Osteosarcomas in retinoblastoma-survivors. A report of 28 affected patients from the Cooperative Osteosarcoma Study Group (COSS)
Bielack SS, Baumhoer D, Hecker-Nolting S, Hettmer S, Kager L, Ketteler P, Kevric M, Kratz CP, Kühne T, Mettmann V, Metzler M, Reinhardt D, Sorg B, Blattmann C (2024)
Publication Type: Journal article
Publication year: 2024
Journal
Book Volume: 3
Article Number: 100158
DOI: 10.1016/j.ejcped.2024.100158
Abstract
Purpose: Many publications address the epidemiology of secondary osteosarcomas following retinoblastoma. Treatment- and outcome-related information is, however, scarce. We used a large cooperative group's database to study this issue. Patients and methods: The database Cooperative Osteosarcoma Study Group COSS was searched for patients with osteosarcoma following a previous retinoblastoma. Patients were then analyzed for demographic factors, local/systemic treatments received, and outcomes. Results: 28 eligible patients were identified. Median age at retinoblastoma was.5 years, 89% occurred bilaterally. Retinoblastoma-therapy was by surgery in 26/27, radiotherapy in 26/27, chemotherapy in 10/26 (rest unknown). Osteosarcoma was diagnosed after 13.7 (3.1 – 36.1) years, extremities and head/neck affected in 14/28, each. Four/28 patients had primary metastases. Osteosarcoma treatment included chemotherapy in all cases, local therapy surgery in 27/28. Histological response was good in 9/16 evaluable cases. Surgery of all sites was macroscopically complete in 23/27 operated tumors and microscopically complete in 17/26 (1 unknown). Radiotherapy was administered to 3 craniofacial tumors. Median follow-up was 3.5 (.4 – 30.1) years from osteosarcoma diagnosis, 8/28 patients remaining event-free. Altogether, five patients suffered further secondary malignancies. Actuarial overall and event-free survival at 2 and 5 years from osteosarcoma were 73% (standard error: 8%) / 50% (10%) and 47% (10%) / 22% (9%), respectively. Conclusion: This comparatively large cohort of osteosarcomas after retinoblastoma proves that the latter may be treated curatively. While their prognosis is far worse than that of primary osteosarcomas, partly due to a predilection for craniofacial involvement, selected patients may still become long-term survivors with appropriate therapies.
Authors with CRIS profile
Markus Metzler
Professur für Kinder- und Jugendmedizin mit dem Schwerpunkt Pädiatrische Onkologie und Hämatologie
Involved external institutions
Klinikum Stuttgart
Germany (DE)
Universitätsspital Basel
Switzerland (CH)
Universitätsklinikum Freiburg
Germany (DE)
Medizinische Universität Wien
Austria (AT)
Universitätsklinikum Essen
Germany (DE)
Medizinische Hochschule Hannover (MHH) / Hannover Medical School
Germany (DE)
Universitäts-Kinderspital beider Basel (UKBB)
Switzerland (CH)
Germany (DE)
Universitätsspital Basel
Switzerland (CH)
Universitätsklinikum Freiburg
Germany (DE)
Medizinische Universität Wien
Austria (AT)
Universitätsklinikum Essen
Germany (DE)
Medizinische Hochschule Hannover (MHH) / Hannover Medical School
Germany (DE)
Universitäts-Kinderspital beider Basel (UKBB)
Switzerland (CH)
How to cite
APA:
Bielack, S.S., Baumhoer, D., Hecker-Nolting, S., Hettmer, S., Kager, L., Ketteler, P.,... Blattmann, C. (2024). Osteosarcomas in retinoblastoma-survivors. A report of 28 affected patients from the Cooperative Osteosarcoma Study Group (COSS). EJC Pediatric Oncology, 3. https://doi.org/10.1016/j.ejcped.2024.100158
MLA:
Bielack, Stefan S., et al. "Osteosarcomas in retinoblastoma-survivors. A report of 28 affected patients from the Cooperative Osteosarcoma Study Group (COSS)." EJC Pediatric Oncology 3 (2024).
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