Among the wide array of human neoplasms, primary tumors of bone are relatively uncommon and sundry group of solid tumors traditionally categorized according to their presumed mesenchymal differentiation. A locally aggressive or malignant group of cartilaginous matrix-producing neoplasms with diverse morphological features and clinical behavior require additional ancillary studies for prompt diagnosis and appropriate surgical treatment. They are histologically, behaviorally and genetically diverse, their pathogenesis is poorly understood Moreover treatment options are limited with surgical resection continuing to provide the only possibility of cure in many cases. However, there has been tremendous progress in the last decade in understanding the molecular pathogenesis of sarcoma, which may ultimately lead to more effective therapy and prognostification for these rare malignancies (1. Atypical cartilaginous tumor/grade1chondrosarcomas behave as locally aggressive lesions, and only metastasize in exceptional cases. Only a small percentage of the IDH1mutations can be identified using the specific IDHIRI32H antibody Histologic grade is the most important predictor of local recurrence and metastasis in chondrosarcoma , commonly patents die from locally recurrent tumor ofpelvis or scull, that is difficult to manage surgically Association between ratio of matrix metalloproteinaise- 1 and tissue inhibitor ofmetalloproteinase- 1, expression of metalloproteinase-1, -2 and-9, Col-IV, Cox-2, Bcl-2, Bax in context with histological and clinical data could play a significant role in determining prognosis in patients with borderline cartilaginous tumors. The mandatory application of multidisciplinary care in management of atypical cartilaginous tumor/grade 1 chondrosarcomas with integration of histologic, molecular, radiographic and clinical data is difficult to overestimate.