Fiche publication
Date publication
février 2017
Journal
Pathology, research and practice
Auteurs
Membres identifiés du Cancéropôle Est :
Dr BARTHELEMY Philippe
,
Pr JACQMIN Didier
,
Pr LANG Hervé
,
Dr LINDNER Véronique
Tous les auteurs :
Bergerat S, Barthelemy P, Mouracade P, Lang H, Saussine C, Lindner V, Jacqmin D
Lien Pubmed
Résumé
The CIC-DUX4 sarcoma is a subset of the undifferentiated small round cell sarcoma family, presently recognized as a new clinicopathological entity. It is a rare and highly aggressive tumor usually arising in the soft parts of the limbs and the trunk. Only a very few cases of primitive visceral CIC-DUX4 have been hitherto described. We report the case of a 29 year-old male patient with a primary CIC-DUX4 sarcoma of the kidney with lung metastasis. The outcome of the disease was rapidly unfavorable. Despite radical nephrectomy, the patient experienced an early local retroperitoneal recurrence associated with lung and liver metastases. The tumor did not respond to four successive lines of chemotherapy nor to palliative radiotherapy. Due to partial morphologic and immunohistochemical overlap with Ewing sarcoma, CIC-DUX4 positive tumors have generally been considered as Ewing-like sarcomas and managed similarly. However, this tumor shows a high propensity to metastasize and is much less sensitive to chemotherapy than Ewing sarcomas. The management of this type of very aggressive sarcoma needs to be defined by comprehensive biological and clinical studies.
Mots clés
CIC-DUX4, Kidney sarcoma, Small round cell sarcoma
Référence
Pathol. Res. Pract.. 2017 Feb;213(2):154-160
