Management of advanced uni‐ or bilateral retinoblastoma with macroscopic optic nerve invasion

Abstract

Background Retinoblastoma with macroscopic optic nerve (ON) invasion depicted by imaging at diagnosis remains a major problem and carries a poor prognosis. We sought to describe the treatment and outcome of these high‐risk patients. Methods Retrospective mono‐institutional clinical, radiological, and histological review of patients with uni‐ or bilateral retinoblastoma with obvious ON invasion, defined by radiological optic nerve enlargement (RONE) depicted by computed tomography scan or magnetic resonance imaging (MRI), was performed. Results Between 1997 and 2014, among the 936 patients with retinoblastoma treated at Institut Curie, 11 had detectable RONE. Retinoblastoma was unilateral in 10 and bilateral in one. Median age at diagnosis was 28 months (range, 11‐96). ON enlargement extended to the orbital portion in three patients, to the optic canal in five, to the prechiasmatic portion in two, and to the optic chiasm in one. Nine patients received neoadjuvant chemotherapy and partial response was obtained in all. Enucleation was performed in 10/11 patients—by an anterior approach in three and by anterior and subfrontal approaches in seven. Three patients had a positive ON resection margin (2/3 after primary enucleation). All enucleated patients received adjuvant treatment (conventional chemotherapy: 10, high‐dose chemotherapy: seven, radiotherapy: five). Leptomeningeal progression occurred in four patients. Seven are in first complete remission (median follow up: 8 years [3.5‐19.4]). Conclusion Neoadjuvant chemotherapy and microscopic complete resection have a pivotal role in the management of retinoblastoma with RONE. MRI is recommended for initial and pre‐operative accurate staging. Surgery should be performed by neurosurgeons in case of posterior nerve invasion. Radiotherapy is required in case of incomplete resection.

The COVID‐19 pandemic: A rapid global response for children with cancer from SIOP, COG, SIOP‐E, SIOP‐PODC, IPSO, PROS, CCI, and St Jude Global

“…Given the likely resource constraints for interventional radiology and prolonged anesthesia, services should consider deferring intraarterial chemotherapy programs. In resource‐limited settings, most patients with advanced intraocular disease and no salvageable vison will require immediate enucleation to control disease followed by systemic chemotherapy 33,36 . We recommend standard post‐enucleation chemotherapy without dose modification as an outpatient 37 .…”

“…When diagnosed early and treated in a well-resourced setting, retinoblastoma is nearly always cured, so the paramount considerations are the preservation of vision, the preservation of the globe, and the determination of a genetic predisposition. 6,[33][34][35][36] However, in many LMIC settings, retinoblastoma presents with advanced disease, with extraocular extension and local or distant metastasis, which carry a poor prognosis. 37,38 A pandemic-adapted approach to the diagnosis and management of intra-and extraocular retinoblastoma in various resource settings is discussed in Supplement I and listed in Supporting Information Table S4.…”

“…In resourcelimited settings, most patients with advanced intraocular disease and no salvageable vison will require immediate enucleation to control disease followed by systemic chemotherapy. 33,36 We recommend standard post-enucleation chemotherapy without dose modification as an outpatient. 37 Guidance is provided in Supplement I and Supporting…”

Plumbagin Induces Cytotoxicity via Loss of Mitochondrial Membrane Potential and Caspase Activation in Metastatic Retinoblastoma

“…The long-term survival rates of patients with metastatic RB remain unsatisfactory, mainly a result of invasion and metastasis (5). The frequency of metastatic RB ranges from 4.8 to 11% (6,7) and it remains major contributor to mortality and is associated with life-long poor prognosis (6,(8)(9)(10)(11). Frequent common sites of extraocular RB include the orbit, pre-auricular nodes, bones, central nervous system and liver, and each of these may influence the outcome of metastatic RB.…”

“…Frequent common sites of extraocular RB include the orbit, pre-auricular nodes, bones, central nervous system and liver, and each of these may influence the outcome of metastatic RB. For example, RB diagnosis with orbital invasion is associated with a 10-to 27fold higher risk of metastasis when compared to cases without orbital extension (10)(11)(12). The clinical presentations are quite variable and depend on the site or sites involved.…”