Primary Bone Cancers: Latest Trends in Pathogenesis, Diagnosis and Management

  • Nursuffi Rathiyah binti Mohd Hizam International Medical School, Management and Science University (MSU), University Drive, Off Persiaran Olahraga, 40100 Shah Alam, Selangor, Malaysia
  • Kaswinna Malar A/P Ananthan International Medical School, Management and Science University (MSU), University Drive, Off Persiaran Olahraga, 40100 Shah Alam, Selangor, Malaysia
  • Nur Zahrin Syahirah binti Raman International Medical School, Management and Science University (MSU), University Drive, Off Persiaran Olahraga, 40100 Shah Alam, Selangor, Malaysia
  • Kishore Gopal Banerjee Corresponding author: Senior Lecturer, Department of Pathology, International Medical School, Management & Science University, 40100 Shah Alam, Selangor, Malaysia
Keywords: Primary bone tumour, osteosarcoma, Ewing sarcoma, chondrosarcoma, pathogenesis, treatment, diagnosis


Primary bone tumours arising from the bony tissue are further classified into three groups; bone-producing, cartilage-producing, and tumours of unknown origin. In this review article, we have analyzed the latest trends in the pathogenesis, diagnosis, and management of the key primary malignant tumours (osteosarcoma, chondrosarcoma, and Ewing sarcoma) of bony origin. The Hippo-Yap signaling pathway is a newly discovered pathogenesis for osteosarcoma, and the inhibition contributes to the treatment of the malignancy. Moreover, carbon-ion particle therapy is proven to be a remedy for radioresistant tumours. The advancement of diagnosis methods for osteosarcoma is hastened with the identification of biomarker microRNA, liquid biopsy, and ‘Omic’ technology. Chondrosarcoma has a different and tailored approach to manage patients. Due to the unknown pathogenesis, treatments are being considered holistically depending on the patients’ age, overall health, medical history, type and stages of chondrosarcoma, the severity of the carcinoma, and patients' preference or opinion on the treatment plans. It has no specific treatment, and the ideal goal is to remove the mass and reduce the likelihood of relapse.  Lastly, for Ewing sarcoma, there is a variety of new targeted therapy such as Lysine-specific demethylase-1(LSD-1), PRKCB, Poly(ADP-Ribose)polymerase1 inhibitors(PARBP1)  have been developed to treat recurrent ES, and a large dose of chemotherapy with autologous hematopoietic stem cell has been identified to cause toxicity in Ewing sarcoma. The origin of Ewing sarcoma remained unknown, but the rearrangement of the gene is thought to be the cause of tumorigenesis of Ewing sarcoma.


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