Syndromic osteosarcoma, does it carry a poor prognosis? King Hussein Cancer Center experience. A case series report with literature review

Osteosarcoma is a tumor derived from mesenchymal cells. Osteosarcoma mainly affects children of 10-14 years old, usually of primary aetiology. Some genetic disorders have been shown to increase osteosarcoma incidence. The aim of our study was to investigate the association of osteosarcoma with syndromic features and the potential effects in the prognosis of osteosarcoma. A case series report of six cases with different syndromes was reported in this study and we have retrospectively evaluated the prognosis of the cases. Data was collected from January 2003 till December 2011 at King Hussein Cancer Center in Jordan. A total of 69 patients were diagnosed to have osteosarcoma during the studied period, 6 of them having associated syndromes, constituting 8.7% of all cases. The syndromes identified were Rothmund-Thomson Syndrome, Osteogenesis Imperfecta, Diamond-Blackfan Anemia, Osteopoikilosis, and Cockayne Syndrome. From the 63 non-syndromic patients follow up was lost in 14 of them. Among the remaining 49 patients, 34 (69.3%) were alive and 15 (30.6%) died, while among the syndromic patients one was lost for follow up, only one remained alive (20%), and 4 died (80%). The statistical analysis indicated that syndromic patients presented a poorer prognosis than non-syndromic patients since an association with a higher mortality rate was observed (p