[Cardiac glomus tumor: An unusual localization of pericytic (perivascular) tumor]. / Tumeur glomique intracardiaque : une tumeur péricytaire (périvasculaire) de localisation inhabituelle.

Glomus tumor are rare mesenchymal neoplasm, belonging to the pericytic (perivascular) tumor family, witch recent molecular characterization has allowed highlight recurrent molecular abnormalities. In fact, glomus tumor involves frequent MIR143-NOTCH gene fusion whereas others pericytic tumor (myopericytoma and myofibroma) involve mutations of PDGFRB gene. Glomus tumor are usually developed in superficial localization. However visceral locations have been described. Cardiac location is exceptional with only one case reported in literature. Here, we report the case of cardiac glomus tumor (glomangiomyoma) developed in the left ventricle in a 34 year-old patient, diagnosed after chest pain. The length of tumor was 4cm in greatest dimension. Histologically, the tumor concerned both round glomus cells and smooth muscle cells with prominent branching thin-walled vessels. By immunohistochemistry, these two contingents exhibited diffuse expression of smooth muscle actin and heterogeneous expression of H-caldesmone whereas cytokeratins, melanocytic markers and chomogranine were negative. Next Generation molecular analysis using RNA sequencing highlighted the characteristic MIR143-NOTCH gene fusion witch supports the diagnosis of glomus tumor. In this observation, we recall histological and immunohistochemistry features of glomus tumor and we make a synthesis concerning the molecular data recently described in sporadic glomus tumor.

[Cardiac fibroma: A rare cause of sudden child death]. / Le fibrome cardiaque : une cause rare de mort subite de l'enfant.

We report the case of a 3-year-old child who died from the consequences of a cardio-respiratory arrest despite reanimation procedures. Echocardiography and magnetic resonance imaging (MRI) revealed a mass of the free wall of the left ventricle. Autopsy confirmed the existence of a solitary myocardial tumor, well-circumscribed, firm, with a whitish and trabeculated cut surface. Histologically, the tumor consisted of bundles of spindle-shaped and regular cells mingling with collagen and elastic fibers, insinuating themselves between myocytes in periphery. Calcifications were present. After immunohistochemistry, the cells were highlighted by anti-actin smooth muscle antibody; but they were not highlighted by anti-desmin, anti-ß catenin and anti-Ki67 antibodies. The diagnosis of cardiac fibroma was made. The primary cardiac tumors of child are rare and usually benign. They are essentially represented by rhabdomyoma and fibroma. Cardiac fibroma mostly occurs during the first year of life. It can be revealed by cardiac insufficiency, arrhythmia, chest pain or sudden death.

[Fatal outcome of complete resection of undifferentiated pleomorphic cardiac sarcoma : About a case]. / Évolution fatale d'une résection complète d'un sarcome cardiaque pléomorphe indifférencié : à propos d'un cas.

INTRODUCTION: Undifferentiated pleomorphic sarcoma (UPS) is a very rare malignant primary cardiac tumor with a poor prognosis. OBSERVATION: A 34-year-old woman complained of dyspnea evolving in a febrile context with altered general condition: Transthoracic echocardiography (TTE) revealed a left intra-atrial mass measuring 51/26 mm in diameter. A thoracic computed tomography (CT) revealed a mass occupying the left atrium measuring 42/28/34 mm, associated with two pulmonary metastases. The cardiac mass was resected and the postoperative suites were fatal. Histological diagnosis was UPS. CONCLUSION: We present an atypical case of invasive UPS, which was surgically resected and scheduled for radio-chemotherapy with a fatal outcome.

[Heart imaging by CT-scan and MRI in cardiac tumors]. / L'imagerie en coupe dans le bilan des tumeurs intra-cardiaques.

The etiology of cardiac masses is often oncological or thrombotic, rarely inflammatory. Among heart tumors, the vast majority are metastatic. We describe the most frequent benign primary cardiac tumors and the most frequent malignant primary cardiac tumors and give information about the advantages of using a multi-modality approach for the accurate diagnosis of a cardiac mass using Computed Tomography Scanner and Magnetic Resonance Investigation.

Unusual cause of dyspnea: Cardiac fusiform cells carcinoma.

This manuscript illustrates an unusual cause of dyspnea. Cardiac tumor are uncommon, especially fusiform cells carcinoma.

[Undifferentiated sarcoma: usefulness of multimodality cardiac imaging in characterizing a rare intracardiac mass]. / Sarcome cardiaque indifférencié : intérêt de l'imagerie multimodale dans la caractérisation d'une masse intracardiaque.

We report the case of a man presenting with a Pierre Marie-Bamberger syndrome. This paraneoplastic syndrome revealed an undifferentiated intracardiac sarcoma. This case emphasizes the need for multimodality imaging to characterize intracardiac tumor.