Results of a prospective study for the treatment of unilateral retinoblastoma

Abstract

The survival of patients with unilateral retinoblastoma was excellent and 60% were spared from adjuvant treatment. Our intensive regimen was likely to be effective for prevention of metastasis in patients with higher risk PRFs.

Strategies to manage retinoblastoma in developing countries

“…Isolated choroidal invasion is the typical example where a higher risk of extraocular relapse has been reported and adjuvant therapy has been recommended by some authors while others propose withholding adjuvant therapy [49]. According to the results of studies conducted in MICs, the relapse rate is near 3% [45]; therefore, the role of adjuvant chemotherapy would be difficult to estimate, even with adequately designed prospective studies. However, a prerequisite for safely withholding adjuvant therapy is a reliable histopathological staging of the enucleated eye.…”

“…Such cases are more frequent in developing countries, occurring in more than 50% of children in some MICs compared to developed countries where they usually occur in less than 20% of the enucleated eyes. Studies in many upper MICs have yielded encouraging results, with survival rates greater than 80% for patients with these conditions [23,45,46]. However, as opposed to other childhood malignancies, there are no randomized studies to support the treatment decisions in these cases.…”

“…However, the most effective regimen is not known. There are reports suggesting that more intensive regimens are more effective in preventing extraocular relapse [41,45,50] and the Children Oncology Group is performing an international study using an intensive regimen for patents with tumor invasion at the resection margin of the optic nerve. This is a common problem in developing countries that can be a consequence of advanced disease, when the tumor extends through the whole optic nerve length; but it also occurs when patients undergo enucleation in nonspecialized centers and an adequate optic nerve stump is not obtained during enucleation, thereby resulting in subsequent neoplastic invasion [51].…”

Successful treatment of metastatic retinoblastoma with high-dose chemotherapy and autologous stem cell rescue in South America

“…No adjuvant therapy was given to any patient, according to the local treatment protocols. 8,12 In four children (three with bilateral disease), metastatic disease developed after an initial conservative approach with chemoreduction was attempted. They had received a median of six cycles of chemoreduction with carboplatin, etoposide and vincristine, and all received external beam radiotherapy for conservative treatment.…”

“…As patients were treated in different institutions, there were no common guidelines; however, they received treatment with conventional chemotherapy with fairly similar regimens, including previously reported regimens, with alternating etoposide-carboplatin and vincristine, idarubicin and CY in five children, included in the Hospital JP Garrahan protocol. 8,12 The remaining children received this combination with topotecan in one, or ifosfamide with carboplatin and a topoisomerase inhibitor and doxorubicin in four or cisplatin, etoposide, CY and vincristine as per the COG-ARET 0321 regimen in one case.…”