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Peer-reviewedCC BY-NC-ND 4.0BMC Cancer

Alternative lengthening of telomeres and CINSARC are interconnected toward non-translocation-related sarcomas progression

  • Gaëlle Pérot1,2
  • Clémence Guerriau3,4
  • Natacha Roussel1
  • Emeline Sarot1
  • Carine Valle1
  • Pascal Pomiès5
  • Franck Tirode6
  • Delphine Poncet3,4
  • Frédéric Chibon1,7
7 affiliations
  1. 1Univ Toulouse, CNRS, Inserm, CRCT, 2 Avenue Hubert Curien, Toulouse, Cedex 1, 31037 France
  2. 2Centre Hospitalier Universitaire (CHU) de Toulouse, IUCT‐Oncopole, Toulouse, France
  3. 3Department of Pathology, Groupe Hospitalier Est, Hospices Civils de Lyon, Bron, France
  4. 4Pathophysiology and Genetics of Neuron and Muscle (PGNM), Institut Neuro Myo Gène (INMG), Université Claude Bernard Lyon 1, CNRS UMR 5261-INSERM U1315, Neuron-Muscle Interaction Team, Lyon, France
  5. 5PhyMedExp, Inserm, University of Montpellier, CNRS, Montpellier, France
  6. 6Cancer Research Center of Lyon, Centre Léon Bérard, University Claude Bernard Lyon 1, INSERM 1052, CNRS 5286, Lyon, France
  7. 7Department of Pathology, Oncopole Claudius Régaud, IUCT‐Oncopole, Toulouse, France
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Abstract

Alternative lengthening of telomeres (ALT) is a telomere elongation mechanism activated during oncogenesis and primarily acting in tumors of mesenchymal origin. Although the proteins involved in the machinery enabling ALT to elongate telomeres are becoming better understood, the underlying biology of this mechanism remains unclear. In the present study, we took advantage of a fully characterized cohort of 98 leiomyosarcomas (LMS) from the French Sarcoma Group to further our understanding of the ALT mechanism. We first compared the transcriptomic profiles of ALT+ and TERT+ LMS and demonstrated a strong enrichment of the CINSARC signature in ALT+ tumors. The establishment of an ALT+-related signature in these LMS confirmed the close association between CINSARC and ALT in two additional cohorts of non-translocation-related sarcomas. In vitro mesenchymal models of spontaneous ALT induction showed increased CINSARC expression following acquisition of the ALT mechanism. Conversely, ALT inactivation, through BLM inhibition, led to decreased CINSARC expression. These results establish CINSARC as a new hallmark of the ALT mechanism in non-translocation-related sarcomas and demonstrate the association of a cellular biological process with the CINSARC prognostic signature, namely the ALT mechanism.

Supplementary Information: The online version contains supplementary material available at https://doi.org/10.1186/s12885-026-16151-7.

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Cite

BibTeXRIS
BibTeX
@article{perot2026alternative,
  title = {{Alternative lengthening of telomeres and CINSARC are interconnected toward non-translocation-related sarcomas progression}},
  author = {Gaëlle Pérot and Clémence Guerriau and Natacha Roussel and Emeline Sarot and Carine Valle and Pascal Pomiès and Franck Tirode and Delphine Poncet and Frédéric Chibon},
  journal = {BMC Cancer},
  year = {2026},
  month = jun,
  doi = {10.1186/s12885-026-16151-7},
  url = {https://doi.org/10.1186/s12885-026-16151-7},
  note = {CC BY-NC-ND 4.0},
}
RIS
TY  - JOUR
TI  - Alternative lengthening of telomeres and CINSARC are interconnected toward non-translocation-related sarcomas progression
AU  - Gaëlle Pérot
AU  - Clémence Guerriau
AU  - Natacha Roussel
AU  - Emeline Sarot
AU  - Carine Valle
AU  - Pascal Pomiès
AU  - Franck Tirode
AU  - Delphine Poncet
AU  - Frédéric Chibon
PY  - 2026
DA  - 2026/06/09
JO  - BMC Cancer
DO  - 10.1186/s12885-026-16151-7
UR  - https://doi.org/10.1186/s12885-026-16151-7
AB  - Alternative lengthening of telomeres (ALT) is a telomere elongation mechanism activated during oncogenesis and primarily acting in tumors of mesenchymal origin. Although the proteins involved in the machinery enabling ALT to elongate telomeres are becoming better understood, the underlying biology of this mechanism remains unclear. In the present study, we took advantage of a fully characterized cohort of 98 leiomyosarcomas (LMS) from the French Sarcoma Group to further our understanding of the ALT mechanism. We first compared the transcriptomic profiles of ALT+ and TERT+ LMS and demonstrated a strong enrichment of the CINSARC signature in ALT+ tumors. The establishment of an ALT+-related signature in these LMS confirmed the close association between CINSARC and ALT in two additional cohorts of non-translocation-related sarcomas. In vitro mesenchymal models of spontaneous ALT induction showed increased CINSARC expression following acquisition of the ALT mechanism. Conversely, ALT inactivation, through BLM inhibition, led to decreased CINSARC expression. These results establish CINSARC as a new hallmark of the ALT mechanism in non-translocation-related sarcomas and demonstrate the association of a cellular biological process with the CINSARC prognostic signature, namely the ALT mechanism.  Supplementary Information: The online version contains supplementary material available at https://doi.org/10.1186/s12885-026-16151-7.
SN  - 1471-2407
ER  -