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<p>Dedifferentiated chondrosarcoma (DDCS) is a rare and aggressive subtype of chondrosarcoma, characterized by the coexistence of a high-grade spindle or pleomorphic tumor that lacks a substantial cartilaginous matrix. Notably, it shows a mutant <i>IDH1</i> incidence of over 80%. This study established a novel DDCS cell line with an <i>IDH1</i> mutation, TK-DDCS1, derived from the right ilium of a 67-year-old Japanese female patient. TK-DDCS1 cells maintain the undifferentiated DDCS phenotype with the <i>IDH1</i>p.R132L mutation. The <i>IDH1</i>R132 mutation is known to be associated with a poor prognosis for chondrosarcoma, and the p.R132L mutation is a novel variant among the registered DDCS cell lines in the Cellosaurus database. The mutant <i>IDH1</i> inhibitor, DS-1001b, inhibited the proliferation of TK-DDCS1 in a dose-dependent manner in both two-dimensional and spheroid cultures. The tumorigenicity of TK-DDCS1 was demonstrated through xenografting into EGFP-transgenic BALB/c Rag2-/-/Jak3-/- (EGFP-BRJ) mice, where the tumors exhibited undifferentiated phenotypes of DDCS in both morphological and immunohistochemical features. Thus, TK-DDCS1 serves as a valuable model for investigating the characteristics of DDCS and exploring molecular targeted therapies.</p>

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Establishment and characterization of TK-DDCS1: a novel IDH1 mutated dedifferentiated chondrosarcoma cell line

  • 期刊论文

  • 发表日期：

    2025年6月3日

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  • Krittamate Saisuwan,
  • Piyanard Boonnate,
  • Hiroki Goto,
  • Rumi Nakagawa,
  • Makoto Abe,
  • Kaoru Hirabayashi,
  • Yukio Fujiwara,
  • Kazutaka Kikuta,
  • Seiji Okada

摘要

Dedifferentiated chondrosarcoma (DDCS) is a rare and aggressive subtype of chondrosarcoma, characterized by the coexistence of a high-grade spindle or pleomorphic tumor that lacks a substantial cartilaginous matrix. Notably, it shows a mutant IDH1 incidence of over 80%. This study established a novel DDCS cell line with an IDH1 mutation, TK-DDCS1, derived from the right ilium of a 67-year-old Japanese female patient. TK-DDCS1 cells maintain the undifferentiated DDCS phenotype with the IDH1p.R132L mutation. The IDH1R132 mutation is known to be associated with a poor prognosis for chondrosarcoma, and the p.R132L mutation is a novel variant among the registered DDCS cell lines in the Cellosaurus database. The mutant IDH1 inhibitor, DS-1001b, inhibited the proliferation of TK-DDCS1 in a dose-dependent manner in both two-dimensional and spheroid cultures. The tumorigenicity of TK-DDCS1 was demonstrated through xenografting into EGFP-transgenic BALB/c Rag2-/-/Jak3-/- (EGFP-BRJ) mice, where the tumors exhibited undifferentiated phenotypes of DDCS in both morphological and immunohistochemical features. Thus, TK-DDCS1 serves as a valuable model for investigating the characteristics of DDCS and exploring molecular targeted therapies.