Retinoblastoma patients with high risk ocular pathological features: who needs adjuvant therapy?

Abstract

Aims: To describe the outcome of patients with non-metastatic unilateral retinoblastoma with high risk histopathological features after primary enucleation, and to clarify the need and results of adjuvant therapy. Patients and methods: From 1980 to 2001 adjuvant therapy was recommended only to patients with scleral involvement, post-laminar optic nerve involvement (PLONI) with either a positive margin or associated choroidal involvement, or (before 1994) isolated PLONI. Results: 108 of 224 patients had at least one high risk feature (choroidal, scleral, anterior chamber, and/ or PLONI). Patients with isolated choroidal (n = 55) or anterior chamber (n = 2) invasion, and most with PLONI without other risk factors (n = 21) were not treated; three relapsed but are long term survivors after intensive therapy. Four with isolated PLONI received adjuvant chemotherapy and none relapsed. Three of 11 with PLONI and concomitant choroidal or scleral involvement who received adjuvant therapy relapsed, versus two of four not treated. Two of five with scleral disease relapsed. All 12 with cut end involvement received adjuvant treatment and none relapsed. In the total group, all four patients who relapsed after adjuvant therapy died. Conclusions: Relapsing patients can be rescued with intensive therapy. Those with isolated choroidal or PLONI have a good prognosis without adjuvant therapy. Patients with PLONI with a positive margin have a good prognosis if treated with combined therapy. Those with scleral involvement or PLONI with concomitant choroid disease may benefit from adjuvant therapy. E ven though the event free survival for patients with nonmetastatic retinoblastoma is about 95% in industrialised countries, some patients suffer extraocular relapses that may lead to death, especially when the central nervous system (CNS) is involved. Several factors thought to be associated with a high risk for extraocular recurrence have been identified upon histopathological examination of enucleated eyes, including invasion of the post-laminar optic nerve, choroid, sclera and in some studies, the anterior chamber.

Clinical and Pathological Predictor Factors of Fellow Eye Affliction in Patients with an Initial Unilateral Retinoblastoma

“…When we investigated the possible relationship between tumor focality at diagnosis and the asynchronous bilateralization of retinoblastoma, we detected multifocal lesions in 1 (33.3%) of the 3 patients with asynchronous bilateralization of retinoblastoma (P > resection margin; P < 0.05] and poor tumor differentiation with no detectable rosettes or fleurettes [4 (80%) of 5 patients with poor tumor differentiation; P < 0.05], findings that are in agreement with those of several studies [5,11,12,15].…”

“…It seems that optic nerve invasion and poor tumor differentiation are more closely correlated with metastatic disease than to the metachronous bilateralization of the disease [10][11][12]. Furthermore, a statistically significant correlation was found between negative NSE staining and asynchronous bilateralization of retinoblastoma (P < 0.05).…”

A Histopathologic Analysis of Eyes Primarily Enucleated for Advanced Intraocular Retinoblastoma From a Developing Country

“…The incidence of choroidal invasion varies greatly in various reported series, ranging from 15.2% to 62%. 2,23,[25][26][27] We observed choroidal invasion in 47.4% of cases, with 24.6% of cases having massive involvement. Comparisons of optic nerve and choroidal invasion in other major series are given in Tables 3 and 4, respectively.…”

Postenucleation Adjuvant Chemotherapy With Vincristine, Etoposide, and Carboplatin for the Treatment of High-Risk Retinoblastoma

“…The significance of isolated anterior segment involvement remains debatable, but we have previously witnessed metastasis in such cases, so this was included as a factor. 10 Some authors 5,13 have suggested that anterior segment involvement and isolated choroidal invasion are not risk factors for metastasis. The other major source of variation is related to the definition of massive choroidal invasion.…”