A Primary Epithelioid Hemangioendothelioma of the Right Atrium: Report of a Case and Literature Review

Tsutomu Sugimoto, Kazuo Yamamoto, Shinpei Yoshii

Department of Cardiovascular Surgery, Tachikawa Medical Center, Nagaoka, Japan.

DOI: 10.4236/ojts.2013.32013                

Abstract

Primary cardiac epithelioid hemangioendothelioma (EHE) is extremely uncommon. To our knowledge, only fourteen cases have been reported. We report a case of a primary EHE of the right atrium which incidentally detected in 77-year-old woman. Echocardiograhpy revealed a 26 × 16 mm right atrial mass attached to the base of the interatrial septum and moderate regurgitation of the tricuspid valve. The tumor and stalk were completely resected from the interatrial septum with a partial resection of the septal leaflet of the tricuspid valve. The patient had an uneventful postoperative course and no signs of recurrence nor metastasis of EHE 4 years after operation. Because of malignant potential of EHE, a longer period of close follow up is necessary.

Keywords

Epithelioid Hemangioendothelioma; Heart; Right Atrium

Conflicts of Interest

The authors declare no conflicts of interest.

References

[1]	N. P. Ohri, S. A. Yousem, E. Sonmez-Alpan and T. V. Colby, “Estrogen and Progesterone Receptors in Lymphangioleiomyomatosis, Epithelioid Hemangioendothelioma, and Sclerosing Hemangioma of Lung,” American Journal of Clinical Pathology, Vol. 96, No. 4, 1991, pp. 529-535.
[2]	R. H. Hayward and F. L. Korompai, “Endothelioma of the Mitral Valve,” The Annals of Thoracic Surgery, Vol. 28, No. 1, 1979, pp. 87-89. doi:10.1016/S0003-4975(10)63398-X
[3]	K. Singal, “Intracardiac Mass in a Young Woman with a History of Brain Tumor,” Chest, Vol. 92, No. 2, 1987, pp. 337-341. doi:10.1378/chest.92.2.337
[4]	D. Marchiano, F. Fisher and S. Hofstetter, “Epihtlioid Hemangioendothelioma of the Heart with Distant Metastases. A Case Report and Literature Review,” The Journal of Cardiovascular Surgery, Vol. 34, No. 6, 1993, pp. 529-533.
[5]	F. Bille’-turk, R. Padovani, R. Rosario, D. Heckenroth, M. Gracia, G. Duport, P. Bergeron and J. Bille, “Hemangioendothelioma of the Aortic Valve Revealed by Transient Ischemic Episodes,” La Presse Médicale, Vol. 22, No. 38, 1993, p. 1928.
[6]	T. Tansel, U. Aydogan, D. Yilmazbayhan, B. Bilgic, M. Demiryount and E. Onursal, “Epitheloid Hemangioendothelioma of the Heart in Infancy,” The Annals of Thoracic Surgery, Vol. 79, No. 4, 2005, pp. 1402-1405. doi:10.1016/j.athoracsur.2003.10.053
[7]	J. F. Val-Bernal, E. Gracia-Alberdi, J. A. Gutierrez and M. F. Garijo, “Incidental in Vivo of an Epithelioid Hemangioendothelioma of the Mitral Valve,” Pathology International, Vol. 55, 2005, pp. 644-648. doi:10.1111/j.1440-1827.2005.01883.x
[8]	H. Kitamura, H. Okabayashi, M. Hanyu, Y. Soga, T. Nomoto and H. Johno, “Successful Enucleation of a Giant Cardiac Hemangioendothelioma Showing an Unusual Proliferation Pattern,” The Journal of Thoracic and Cardiovascular Surgery, Vol. 130, No. 4, 2005, pp. 1199-1201. doi:10.1016/j.jtcvs.2005.05.018
[9]	M. Lisy, W. Beierlein, H. Muller, B. Bultmann and G. Ziemer, “Left Atrial Epithelioid Hemangioendothelioma,” The Journal of Thoracic and Cardiovascular Surgery, Vol. 133, No. 3, 2007, pp. 803-804. doi:10.1016/j.jtcvs.2006.09.076
[10]	N. Moulai, O. Chavanon, L. Guillou, M. Noirclerc, D. Blin and E. Brambilla, “Atypical Primary Epithelioid Hemangioendothelioma of the Heart,” Journal of Thoracic Oncology, Vol. 1, No. 2, 2006, pp. 188-189. doi:10.1097/01243894-200602000-00017
[11]	J. Safirstein, S. Aksenov and F. Smith, “Cardiac Epithelioid Hemangioendothelioma with 8-Year Follow up,” Cardiovascular Pathology, Vol. 16, No. 3, 2007, pp. 183-186. doi:10.1016/j.carpath.2006.10.003
[12]	P. Messias, J. Bernardo and M. J. Antunes, “Primary Left Atrial Hemangioendothelioma,” Interactive CardioVasc Thoracic Surgery, Vol. 7, No. 5, 2008, pp. 945-946. doi:10.1510/icvts.2008.184085
[13]	R. S. Cotran, V. Kumar and S. L Robbins, “Tumors of Blood Vessels,” In: W B. Saunders, Ed., Robbins Pathologic Basis of Disease, WB Saunders Company, Philadelphia, 1989, pp. 587-592.
[14]	S. W. Weiss, K. G. Ishak, D. H. Dail, D. E. Sweet and F. M. Enzinger, “Epithelioid Hemangioendothelioma and Related Lesions,” Seminars in Diagnostic Pathology, Vol. 3, No. 4, 1986, pp. 259-287.
[15]	F. M. Enziger and S. W. Weiss, “Benign Tumors and tumorlike Lesions of Blood Vessels,” In: F. M. Enziger and S. W. Weiss, Eds., Soft Tissue Tumors, 2nd Edition, Mosby, St Louis, 1988, pp. 489-580.
[16]	B. R. De Young, H. F. Frierson Jr., M. N. Ly, D. Smith and P. E. Swanson, “CD31 Immunoreactivity in Carcinomas and Mesotheliomas,” American Journal of Clinical Pathology, Vol. 110, No. 3, 1998, pp. 374-377.
[17]	T. Mentzel, A. Beham, E. Calonje, D. Katenkamp and C. D. Fletcher, “Epihtelioid Hemangioendothelioma of Skin and Soft Tissues: Clinicopathologic and ImmunohistoChemical Study of 30 Cases,” The American Journal of Surgical Pathology, Vol. 21, No. 4, 1997, pp. 363-374. doi:10.1097/00000478-199704000-00001
[18]	S. W. Weiss and F. M. Enzinger, “Epithelioid Hemangioendothelioma: A Vascular Tumor Often Mistaken for Carcinoma,” Cancer, Vol. 50, No. 5, 1982, pp. 970-981. doi:10.1002/1097-0142(19820901)50:5<970::AID-CNCR2820500527>3.0.CO;2-Z