Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2021 Jun;68(6):e28963.
doi: 10.1002/pbc.28963. Epub 2021 Mar 15.

Adjuvant therapy of histopathological risk factors of retinoblastoma in Europe: A survey by the European Retinoblastoma Group (EURbG)

Sabine Dittner-Moormann  1 , Madlen Reschke  2 , Floor C H Abbink  3 , Isabelle Aerts  4 , Hatice Tuba Atalay  5 , Nadezhda Fedorovna Bobrova  6 , Eva Biewald  7 , Ines B Brecht  8 , Shani Caspi  9 , Nathalie Cassoux  4 , Guilherme Castela  10 , Yelena Diarra  1 , Catriona Duncan  11 , Martin Ebinger  8 , David Garcia Aldana  12 , Doris Hadjistilianou  13 , Tomáš Kepák  14 , Artur Klett  15 , Hayyam Kiratli  16 , Erika Maka  17 , Enrico Opocher  11   18 , Katarzyna Pawinska-Wasikowska  19 , Jelena Rascon  20 , Ida Russo  21 , Olga Rutynowska-Pronicka  22 , Constantino Sábado Álvarez  23 , Sonsoles San Roman Pacheco  24 , Karel Svojgr  25 , Beate Timmermann  26   27 , Vicktoria Vishnevskia-Dai  28 , Angelika Eggert  2 , Petra Ritter-Sovinz  29 , Nikolaos E Bechrakis  7 , Helen Jenkinson  30 , Annette Moll  31 , Francis L Munier  32 , Maja Beck Popovic  33 , Guillermo Chantada  34 , François Doz  4 , Petra Ketteler  1   27
Affiliations
Free article

Adjuvant therapy of histopathological risk factors of retinoblastoma in Europe: A survey by the European Retinoblastoma Group (EURbG)

Sabine Dittner-Moormann et al. Pediatr Blood Cancer. 2021 Jun.
Free article

Abstract

Introduction: Advanced intraocular retinoblastoma can be cured by enucleation, but spread of retinoblastoma cells beyond the natural limits of the eye is related to a high mortality. Adjuvant therapy after enucleation has been shown to prevent metastasis in children with risk factors for extraocular retinoblastoma. However, histological criteria and adjuvant treatment regimens vary and there is no unifying consensus on the optimal choice of treatment.

Method: Data on guidelines for adjuvant treatment in European retinoblastoma referral centres were collected in an online survey among all members of the European Retinoblastoma Group (EURbG) network. Extended information was gathered via personal email communication.

Results: Data were collected from 26 centres in 17 countries. Guidelines for adjuvant treatment were in place at 92.3% of retinoblastoma centres. There was a consensus on indication for and intensity of adjuvant treatment among more than 80% of all centres. The majority of centres use no adjuvant treatment for isolated focal choroidal invasion or prelaminar optic nerve invasion. Patients with massive choroidal invasion or postlaminar optic nerve invasion receive adjuvant chemotherapy, while microscopic invasion of the resection margin of the optic nerve or extension through the sclera are treated with combined chemo- and radiotherapy.

Conclusion: Indications and adjuvant treatment regimens in European retinoblastoma referral centres are similar but not uniform. Further biomarkers in addition to histopathological risk factors could improve treatment stratification. The high consensus in European centres is an excellent foundation for a common European study with prospective validation of new biomarkers.

Keywords: RB1 gene; biomarker; chemotherapy; childhood cancer; metastasis; radiotherapy.

PubMed Disclaimer

References

REFERENCES

    1. Temming P, Arendt M, Viehmann A, et al. How eye-preserving therapy affects long-term overall survival in heritable retinoblastoma survivors. J Clin Oncol. 2016;34(26):3183-3188.
    1. Lumbroso-Le Rouic L, Aerts I, Hajage D, et al. Conservative treatment of retinoblastoma: a prospective phase II randomized trial of neoadjuvant chemotherapy followed by local treatments and chemothermotherapy. Eye (Lond). 2016;30(1):46-52.
    1. Gunduz AK, Mirzayev I, Temel E, et al. A 20-year audit of retinoblastoma treatment outcomes. Eye (Lond). 2020;34(10):1916-1924.
    1. Dunkel IJ, Krailo MD, Chantada GL, et al. Intensive multi-modality therapy for extra-ocular retinoblastoma (RB): a Children's Oncology Group (COG) trial (ARET0321). J Clin Oncol. 2017;35(15_suppl):10506-10506.
    1. Luna-Fineman S, Chantada G, Alejos A, et al. Delayed enucleation with neoadjuvant chemotherapy in advanced intraocular unilateral retinoblastoma: AHOPCA II, a prospective, multi-institutional protocol in Central America. J Clin Oncol. 2019;37(31):2875-2882.

Publication types