#chondrosarcoma

“This Little Piggy” (2019) by Beau White

Chondrosarcoma of the finger as a result of Ollier’s disease. Ollier disease is a rare condition that results in benign intraosseous cartilaginous tumours (called enchondroma) forming near growth plate cartilage. In this particular case the chondrosarcoma (a form of bone cancer) may have resulted from an untreated enchondroma, and ultimately the second finger required amputation.

From Ancient Greek μέσος (middle of, between, amidst, half), ἔγχυμα (infusion, lymph, humor), χόνδρος (lump, cartilage, grain), σαρκός, genitive of σάρξ (flesh, body) and -ωμα (suffix indicating action and processing, in this case tumor, disease)

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Chondrosarcoma is a rare form of cancer that originates in the bones and cartilage. It accounts for approximately 20% of all bone tumors and is commonly found in adults between the ages of 30 and 60. Chondrosarcoma poses significant challenges in terms of treatment and diagnosis, and patients often face a long and difficult journey in finding effective therapies.

Mutations in isocitrate dehydrogenase 1 (IDH1) and IDH2 are found in a subset of benign and malignant cartilage tumors, gliomas and leukaemias. The mutant enzyme causes the production of D-2-hydroxyglutarate (D-2-HG), affecting CpG island and histone methylation. While mutations in IDH1/2 are early events in benign cartilage tumors, we evaluated whether these mutations play a role in malignant chondrosarcomas. Compared to IDH1/2 wildtype cell lines, chondrosarcoma cell lines harboring an endogenous IDH1 (n=3) or IDH2 mutation (n=2) showed up to a 100-fold increase in intracellular and extracellular D-2-HG levels. Specific inhibition of mutant IDH1 using AGI-5198 decreased levels of D-2-HG in a dose dependent manner. After 72 hours of treatment one out of three mutant IDH1 cell lines showed a moderate decrease in viability , while D-2-HG levels decreased >90%. Likewise, prolonged treatment (up to 20 passages) did not affect proliferation and migration. Furthermore, global gene expression, CpG island methylation as well as histone H3K4, -9, and -27 trimethylation levels remained unchanged. Thus, while IDH1/2 mutations cause enchondroma, malignant progression towards central chondrosarcoma renders chondrosarcoma growth independent of these mutations. Thus, monotherapy based on inhibition of mutant IDH1 appears insufficient for treatment of inoperable or metastasized chondrosarcoma patients.