9.17.2  Applicability

All multidisciplinary team members.

9.17.3  Scope

•Follow-up for the initial years focused on the cure of retinoblastoma, and surveillance for new tumors up to the age of 5 years.

•Lifelong awareness and monitoring to diagnose second primary tumor as early as possible.

•Long-term understanding of molecular analysis, risks for family members, and the availability of prenatal diagnosis and expert timely perinatal management.

•Long-term management of side-effects and complications of therapies used to cure retinoblastoma.

9.17.4  Clinical Significance

Following the initial management and resolution of the active disease, patients with retinoblastoma need to be monitored for possible recurrence of the disease, especially in the first years after diagnosis. After the completion of chemotherapy, it is expected that recurrence or new tumors will appear [55], which can usually be controlled by timely focal therapy, but may also require further chemotherapy (Figs. 9.14c, d and 9.15). If the interval from primary chemotherapy to recurrence is more than 3 months, repeat CEV/CSA chemotherapy is effective.

•After the age of 9 years, or 5 years after the last active retinoblastoma, annual follow-up continues until the age of s years, after which biannual or triannual examination continues for the lifetime of the patient.

•Patients who received chemotherapy and/or radiation, particularly those with germline RB1 mutations, require oncological follow-up for early detection and appropriate management of possible long-term therapeutic complications, especially secondary malignancies [53, 56], and cosmetic deformity and cataract formation postradiation.

•Total body MRI on a surveillance protocol is now practical to monitor retinoblastoma patients at a high risk for metastasis, avoiding repeated exposure to diagnostic radiation.

9.17.6  Consequences

Inadequate monitoring will compromise the outcome for the child, potentially leading to blindness or death. Lack of awareness of the risk for second tumors by the retinoblastoma survivor and the family may result in late diagnosis and inherent risks for blindness and death.

9.17.7  Related SOPs

OPHTH long term; CHEM long term; CHEM total body MRI; CHEM audio; RAD long term; GENE long term.

Acknowledgements  The authors wish to acknowledge Dr. Helen Dimaras for her assistance in editing this manuscript.

Supported in part by the Ontario Cancer Research Network (Project 02-Nov-0243).

•EUA for full retinal examination is required until the child is cooperative enough for a full dilatedexamination in clinic, and the risk is minimal for peripheral retina tumor recurrence or new retinoblastoma development.

•Eye examinations in the clinic include visual acuity assessment, refraction, ocular alignment, and evaluation for keratopathy, cataract, and vitreoretinal complications resulting from focal therapy or radiotherapy.

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