Les sarcomes cardiaques primitifs sont extrêmement rares et de pronostic sombre. L’existence du sarcome intimal, tumeur mésenchymateuse maligne affectant principalement l’artère pulmonaire et l’aorte, est rapportée au niveau du cœur mais reste encore débattue. Nous rapportons le cas d’un sarcome intimal développé chez une patiente de 70 ans, avec ses caractéristiques macroscopiques, microscopiques et moléculaires.

Primary cardiac sarcomas are rare tumors with poor prognosis. Intimal sarcoma, a mesenchymal malignant tumor described mainly in the great vessels, may rarely involve the heart. Herein we describe the case of a 70-years-old female who was found to have a left atrial mass during an investigation of a new onset dyspnea. The patient underwent surgery and the resected mass was found to be an intimal sarcoma. The objectives of this report were to describe a case of this rare disease entity and to discuss its pathological and molecular findings based on relevant literature.