Abstract
Purpose
The course of osteosarcoma patients primarily treated as such has been well described. Little, however, is known about patients who were primarily treated assuming a different tumor diagnosis.
Methods
The database of the Cooperative Osteosarcoma Study Group COSS was searched (4.435 primary high-grade central osteosarcomas registered prior to 01/01/21). A different tumor entity had to have been assumed for at least one month after the initial diagnostic procedure before the correct diagnosis of osteosarcoma was finally made. Identified patients were analyzed for demographic, tumor-, and treatment-related factors as well as for survival outcomes.
Results
37 patients were identified. They were a median of 19.7 (2.7—60.4) years old at first presentation and were more likely to be females than males (23:14). Bone cysts (n = 8), giant cell tumor of bone (n = 6), and osteoblastoma (n = 6) were the most frequent of 29/37 (78%) benign, chondrosarcoma and its variants (n = 6) the most frequent of 8/37 (22%) malignant original diagnoses. Tumors affected the extremities in 23 (62%), the trunk in 11 (30%), and the craniofacial bones in 3 (8%). Only one patient received systemic treatment while assuming the different diagnosis (1/37, 3%). The median time until the correct diagnosis of osteosarcoma was made was 8 months (range: 1 month–14.1 years). At that time, 6/37 (16%) presented with metastatic disease. All patients went on to receive chemotherapy, 17/37 (46%) neo-adjuvantly. Histologic response was only evaluated in 13/17 (76%) patients and was good (< 10% viable tumor) in only 4/13 (31%) patients. In 31/37 (84%) patients, a surgically complete resection of all macroscopically identified tumor manifestations could be achieved. Five-year overall and event-free survival rates at 5 years were 50.2% (standard error: 8.6%) and 42.6% (8.5%), respectively.
Conclusion
Osteosarcoma may initially be misdiagnosed and hence subjected to inappropriate treatment including misguided surgery. Once diagnosed correctly, some of the affected patients may still be cured if finally treated according to modern osteosarcoma standards.
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Acknowledgements
We thank all patients and parents as well as the staff of the participating institutions.
Funding
The studies from which these patients originate were supported by Deutsche Krebshilfe, Deutsche Forschungsgemeinschaft, Fördergemeinschaft Kinderkrebs-Zentrum Hamburg, and Förderkreis krebskranke Kinder Stuttgart.
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Stefanie Hecker-Nolting reports grants from Förderkreis krebskranke Kinder Stuttgart e.V., during the conduct of the study; grants from EISAI, grants from German Childhood Cancer Foundation, outside the submitted work. Leo Kager reports personal fees from Bayer, personal fees from Novartis, personal fees from Amgen, personal fees from Agios, all outside the submitted work. Stefan Bielack reports personal fees from Eli Lilly, personal fees from Ipsen, personal fees from Hoffmann-La Roche, personal fees from Bayer Healthcare, personal fees from Boehringer-Ingelheim, personal fees from EISAI, and personal fees from MAP Biopharma, all outside the submitted work. Daniel Baumhoer, Claudia Blattmann, Thomas Kühne, Matthias Kevric, Susanna Lang, Vanessa Mettmann, Benjamin Sorg, and Mathias Werner do not report any potential conflicts of interest.
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Hecker-Nolting, S., Baumhoer, D., Blattmann, C. et al. Osteosarcoma pre-diagnosed as another tumor: a report from the Cooperative Osteosarcoma Study Group (COSS). J Cancer Res Clin Oncol 149, 1961–1967 (2023). https://doi.org/10.1007/s00432-022-04156-1
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DOI: https://doi.org/10.1007/s00432-022-04156-1