Marie-Louise Choucair1, Hervé J Brisse2,3, Paul Fréneaux3,4, Laurence Desjardins3,5, Georg Dorfmüller6, Stéphanie Puget7,8, Rémi Dendale3,9, Marion Chevrier3,10, François Doz1,8, Livia Lumbroso-Le Rouic3,5, Isabelle Aerts1,3. 1. Oncology Center SIREDO: Care, Innovation and Research for Children, Adolescents and Young Adults with Cancer, Institut Curie, Paris, France. 2. Radiology Department, Institut Curie, Paris, France. 3. Paris Sciences et Lettres Research University, Paris, France. 4. Tumor Biology Department, Institut Curie, Paris, France. 5. Ocular Oncology, Institut Curie, Paris, France. 6. Pediatric Neurosurgery Department, Fondation Rothschild, Paris, France. 7. Department of Pediatric Neurosurgery, Necker Hospital, Paris, France. 8. Université Paris Descartes, Paris, France. 9. Radiation Oncology Department, Institut Curie, Paris, France. 10. Biostatistics Department, Institut Curie, Paris, France.
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.
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.
Authors: Michael Sullivan; Eric Bouffet; Carlos Rodriguez-Galindo; Sandra Luna-Fineman; Muhammad Saghir Khan; Pam Kearns; Douglas S Hawkins; Julia Challinor; Lisa Morrissey; Jörg Fuchs; Karen Marcus; Adriana Balduzzi; Luisa Basset-Salom; Miguela Caniza; Justin N Baker; Rejin Kebudi; Laila Hessissen; Richard Sullivan; Kathy Pritchard-Jones Journal: Pediatr Blood Cancer Date: 2020-05-13 Impact factor: 3.838