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

• Published in: Pediatric blood & cancer 2020-01, Vol.67 (1), p.e27998-n/a
• Main Authors: Choucair, Marie‐Louise, Brisse, Hervé J., Fréneaux, Paul, Desjardins, Laurence, Dorfmüller, Georg, Puget, Stéphanie, Dendale, Rémi, Chevrier, Marion, Doz, François, Lumbroso‐Le Rouic, Livia, Aerts, Isabelle
• Format: Article
• Language: English
• Subjects: Antineoplastic Combined Chemotherapy Protocols - therapeutic use; Cancer; Chemotherapy; Child; Child, Preschool; Combined Modality Therapy; Computed tomography; Diagnosis; Disease Management; Enlargement; Enucleation; Female; Follow-Up Studies; Hematology; Humans; Infant; long‐term effects; Magnetic resonance imaging; Magnetic Resonance Imaging - methods; Male; Meninges; MRI; Neoplasm Invasiveness; NMR; Nuclear magnetic resonance; Oncology; Optic chiasm; Optic nerve; Optic Nerve - pathology; Optic Nerve Neoplasms - diagnostic imaging; Optic Nerve Neoplasms - pathology; Optic Nerve Neoplasms - therapy; Patients; Pediatrics; Prognosis; Radiation therapy; Remission; Retina; Retinal Neoplasms - diagnostic imaging; Retinal Neoplasms - pathology; Retinal Neoplasms - therapy; Retinoblastoma; Retinoblastoma - diagnostic imaging; Retinoblastoma - pathology; Retinoblastoma - therapy; Retrospective Studies; Surgery; Tomography, X-Ray Computed - methods; treatment; Tumors
• ISSN: 1545-5009; 1545-5017
• DOI: 10.1002/pbc.27998

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.