First Author: S.Boeckx BELGIUM
Co Author(s): E. Biewald S. Schluter S. Goricke P. Temming D. Lohmann N. Bornfeld
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The purpose of this study is to evaluate the incidence, location and treatment of a late recurrent retinoblastoma after a tumour free period of three years.
All patients were treated and followed for retinoblastoma at the University Hospital of Essen, Germany.
It’s a retrospective review of patients who are presenting with a recurrence of retinoblastoma between 2006 and 2016 after a successful treatment of the initial tumour and inactivity of the tumour for at least 3 years. All retinoblastomas were categorized according the international classification of retinoblastoma (ICRB classification) and all patients underwent a MRI of the brain and orbit to exclude metastasis to the brain. Every patient with newly diagnosed or recurrence retinoblastoma was discussed in a tumour board with paediatrics, radiologists and ophthalmologists to define the best treatment. According the size and localization of the tumour and according the age of the patient, the tumour was treated with lasercoagulation, cryocoagulation, intravitreal Melphalan, intraarterial Melphalan, radiation with Ruthenium, enucleation, proton beam radiation or systemical Chemotherapy. After therapy, follow-ups every 2-4 weeks were done, until inactivity of the tumour was seen.
Three patients with late recurrence of retinoblastoma were seen, which count for less than 0.01%. All of them were bilateral diseased at the time of first diagnosis, and underwent an enucleation of one eye by initial treatment. Considering the eye of recurrence retinoblastoma, all initial retinoblastomas were categorized in a different ICRB group and therefore underwent a different initial therapy. Patient 1 had an ICRB-A retinoblastoma wherefore laser- and cryocoagulation therapy was done. Patient 2 had an ICRB-D retinoblastoma and underwent 6 cycles of systemic chemotherapy. Patient 3 had an ICRB-D wherefore a percutaneous radiation therapy was done. Al tumours were inactive after first treatment. After respectively 5, 6 and 3 years of inactivity, in all of these patients a recurrence of the retinoblastoma was seen. This late recurrence was treated different in all patients: Patient 1 was treated with 6 times of intravitreal Melphalan injection and radiation therapy with Ruthenium, patient 2 underwent 3 times intraarterial Melphalan injection and a Proton beam radiation, patient 3 became a treatment with 6 cycles of systemic chemotherapy, 1 time intraarterial Melphalan injection and a radiation therapy with Ruthenium.
Follow-up controls are very important to diagnose a retinoblastoma recurrence early, even years after initial and successful treatment. Late recurrence of a retinoblastoma is a rare but serious complication, which requires often an aggressive therapy. There is no standard or optimal treatment in case of recurrence, but every case should be discussed individual to find the best therapy.