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Diagnosis and management of secondary epipapillary retinoblastoma
  1. Ido Didi Fabian1,2,
  2. Francesco Puccinelli3,
  3. Marie-Claire Gaillard1,
  4. Maja Beck-Popovic4,
  5. Francis L Munier1
  1. 1Unit of Pediatric Ocular Oncology, Jules-Gonin Eye Hospital, University of Lausanne, Lausanne, Switzerland
  2. 2Ocular Oncology Service, Goldschleger Eye Institute, Sheba Medical Center, Tel-Aviv University, Tel Aviv, Israel
  3. 3Department of Radiology, Interventional Neuroradiology Unit, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland
  4. 4Unit of Pediatric Hematology-Oncology, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland
  1. Correspondence to Professor Francis L Munier, Unit of Pediatric Ocular Oncology, Jules-Gonin Eye Hospital, Avenue de France 15, Lausanne 1004, Switzerland; francis.munier{at}fa2.ch

Abstract

Background Reports on retinoblastoma relapse at the optic nerve head (ONH) are anecdotal and include only treatments by external beam radiotherapy (EBRT) or enucleation. We aimed to describe such cases, termed secondary epipapillary retinoblastoma, diagnosed and monitored with the assistance of hand-held spectral domain optical coherence tomography (HHSD-OCT) and treated with intraophthalmic artery chemotherapy (IAC) and/or intravitreous chemotherapy (IViC).

Methods A retrospective analysis of secondary epipapillary retinoblastoma cases treated conservatively.

Results Four males and two females were included, diagnosed with secondary epipapillary retinoblastoma at a median time of 8.6 months (mean 24.0) from initial retinoblastoma diagnosis. HHSD-OCT was used in all cases for accurate diagnosis; in 2/6, the epipapillary relapse was detected only by means of HHSD-OCT. Treatments for secondary epipapillary retinoblastoma included IAC and IViC (n=4), IAC alone (n=1) and IViC alone (n=1). HHSD-OCT demonstrated complete epipapillary tumour regression in all cases, achieved in a median time of 1.6 months (mean 1.8). The median time from secondary epipapillary retinoblastoma resolution to last visit was 29.2 months (mean 27.5). At last visit, all eyes were tumour-free and no cases of metastasis recorded.

Conclusions Cases of retinoblastoma relapse at the ONH show common clinical features and represent specific diagnostic and therapeutic challenge; hence, we propose to consider this condition as a subset of retinoblastoma, termed secondary epipapillary retinoblastoma. HHSD-OCT is an invaluable diagnostic tool in the initial diagnosis as well as in monitoring these lesions, and IAC and IViC are efficient modalities for this clinical scenario, obviating the need for EBRT or enucleation.

  • Imaging
  • Neoplasia
  • Optic Nerve
  • Retina
  • Treatment other
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Footnotes

  • Contributors IDF and FP had substantial contribution to the design of the work, collection and analysis of the data, drafting the work, final approval of the version published and agrees to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. M-CG had substantial contribution to the acquisition, collection and analysis of the work, drafting the work, final approval of the version published and agrees to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. MB-P had substantial contribution to the collection and analysis of the data, drafting the work, final approval of the version published and agrees to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved, and FLM had substantial contribution to the design of the work and analysis of the data, significantly drafting the work, final approval of the version published and agrees to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

  • Competing interests None declared.

  • Ethics approval The Swiss Federal Department of Health (authorisation # 035.0003-48) in accordance with the tenets of the Declaration of Helsinki.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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