Intimal Sarcoma Represents the Most Common Primary Cardiac Sarcoma: A French National Retrospective Clinicopathologic and Molecular Study of 84 Cardiac Sarcomas. Implications for Diagnosis and Treatment
Agnes Neuville, Francois Collin, Patrick Bruneval, Marie Parrens, Francois Thivolet-Bejui, Anne Gomez-Brouchet, Philippe Terrier, Francois Piard, Vincent Thomas De Montpreville, Pauline Lagarde, Frederic Chibon, Jean-Michel Coindre. Institut Bergonié, Bordeaux, France; Centre Georges-François Leclerc, Dijon, France; CHU-Hôpital Européen Georges Pompidou, Paris, France; CHU-Hôpital Haut-Levêque, Bordeaux, France; CHU-Groupement Hospitalier Est, Lyon, France; CHU Rangueil, Toulouse, France; Institut Gustave Roussy, Paris, France; CHU de Dijon, Dijon, France; Centre Chirurgical Marie Lannelongue, Le Plessis Robinson, France
Background: Sarcomas constitute the majority of malignant primary cardiac tumors. Angiosarcomas are predominantly right-sided and other sarcomas (undifferentiated, leiomyosarcomas…) left-sided, whereas intimal sarcomas involve large vessels. We report novel molecular and pathologic features within the largest described series of primary cardiac sarcomas.
Design: 84 cases of sarcomas involving the heart were retrieved from the French national territory, between 1977 and 2010. Histological materials were centralized and reviewed. Complementary immunohistochemistry, FISH, qPCR, arrayCGH, RT-PCR were performed on paraffin embedded material or frozen sample if available.
Results: There were 56 women and 28 men, 48 years mean age (range 17-77). Right and left heart were respectively involved in 37 and 47 cases. There were 36 intimal sarcomas, 20 angiosarcomas, 18 unclassified sarcomas, 7 synovial sarcomas, 2 leiomyosarcomas and 1 PNET. All 20 angiosarcomas originated from the right heart, whereas 30 of the intimal sarcomas and 13 of the unclassified sarcomas were from the left heart. Before complementary techniques, intimal sarcomas were diagnosed as unclassified sarcomas, leiomyosarcomas, rhabdomyosarcomas, myxofibrosarcomas... MDM2 immunohistochemical overexpression was observed in all intimal sarcomas with MDM2 amplification, but also in 8/18 unclassified sarcomas without MDM2 amplification. Rhabdomyosarcomas were all intimal sarcomas. arrayCGH shows a complex genomic profile, with 12q13-14 amplicon, 4q12 amplicon and 9p21.3-p21.2 deletion.
Conclusions: Intimal Sarcoma involves the heart and appears in our series as the most common primary cardiac sarcoma, predominantly left-sided. MDM2 immunohistochemical overexpression is not sufficient to make this diagnosis. Molecular aberration should be proved. As resections are limited in left atrium, this histological subtype could benefit from therapies that target PDGFRA or MDM2.
Category: Bone & Soft Tissue
Monday, March 19, 2012 1:00 PM
Poster Session II # 36, Monday Afternoon