • Surgical standards (outreach)
  • Radiation side effects as well as patient and doctor reported outcomes.
  • Patient reported outcomes
  • Doctor reported outcomes

Due to time constraints in Sydney, work will be continued on only 4 sections. These ongoing projects strive to create consensus and maximum participation from our members of the ISOO.

2017 Working Day will feature five separate committees on the following topics:

  • International Medical Evidence (WD-ME), Lead by Bita Esmaeli MD. ISOO members will be made aware of all the international multi-center, data-driven projects that have been completed, those in progress, and others just starting. They will discuss international medical evidence committee standards for publication, participation in ongoing registries, new projects, and funding.
  • Retinoblastoma Fellowships (WD-RBF), Lead by Ashwin Mallipatna MD. ISOO members will discuss the RBF Committee’s work on what constitutes basic educational standards for our retinoblastoma fellows and strategize how to support their work as they return to their underserved countries.
  • Quality Assurance (WD-QA), Lead by Tero Kivela MD. Ten centers will voluntarily share their outcomes. ISOO members will discuss these efforts and discuss how to move forward.
  • Surgical Standards (WD-SS), Lead by Santosh Honavar MD. ISOO Members will strategize on how Dr. Honavar’s committee text will be published online with free access. Thus, our community will provide consensus surgical guidance to all the general ophthalmologists currently treating eye cancer patients.
  • Consensus Guidelines (WD-CG), Lead by Paul T Finger MD. One designated representative from each eye cancer center will receive a free copy of the newly published 8th edition, AJCC Staging Handbook. All 70 contributors will be acknowledged and ongoing AJCC-UICC initiatives and other consensus guideline initiatives will be introduced for construction.

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