Yonsei Med J.  2007 Dec;48(6):1043-1047. 10.3349/ymj.2007.48.6.1043.

A Case of Cardiac Lymphangioma Presenting as a Cystic Mass in the Right Atrium

• Kim SJ
• Shin ES
• Kim SW
• Shin JK
• Cheong JP
• Kim YM
• Lee SG

• KMID: 1786223
• DOI: http://doi.org/10.3349/ymj.2007.48.6.1043

Abstract

A 44-year-old woman underwent surgery for an asymptomatic primary tumor of the heart located in the right atrium. The tumor was detected incidentally during follow-up computed tomography for a resected breast cancer. The mass, lying along the lower portion of the right atrial septum, was homogenous and cystic in nature, as detected by transthoracic and transesophageal echocardiography. Complete resection was performed via a median sternotomy under cardiopulmonary bypass. The postoperative course was uneventful. However, the histological result was surprising: the mass was a cardiac lymphangioma.

Keyword

Cystic lymphangioma; heart neoplasms; contrast echocardiography; right atrium

MeSH Terms

Adult
Breast Neoplasms/complications/pathology
Cysts/etiology/pathology
Echocardiography, Transesophageal
Female
Heart Atria
Heart Neoplasms/complications/*diagnosis/surgery
Humans
Lymphangioma/complications/*diagnosis/surgery
Myocardium/*pathology
Tomography, X-Ray Computed

Figure

• 

  Fig. 1 Enhanced computed tomography imaging showing a slightly enhancing homogenous round mass with low density in the right atrium.

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  Fig. 2 Transesophageal images from a bicaval view of the right atrium at 119°. (A) An encapsulated cystic mass (arrow heads) is noted in the lower portion of the right atrium but the boundary of the mass is not well-defined. (B) Contrast enhances the right atrium revealing a lobular mass that is not arising from the mid portion of the fossa ovalis. LA, left atrium; RA, right atrium; SVC, superior vena cava.

• 

  Fig. 3 Magnified transesophageal echocardiographic image, in the longitudinal plane at 87°. A cystic mass protrudes into the right atrium, and is attached to the atrial septum near the orifice of the inferior vena cava. Contrast with intravenous injection of agitated saline provided a clearer delineation of the mass. IVC, inferior vena cava; M, mass; RA, right atrium.

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  Fig. 4 Photomicrographs. (A) Collapsed and infolded cyst wall (H&E, × 40). (B) Higher power view of cyst wall structure, showing loose connective tissue stroma lined by flat endothelial cells beneath which is seen a lymphocytic infiltration (H&E, × 400). (C) The endothelial cells show a positive immunostaining for CD 34-related antigen.

Reference

1. Burke A, Virmani R. Tumors of the heart and great vessels: Atlas of tumor pathology series 3. 1996. vol 16. Washington, DC: Armed Forces Institute of Pathology;1–11.

2. Glasson MJ, Taylor SF. Cervical, cervicomediastinal and intrathoracic lymphangioma. Prog Pediatr Surg. 1991. 27:62–83.

3. Armstrong H, Monckeberg JG. Herzblock bedingt durch primaren Hertztumor, bei einem funf Jahrigen Kinde. Deutsch Arch Klin Med. 1911. 102:144–166.

4. Fontaliran F, Guillois B, Colin A, Blanc JJ, Chabaud JJ, Boog G, et al. Congenital atrioventricular block and maternal lupus erythematosus. Histologic discovery of tumor of the atrioventricular node. Arch Mal Coeur Vaiss. 1989. 82:609–613.

5. Nataf P, Mestiri T, Martin de Lasalle E, Benomar M, Gandjbakhch I, Cabrol C. Pericardial hemolymphangioma. Apropos of a case. Arch Mal Coeur Vaiss. 1988. 81:1137–1140.

6. Lymburner RM. Tumours of the heart: histopathological and clinical study. Can Med Assoc J. 1934. 30:368–373.

7. Anbe DT, Fine G. Cardiac lymphangioma and lipoma. Report of a case of simultaneous occurrence in association with lipomatous infiltration of the myocardium and cardiac arrhythmia. Am Heart J. 1973. 86:227–235.

8. Pasaoğlu I, Doğan R, Ozme S, Kale G, Bozer AY. Cardiac lymphangioma. Am Heart J. 1991. 121:1821–1824.

9. Daubeney PE, Ogilvie BC, Moore IE, Webber SA. Intrapericardial lymphangioma presenting as neonatal cardiac tamponade. Pediatr Cardiol. 1996. 17:129–131.

10. Jougon J, Laborde MN, Parrens M, MacBride T. Cystic lymphangioma of the heart mimicking a mediastinal tumor. Eur J Cardiothorac Surg. 2002. 22:476–478.

11. Kaji T, Takamatsu H, Noguchi H, Tahara H, Matsuda H, Nomura Y, et al. Cardiac lymphangioma: case report and review of the literature. J Pediatr Surg. 2002. 37:E32.

12. Miralles A, Bracamonte L, Soncul H, Diaz del Castillo R, Akhtar R, Bors V, et al. Cardiac tumors: clinical experience and surgical results in 74 patients. Ann Thorac Surg. 1991. 52:886–895.

13. Feigenbaum H, Armstrong WF, Ryan T. Masses, tumors, and source of embolus. Feigenbaum's echocardiography. 2005. 6th ed. Philadelphia: Lippincott Willians & Wilkins;708–710.

14. Burke AP, Virmani R. Cardiac myxoma. A clinicopathologic study. Am J Clin Pathol. 1993. 100:671–680.

15. Paniagua JR, Sadaba JR, Davidson LA, Munsch CM. Cystic tumour of the atrioventricular nodal region: report of a case successfully treated with surgery. Heart. 2000. 83:E6.

16. Saito S, Kobayashi J, Tagusari O, Bando K, Niwaya K, Nakajima H, et al. Successful excision of a cystic tumor of the atrioventricular nodal region. Circ J. 2005. 69:1293–1294.

17. Harrity PJ, Tazelaar HD, Edwards WD, Orszulak TA, Freeman WK. Intracardiac varices of the right atrium: a case report and review of the literature. Int J Cardiol. 1995. 48:177–181.

18. Remadi JP, BenAmar A, Baron O. Varix of the heart. Ann Thorac Surg. 2001. 72:264–265.

19. Burke A, Virmani R. Tumors of the heart and great vessels: Atlas of tumor pathology series 3. 1996. vol 16. Washington, DC: Armed Forces Institute of Pathology;87–88.

20. Ozdemir H, Kocakoc E, Bozgeyik Z, Cobanoglu B. Recurrent retroperitoneal cystic lymphangioma. Yonsei Med J. 2005. 46:715–718.

21. Cho BC, Kang SM, Lee SC, Moon JG, Lee DH, Lim SH. Primary idiopathic chylopericardium associated with cervicomediastinal cystic hygroma. Yonsei Med J. 2005. 46:439–444.

22. Icard P, Le Rochais JP, Galateau F, Jehan A, Martel B, Brun J, et al. Cystic lymphangioma of the mediastinum. Apropos of 3 cases, review of the literature. Ann Chir. 1998. 52:629–634.