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Unilateral cancer-associated retinopathy: diagnosis, serology and treatment

  • Clinical Case Report
  • Published:
Documenta Ophthalmologica Aims and scope Submit manuscript

Abstract

Purpose

To report a case of unilateral cancer-associated retinopathy (CAR) with clinical, serological and electroretinogram (ERG) normalization after aggressive cancer treatment combined with steroids and rituximab.

Methods

Work-up included extensive clinical and electrophysiological testing. Also, serological work-up for antiretinal antibodies and oncological screening was organized.

Results

A 45-year-old female presented with progressive photopsias, photophobia and relative central scotoma in the right eye since 6 weeks prior. BCVA was 1.0 in both eyes. Biomicroscopy, IOP and fundus exam were unremarkable. Also, colour vision, autofluorescence imaging, OCT and EOG were normal. Visual fields showed decreased central sensitivity in the right eye. ERG showed a unilateral, electronegative combined and ON-bipolar response. A diagnosis of CAR was suspected. After a diagnosis of an adenocarcinoma of the right ovary, radical ovariectomy and hysterectomy were performed, followed by adjuvant chemotherapy. A whole-body PET scan revealed no metastasis. Treatment with rituximab monoclonal antibodies in combination with corticosteroids was initiated. The patient tested positive for serum autoantibodies against TRPM1, a transient receptor potential cation channel expressed in ON-bipolar cells. During treatment, there was progressive improvement in symptoms and the ERG normalized. Serology confirmed complete clearance of autoantibodies.

Conclusions

Although rare, unilateral CAR does occur and in cases with high clinical suspicion an oncological work-up is mandatory. Aggressive cancer treatment combined with steroids and rituximab can lead to normalization of the clinical and ERG phenotype, with clearing of antiretinal antibodies.

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Acknowledgements

BPL is a senior clinical investigator of the Research Foundation—Flanders (Belgium) (FWO). The authors wish to thank Professors François DUHOUX, Oncologie Médicale, Jean-François BAURAIN, Oncologie Médicale, Jean SQUIFFLET, Gynécologie, and Dr. Nicolas JANIN, Centre de Génétique Humaine, Cliniques Universitaires Saint-Luc, Brussels, Belgium, for the clinical care of the patient.

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Authors and Affiliations

  1. Department of Ophthalmology, Ghent University Hospital, Ghent University, Ghent, Belgium

    Dimitri Roels & Bart P. Leroy

  2. Department of Ophthalmology, Nagoya University Graduate School of Medicine, Nagoya, Japan

    Shinji Ueno

  3. Department of Ophthalmology, Charleroi University Hospital Marie Curie, Charleroi, Belgium

    Cornelia D. Talianu

  4. Department of Ophthalmology, Saint Pierre and Brugmann University Hospitals, Brussels, Belgium

    Dafina Draganova

  5. Department of Ophthalmology, Mie University Graduate School of Medicine, Tsu, Japan

    Mineo Kondo

  6. Department of Ophthalmology, Center for Medical Genetics, Ghent University Hospital, Ghent University, Ghent, Belgium

    Bart P. Leroy

  7. Division of Ophthalmology and CCMT, The Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Bart P. Leroy

Authors
  1. Dimitri Roels
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  2. Shinji Ueno
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  3. Cornelia D. Talianu
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  4. Dafina Draganova
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  5. Mineo Kondo
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  6. Bart P. Leroy
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Corresponding author

Correspondence to Bart P. Leroy.

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Conflict of interest

All authors certify that they have no affiliations with or involvement in any organization or entity with any financial interest (such as honoraria; educational grants; participation in speakers’ bureaus; membership, employment, consultancies, stock ownership, or other equity interest; and expert testimony or patent-licensing arrangements) or nonfinancial interest (such as personal or professional relationships, affiliations, knowledge or beliefs) in the subject matter or materials discussed in this manuscript.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Declaration of Helsinki and its later amendments or comparable ethical standards.

Statement of human rights

All human rights were respected for the patient involved in this study.

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No animals were implicated in this study.

Informed consent

Informed consent was obtained from all individual participants included in the study.

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Roels, D., Ueno, S., Talianu, C.D. et al. Unilateral cancer-associated retinopathy: diagnosis, serology and treatment. Doc Ophthalmol 135, 233–240 (2017). https://doi.org/10.1007/s10633-017-9605-y

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  • DOI: https://doi.org/10.1007/s10633-017-9605-y

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