NCI Definition: A sarcoma that arises from the bone. Representative examples are osteosarcoma and chondrosarcoma. [1]

Bone sarcomas most frequently harbor alterations in TP53, IDH1, CDKN2A, CDKN2B, and RB1 [2].

Most Commonly Altered Genes in Bone Sarcoma

TP53 Mutation, IDH1 Mutation, IDH1 Codon 132 Missense, TP53 c.217-c.1178 Missense, and TP53 Missense are the most common alterations in bone sarcoma [2].

Top Alterations in Bone Sarcoma

Significant Genes in Bone Sarcoma

MLH1 +

MSH2 +

MSH6 +

PMS2 +

Disease Details

Osseous Sarcoma, Sarcoma of the Bone, Sarcoma of Bone, Skeletal Sarcoma
Ewing Sarcoma of Bone, Bone Liposarcoma, Bone Fibrosarcoma, Bone Osteosarcoma, Undifferentiated High Grade Pleomorphic Sarcoma of Bone, Bone Angiosarcoma, Bone Leiomyosarcoma, Chondrosarcoma, and Malignancy in Giant Cell Tumor of Bone


1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].

2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 8. This dataset does not represent the totality of the genetic landscape; see paper for more information.

3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.