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Conservative management of retinoblastoma: Challenging orthodoxy without compromising the state of metastatic grace. "Alive, with good vision and no comorbidity".

  • Munier FL Jules-Gonin Eye Hospital, Fondation Asile des Aveugles, University of Lausanne, Lausanne, Switzerland. Electronic address: francis.munier@fa2.ch.
  • Beck-Popovic M Unit of Pediatric Hematology-Oncology, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland.
  • Chantada GL Hemato-Oncology Service, Hospital JP Garrahan, Buenos Aires, Argentina; Pediatric Hematology and Oncology, Hospital Sant Joan de Deu, Barcelona, Spain; Institut de Recerca Sant Joan de Deu, Barcelona, Spain.
  • Cobrinik D The Vision Center and The Saban Research Institute, Children's Hospital Los Angeles, Los Angeles, CA, USA; USC Roski Eye Institute, Department of Biochemistry & Molecular Medicine, Norris Comprehensive Cancer Center, Keck School of Medicine of the University of Southern California, Los Angeles, CA, USA.
  • Kivelä TT Department of Ophthalmology, Ocular Oncology and Pediatric Ophthalmology Services, Helsinki University Hospital, Helsinki, Finland.
  • Lohmann D Eye Oncogenetics Research Group, Institute of Human Genetics, University Hospital Essen, Essen, Germany.
  • Maeder P Unit of Neuroradiology, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland.
  • Moll AC UMC, Vrije Universiteit Amsterdam, Department of Ophthalmology, Cancer Center Amsterdam, Amsterdam, Netherlands.
  • Carcaboso AM Pediatric Hematology and Oncology, Hospital Sant Joan de Deu, Barcelona, Spain; Institut de Recerca Sant Joan de Deu, Barcelona, Spain.
  • Moulin A Jules-Gonin Eye Hospital, Fondation Asile des Aveugles, University of Lausanne, Lausanne, Switzerland.
  • Schaiquevich P Unit of Clinical Pharmacokinetics, Hospital de Pediatria JP Garrahan, Buenos Aires, Argentina; National Scientific and Technical Research Council (CONICET), Buenos Aires, Argentina.
  • Bergin C Jules-Gonin Eye Hospital, Fondation Asile des Aveugles, University of Lausanne, Lausanne, Switzerland.
  • Dyson PJ Institut des Sciences et Ingénierie Chimiques, Ecole Polytechnique Fédérale de Lausanne (EPFL), CH-1015, Lausanne, Switzerland.
  • Houghton S Jules-Gonin Eye Hospital, Fondation Asile des Aveugles, University of Lausanne, Lausanne, Switzerland.
  • Puccinelli F Interventional Neuroradiology Unit, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland.
  • Vial Y Materno-Fetal Medicine Unit, Woman-Mother-Child Department, University Hospital of Lausanne, Switzerland.
  • Gaillard MC Jules-Gonin Eye Hospital, Fondation Asile des Aveugles, University of Lausanne, Lausanne, Switzerland.
  • Stathopoulos C Jules-Gonin Eye Hospital, Fondation Asile des Aveugles, University of Lausanne, Lausanne, Switzerland.
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  • 2019-06-08
Published in:
  • Progress in retinal and eye research. - 2019
Complication
Intra-arterial chemotherapy
Intracameral chemotherapy
Intravitreal chemotherapy
Liquid biopsy
Metastasis
Retinoblastoma
Treatment
Antineoplastic Agents
Comorbidity
Conservative Treatment
Eye Enucleation
Humans
Infusions, Intra-Arterial
Intravitreal Injections
Neoplasm Recurrence, Local
Quality of Life
Retinal Neoplasms
Retinoblastoma
Visual Acuity
English Retinoblastoma is lethal by metastasis if left untreated, so the primary goal of therapy is to preserve life, with ocular survival, visual preservation and quality of life as secondary aims. Historically, enucleation was the first successful therapeutic approach to decrease mortality, followed over 100 years ago by the first eye salvage attempts with radiotherapy. This led to the empiric delineation of a window for conservative management subject to a "state of metastatic grace" never to be violated. Over the last two decades, conservative management of retinoblastoma witnessed an impressive acceleration of improvements, culminating in two major paradigm shifts in therapeutic strategy. Firstly, the introduction of systemic chemotherapy and focal treatments in the late 1990s enabled radiotherapy to be progressively abandoned. Around 10 years later, the advent of chemotherapy in situ, with the capitalization of new routes of targeted drug delivery, namely intra-arterial, intravitreal and now intracameral injections, allowed significant increase in eye preservation rate, definitive eradication of radiotherapy and reduction of systemic chemotherapy. Here we intend to review the relevant knowledge susceptible to improve the conservative management of retinoblastoma in compliance with the "state of metastatic grace", with particular attention to (i) reviewing how new imaging modalities impact the frontiers of conservative management, (ii) dissecting retinoblastoma genesis, growth patterns, and intraocular routes of tumor propagation, (iii) assessing major therapeutic changes and trends, (iv) proposing a classification of relapsing retinoblastoma, (v) examining treatable/preventable disease-related or treatment-induced complications, and (vi) appraising new therapeutic targets and concepts, as well as liquid biopsy potentiality.
Language
  • English
Open access status
green
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Persistent URL
https://sonar.ch/global/documents/126281
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