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It is particularly difficult to confirm the diagnosis when a sarcoma lacking cartilaginous component occurs at a distant location from the primary lesion. The patient was a 72\u2010year\u2010old woman with multiple lesions in the pelvis, lungs, and liver, 18\u2009months after resection of grade 2 central chondrosarcoma of the sternum. Imaging showed no cartilage component in any location. Although a needle biopsy from the pelvic region confirmed the diagnosis as high\u2010grade sarcoma without a cartilage component, it was difficult to distinguish between a new primary sarcoma and metachronous metastatic lesions from patient's known prior dedifferentiated chondrosarcoma. We therefore performed a comparative molecular analysis by whole\u2010exome sequencing of the biopsy sample and the resected sternal central chondrosarcoma. Both lesions had no <jats:italic>IDH1\/2<\/jats:italic> mutations but shared 19 somatic mutations and wide\u2010range chromosomal losses, indicating similar origin. This case illustrates the challenge is coupling a diagnosis of metastatic dedifferentiated chondrosarcoma when no chondroid component is evident. Our study also highlights the benefit of genomic analysis in this differential diagnosis, especially in the context of dedifferentiated chondrosarcoma lacking <jats:italic>IDH<\/jats:italic>1\/2 mutations.<\/jats:p>","DOI":"10.1002\/gcc.23204","type":"journal-article","created":{"date-parts":[[2023,9,21]],"date-time":"2023-09-21T11:59:43Z","timestamp":1695297583000},"page":"755-760","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["<scp>IDH<\/scp>\u2010negative chondrosarcoma with metachronous dedifferentiation only in the metastatic site\u2014A diagnostic pitfall"],"prefix":"10.1002","volume":"62","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4000-6891","authenticated-orcid":false,"given":"Tetsuya","family":"Sekita","sequence":"first","affiliation":[{"name":"Department of Musculoskeletal Oncology National Cancer Center Hospital  Tokyo Japan"},{"name":"Department of Clinical Genomics National Cancer Center 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