Risk of metastasis and orbital recurrence in advanced retinoblastoma eyes treated with systemic chemoreduction versus primary enucleation.

BACKGROUND: The purpose of this study was to evaluate the risk of metastatic disease and orbital recurrence in advanced retinoblastoma treated with systemic chemoreduction versus primary enucleation. METHODS: A retrospective review of patients with Group D/E retinoblastoma was conducted with data collection from 1995 to 2015. Overall, 345 eyes (294 patients) were included (165 Group D and 180 Group E). Primary outcome measures were orbital recurrence and metastatic disease. RESULTS: Of the 345 eyes, 139 were treated with systemic chemoreduction (102 Group D, 37 Group E) and 206 with primary enucleation (63 Group D, 143 Group E). In the chemoreduction group, one patient developed metastasis (0.7%) and one an orbital recurrence (0.7%). In the primary enucleation group, two patients developed metastases (0.9%) and one an orbital recurrence (0.5%). After systemic chemoreduction, 58 of the 139 eyes (30 Group D, 28 Group E) were secondarily enucleated for treatment failure (41.7%). The median time to secondary enucleation from diagnosis was 8.1 months. None of the eyes in the systemic chemoreduction group had high-risk pathologic features. In the primary enucleation group, 56 eyes had high-risk pathology. CONCLUSION: Over a 20-year period, 345 eyes were treated for advanced retinoblastoma at Children's Hospital Los Angeles. Incidence of orbital recurrence and metastatic disease was <1% and did not vary by treatment modality or group classification. None of the eyes enucleated for treatment failure had high-risk pathology, and none of these patients developed metastatic disease. Globe salvage therapy with systemic chemoreduction and subsequent enucleation for poor response does not increase the risk of metastatic disease or orbital recurrence.

Remote orbital recurrence of olfactory neuroblastoma (esthesioneuroblastoma).

Olfactory neuroblastoma is a rare and often locally aggressive malignancy that invades the orbit via local destruction. It is known to recur in a delayed fashion, particularly to the neck lymph nodes. This is a case of a 65-year-old gentleman who presents with recurrence in the orbit and a neck lymph node 19 years after treatment for his initial disease. This report describes the longest known interval in orbital recurrence and should alert the monitoring physician that extreme delays in recurrence can occur.

Evisceration and ocular tumors: What are the consequences?

PURPOSE: Evisceration can be performed for blind, painful eyes. This surgery can promote the dissemination of tumor cells within the orbit if an ocular tumor has been missed preoperatively. METHODS: We reviewed the medical records of patients who were eviscerated for blind, painful eyes between 2009 and 2014 and who were referred after the surgery to the Institut Curie or the Rothschild Foundation in Paris. We included the patients with a histological diagnosis of ocular tumor or orbital recurrence. Cytogenetic analysis was performed whenever possible. RESULTS: Four patients turned out to have an ocular tumor after evisceration (two choroidal melanomas, a rhabdoid tumor and an adenocarcinoma of the retinal pigment epithelium); two had a history of prior ocular trauma. The tumors were diagnosed either on histological analysis of the intraocular contents (2 patients) or biopsy of orbital recurrence (2 patients). Prior to evisceration, fundus examination was not performed in 3 patients. One had preoperative imaging but no intraocular tumor was suspected. At the time of this study, 3 patients had had an orbital recurrence and died. We also found 2 patients who had an evisceration despite a past history of choroidal melanoma treated with proton beam therapy. CONCLUSION: We showed that evisceration of eyes with unsuspected ocular malignancies was associated with a poor prognosis due to orbital recurrence and metastasis. The evisceration specimen should therefore always be sent for histological analysis in order to perform prompt adjuvant orbital radiotherapy if an ocular tumor is found.