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

Abdulqader Al-Hebshi Abdulqader Al-Hebshi,Taleb Ismael Taleb Ismael

doi:10.30574/wjarr.2020.6.2.0141

Abdulqader Al-Hebshi Abdulqader Al-Hebshi, Taleb Ismael Taleb Ismael

Open Access

https://doi.org/10.30574/wjarr.2020.6.2.0141

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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

Highlights

Osteosarcoma is a tumor derived from mesenchymal cells that can be a consequence of primary causes or secondary causes
It has been shown that survival rate in patients with osteosarcoma and metastasis is 30% and that the rate lowers to 15% when osteosarcoma relapses [27]
Our results indicated that 2 syndromic patients that developed osteosarcoma were Rothmund-Thomson syndrome (RTS) patients

Summary

Introduction

Osteosarcoma is a tumor derived from mesenchymal cells that can be a consequence of primary causes (without any previous disease) or secondary causes (related to other pathologies). The aetiology of osteosarcoma is still partially known and has been described as multifactorial, including genetic alterations, environmental influence, and association with other diseases [1]. Osteosarcoma is the eighth cause of cancer in children and is the most common primary bone malignancy in children [3]. The incidence of osteosarcoma increases after 65 years old when it is usually originated by secondary causes, such as radiation exposure. Osteosarcoma is classified according to the Enneking system for staging malignant musculoskeletal tumors and American Joint Committee on Cancer system for bone sarcomas [4, 5]

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