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Biologie moléculaire des sarcomes des tissus mous

Molecular biology of soft tissue sarcoma

  • Published:
Oncologie

Résumé

Les sarcomes des tissus mous (STM) regroupent des tumeurs de différentes origines histologiques, ayant une agressivité variable et dont le diagnostic, déterminant sur l’efficacité de la prise en charge du patient, reste parfois délicat à établir. Hétérogènes également sur le plan génétique, ces tumeurs peuvent être regroupées selon la compréhension que nous avons de leur génétique: environ 40 % des sarcomes sont caractérisés par une altération spécifique, translocation ou mutation. Les 60 % restant ne présentent pas de remaniements spécifiques. Dans ce groupe nous pouvons distinguer d’une part, environ 20 % des sarcomes indifférenciés qui présentent une génétique simple très similaire à celle observée dans les liposarcomes bien différenciés; et, d’autre part, les leiomyosarcomes, rhabdomyosarcomes pléomorphes, liposarcomes pléomorphes et les sarcomes pléomorphes indifférenciés ou histocytofibromes malins (MFH) notamment, qui sont génétiquement très instables et pour lesquels aucun remaniement spécifique n’a pu être mis en évidence. L’ensemble de ces caractérisations génétiques font maintenant partie, grâce au développement des technologies, du diagnostic moléculaire des sarcomes des tissus mous.

Abstract

Soft tissue sarcoma includes tumours of various histological origins, with variable aggressiveness, and whose diagnosis, which determines treatment effectiveness, sometimes remains difficult to establish. Heterogeneous on the genetic level, these tumours can be classified according to our understanding of their molecular genetics: approximately 40% of sarcomas are characterized by a specific translocation or mutation. The remaining 60% have no specific modifications. In this group, approximately 20% are undifferentiated sarcomas with very simple genetics similar to differentiated lpiposarcomas, while leiomyosarcomas, pleomorphic rhabdomyosarcomas, pleomorphic liposarcomas, undifferentiated pleomorphic sarcomas, and, in particular, malignant fibrous histocytomas (MFH) are genetically very unstable, no specific rearrangement having been identified. Thanks to a number of technological advances, all of these genetic characteristics play a role in the molecular diagnosis of soft tissue sarcoma.

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Authors and Affiliations

  1. Département de pathologie, institut Bergonié, 229, cours de l’Argonne, F-33000, Bordeaux, France

    F. Chibon

  2. Pathologie moléculaire des cancers, U509, institut Curie, 26, rue d’Ulm, F-75248, Paris, France

    A. Aurias

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  1. F. Chibon
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  2. A. Aurias
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Correspondence to F. Chibon.

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Chibon, F., Aurias, A. Biologie moléculaire des sarcomes des tissus mous. Oncologie 9, 88–96 (2007). https://doi.org/10.1007/s10269-006-0559-z

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  • DOI: https://doi.org/10.1007/s10269-006-0559-z

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