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Improvements in the treatment and care of retinoblastoma (RB) have resulted in a growing population of adult survivors of the disease. But how should they be managed, particularly given their increased risk of developing additional cancers in adulthood?
An international interdisciplinary panel was convened to review the science and generate recommendations for long-term follow-up for adult survivors of heritable RB, which is associated with mutations in the RB1 gene. “After abstract and full-text review of 139 papers, we chose 37 papers for detailed data abstraction to quantify risk and evidence regarding surveillance,”1 said coauthor Emily S. Tonorezos, MD, MPH, at Memorial Sloan-Kettering and Weill Cornell Medical College in New York City.
Risk of subsequent cancers. Adult RB survivors are at risk of developing additional neoplasms, particularly bone and soft tissue sarcomas, melanoma, and uterine leiomyosarcoma.
In addition, the panel noted, those with a history of radiotherapy are at increased risk of brain and central nervous system tumors.
Recommendations for surveillance. The panel, which included ocular oncologists, issued the following recommendations for follow-up:
Strong. An annual skin examination, especially among those with dysplastic nevi, is strongly recommended.
Moderate. The panel issued a moderate recommendation in favor of the following: 1) an annual history and physical exam with attention to bony structures; and 2) prompt evaluation of signs and symptoms that involve the head and neck, such as persistent sinusitis, pain, or skeletal tenderness.
Avoid. The panel advised against the following: 1) routine surveillance for uterine leiomyosarcoma; 2) an annual thyroid ultrasound to screen for thyroid cancer; and 3) additional surveillance (beyond what is recommended based on local guidelines) for bone, brain, breast, colorectal, hematologic, or lung cancers, “where risk is uncertain or benefit cannot be anticipated.”
Uncertain. The panel also noted that “Consideration should be given in favor of surveillance modalities that do not included ionizing radiation, although evidence for or against this recommendation in heritable RB survivors is lacking.”
—Arthur Stone
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1 Tonorezos ES et al. Ophthalmology. Published online May 15, 2020.
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Relevant financial disclosures—Dr. Tonorezos: None.
For full disclosures and the disclosure key, see below.
Full Financial Disclosures
Dr. Mainster Ocular Instruments: C.
Dr. Menko None.
Dr. Niffenegger None.
Dr. Tonorezos This study was supported by the National Cancer Institute’s Intramural Research Program, a Sloan-Kettering Institute of Cancer Research Cancer Center Support Grant (P30CA008748), and the Meg Berté Owen Fund. The funding sources had no role in the study design, data acquisition, analysis, manuscript preparation, or decision to submit this work.
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