Objectives: To describe the outcome of patients with bilateral or unilateral retinoblastoma with high-risk histopathological features managed at a tertiary hospital in the Philippines. Methods: This was a descriptive, retrospective review of retinoblastoma cases with high-risk histopathological characteristics seen at a tertiary hospital from January 1999 to July 2012. Out of 239 patients, those with at least one of the following features were included in the study: positive cut optic nerve margin, postlaminar optic nerve involvement (PLONI), intra- or extrascleral involvement, choroidal invasion, and anterior segment involvement. We determined the number of patients who developed orbital recurrence and metastasis, otherwise known as events, within 1 year after enucleation or exenteration and compared the outcomes between those who received and did not receive adjuvant therapy. Results: Of the 82 eyes of 79 patients with high-risk histopathologic features, 25.6% had orbital recurrence and 18.3% developed central nervous system, lymph node, or distant metastasis. None with isolated choroidal involvement (n=24) or combined choroidal and anterior segment involvement (n=4) developed recurrence or metastasis. Patients with isolated anterior segment (n=1) and PLONI with negative margin involvement (n=1) remained event-free with chemotherapy. The following developed orbital recurrences: 2 of 9 patients with combined choroid and scleral involvement, and 1 of 8 patients with PLONI negative margin and choroidal involvement. These patients did not receive immediate postoperative chemotherapy. Two out of 4 patients with isolated PLONI with positive margin of resection developed metastasis despite adjuvant therapy. Of 12 patients with combined choroid, scleral, or anterior segment and PLONI with positive margin involvement, only 3 of the 7 patients who received full chemotherapy remained event-free, while the rest developed orbital recurrence or metastasis (9 of 12). Of 17 patients with extrascleral involvement and varying degrees of intraocular involvement and optic nerve margin involvement, only 3 of the 9 who received full chemotherapy remained event-free, including two who received an intensive chemotherapy course. Conclusions Isolated choroidal and combined choroid and anterior segment involvement had no recurrences or metastasis either with or without adjuvant therapy. Patients with combined choroid and scleral involvement and those with PLONI, negative margin with choroidal involvement had better outcomes with adjuvant therapy than without. Patients with the following features developed events despite adjuvant therapy: positive optic nerve margin combined with any ocular tissue involvement, and extrascleral involvement combined with any other feature. We recommend giving a timely and complete chemotherapy regimen to prevent recurrence or metastasis in combined choroid and scleral involvement, and PLONI negative margin with choroidal involvement. We suggest intensive chemotherapy for patients with positive margin involvement or extrascleral involvement. Further studies are recommended to establish the need for chemotherapy in isolated PLONI.
Retinoblastoma