Syndromic osteosarcoma, does it carry a poor prognosis? King Hussein Cancer Center experience. A case series report with literature review
1 Hematology & Oncology Division ,Department of Pediatric , Prince Mohammad bin Abdulaziz Hospital (PMBAH), Ministry of National Guard-Health Affairs (MNG-HA), Medina, Saudi Arabia.
2 Hematology Oncology Division ,Department of Pediatric, King Hussein Cancer Center (KHCC), Amman, Jordan.
Research Article
World Journal of Advanced Research and Reviews, 2020, 06(02), 152-162
Article DOI: 10.30574/wjarr.2020.6.2.0141
Publication history:
Received on 03 May 2020; revised on 16 May 2020; accepted on 18 May 2020
Abstract:
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<0.05). Our results suggest that syndromic patient’s show a poorer prognosis and osteosarcoma should be suspected during differential diagnosis and follow up of their syndromes. Early diagnosis and treatment might improve the outcome and survival.
Keywords:
Osteosarcoma; Rothmund Thomson Syndrome; Osteopoikilosis; Osteogenesis imperfecta; Diamond-Blackfan Anemia.
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