ICVTS Click here to locate an Ethicon representative
HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
QUICK SEARCH:   [advanced]


     

This Article
Right arrow Abstract Freely available
Right arrow Full Text (PDF)
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to Personal Folders
Right arrow Download to citation manager
Right arrow Permission Requests
Citing Articles
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Skopin, I.I.
Right arrow Articles by Sazonenkov, M.A.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Skopin, I.I.
Right arrow Articles by Sazonenkov, M.A.
Related Collections
Right arrow Cardiac - other
Interactive Cardiovascular and Thoracic Surgery 2:316-318(2003)
© 2003 European Association of Cardio-Thoracic Surgery

Case report - Cardiac general

Primary rabdomyosarcoma of the right atrium

I.I. Skopina,b,*, R.A. Serova,b, A.A. Makushina,b and M.A. Sazonenkova,b

a Department of Acquired Valve Diseases, Bakoulev Scientific Center for Cardiovascular Surgery, St. Rubliovskoe Shosse 135, Moscow, Russia
b Department of Pathology, Bakoulev Scientific Center for Cardiovascular Surgery, St. Rubliovskoe Shosse 135, Moscow, Russia

* Corresponding author. Tel.: +7-95-414-7965; fax: +7-95-414-7976
sazonenkov{at}email.ru

Received November 21, 2002; received in revised form March 7, 2003; accepted March 11, 2003


   Abstract
Top
 Abstract
1. Introduction
 2. Case report
 3. Discussion
 References
 
Rabdomyosarcoma is the rare malignant tumor of the heart. A case of a large malignant rabdomyosarcoma in a 37 year old woman is presented. The tumor arose from the anterior wall of the right atrium and was treated by surgical resection with replacement of the tricuspid valve with bioprosthesis. The patient passed two courses of adjuvant chemotherapy. Six months later transthoracic echocardiography revealed absence of any tissue masses in right atrium, superior vena cava and normal function of the bioprosthesis.

Key Words: Rabdomyosarcoma; Malignant neoplasm; Cardiac tumors


   1. Introduction
Top
 Abstract
 1. Introduction
2. Case report
 3. Discussion
 References
 
Primary tumors of the heart are rare. They occur with the frequency of 0.001–0.28% of which 75% are benign and 25% are malign in origin [1].

Rabdomyosarcoma is the rare malignant tumor of the heart. It localizes mainly in the right atrium [2], however can occur in the interventricular septum [3]. This tumor is characterized with intraluminal growth, expressed invasion in myocard and cardiac valves.

A case of a large malignant rabdomyosarcoma arising from the anterior wall of the right atrium treated by surgical resection is presented.


   2. Case report
Top
 Abstract
 1. Introduction
 2. Case report
3. Discussion
 References
 
In January 2002, a 37 year old woman was admitted to the hospital because of exertional dispnea, palpitations, periferal oedema and liver enlargement, that have appeared and increased for the past 2 months.

On auscultation any murmures were not found. Transesophageal 2-D echocardiography and magnetic nuclear resonance tomography revealed a huge mass inside the right atrium. The mass was fixed to the anterior wall, filled almost all atrium and extended into the superior vena cava (Fig. 1).



View larger version (174K):
[in this window]
[in a new window]
  		Fig. 1 Preoperative magnetic nuclear resonance tomography revealed a huge tissue mass in right atrium. Ao: aorta, PA: pulmonary artery, LV: left ventricle, RA: right atrium, TM: tissue mass.

 
For excision of the mass cardiopulmonary bypass was instituted with moderate hypothermia, topical cooling and antegrade cold cardioplegia. For the venous drenage inferior vena cava, brachiocephal vena cannulas and suction from pericardium were used. Central venous pressure was monitored through the catheter in vena cava inferior.

Through the right atriotomy a large wide base was found attached to the anterior wall. The mass expanded into the superior vena cava up to the brachiocephal vein with almost complete occlusion of the vessel and in the anterior cusp of the tricuspid valve. The tumor was solid, encapsulated, yellowish, had irregular surface and dimension 9x8x7 cm (Fig. 2a). The stalk of the tumor was excised with the part of the anterior wall. After extracting the mass from the superior vena cava the vein cannula was transferred into it from the brachiocephal vein. Then the tricuspid valve was completely excised and replaced with bioprosthesis. The right atrium defect was closed with the xenopericardial patch. Micrographic examination (Fig. 2b) revealed giant and fusiform cells and capillary vessels. Immunomorphological analysis with DAKO antibodies showed the presence of vimentin, desmin, myogenin in the resected specimen and proved diagnosis of rabdomyosarcoma.



View larger version (136K):
[in this window]
[in a new window]
  		Fig. 2 (a) Macroscopic view of the tumor. Parts of the neoplasm which were locater in RA (1); and in vena cava superior (2). Septal (3);, posterior (4); and anterior (5) leaflets of the TV. (b). Microscopic specimen of the tumor with giant cells (gc), fusiform cells (fc) and capillary vessels (cv).

 
Six months later transthoracic echocardiography revealed absence of any tissue masses in right atrium, superior vena cava and normal function of the bioprosthesis.


   3. Discussion
Top
 Abstract
 1. Introduction
 2. Case report
 3. Discussion
References
 
Primary malignant tumors of the heart are rare. Holley [1] reported their frequency 25% of all primary cardiac neoplasm. However, Blondeau [4] has reported the frequency of malignant tumors 10%. Department of Pathology of the Bakoulev Scientific Center for Cardiovascular Surgery dispose the material of 267 clinical cases of primary neoplasm of which 28 (10.4%) were malignant.

This tumor is characterized with intraluminal growth, expressed invasion in myocard and cardiac valves and rapid obturation of the lumen. In the practice of our center we have met five cases of rabdomiosarcomas of the heart. Four of them occurred in right atrium and one in the left atrium. In our case we also met with onset and progress of clinical symptoms during 2 months.

For the revealing of intracardiac tumors echocardiography and magnetic resonance imaging are used. Transesophageal echocardiography is superior than transthoracic as it provides anatomical definition of the mass [5]. It allows to distinguish tumors from thrombi and extra cardiac mass lesions [6]. The 3-D TEE additionally can assess the volume of the intracardiac masses [7]. Magnetic resonance also provides precise description of localization and extent of the tumor. For the pleural masses it can help to evaluate tumor cell type [8].

In our case we used both methods for clear diagnosis. Magnetic resonance images revealed in the right atrium a huge mass with irregular surface that attached to the anterior wall, expanded into the superior vena cava up to the brachiocephal vein and in the tricuspid valve. These data allowed us to suppose the malignant character of the mass. The microscopy and immunomorphology showed malignant rabdomyosarcoma.

In the presented case we succeeded the radical excision of the neoplasm with implantation of the biological valve because of the tissue infiltration in tricuspid valve anterior cusp. The patient had two courses of chemotherapy and 6 months postoperatively there was no evidence of any tissue mass in the right atrium and vena cava superior.

We consider that the urgent operation is indicated when tumor is not highly infiltrated in heart tissues. It can also be undertaken as palliative procedure in the presence of chamber obturation. We realize that in cases of secondary tumors complete resection may not be possible because of distant metastases. Nevertheless tumor should be surgically excised with obligatory radiation and/or chemotherapy.

doi:10.1016/S1569-9293(03)00059-8


   References
Top
 Abstract
 1. Introduction
 2. Case report
 3. Discussion
 References
 

  1. Holley DG, Martin GR, Brenner JI, Fyfe DA, Hunta JC, Kleinman CS, Ritter SB, Silverman NH. Diagnosis and management of fetal cardiac tumors: a multicenter experience and review of published reports. J Am Coll Cardiol. 1995;:516–520
  2. Chan HS, Sonley MJ, Moes CA, Daneman A, Smith CR, Martin DJ. Primary and secondary tumors of childhood involving the pericardium and great vessels. A report of 75 cases and review of the literature. Cancer. 1985;56:825–836[Medline]
  3. Schmaltz AA, Apitz J. Primary heart tumors in infancy and childhood. Report of four cases and review of literature. Cardiology. 1981;67:12–22[Medline]
  4. Blondeau P. Primary cardiac tumors – French studies of 533 cases. Thorac Cardiovasc Surg. 1996;38(Suppl. 2):192–195
  5. Aru GM, Falchi S, Cardu G, Meloni L, Lixi G, Martelli V. The role of transesophageal echocardiography in the monitoring of cardiac mass removal: a review of 17 cases. J Card Surg. 1993 Sep;8(5):554–557[Medline]
  6. Reeder GS, Khandheria BK, Seward JB, Tajik AJ. Transesophageal echocardiography and cardiac masses. Mayo Clin Proc. 1991 Nov;66(11):1101–1109[Medline]
  7. Ahmed S, Nanda NC, Miller AP, Nekkanti R, Yousif AM, Pacifico AD, Kirklin JK, McGiffin DC. Volume quantification of intracardiac mass lesions by transesophageal three-dimensional echocardiography. Ultrasound Med Biol. 2002 Nov–Dec;28(11–12):1389–1393[Medline]
  8. Heelan RT. CT and MR imaging in the evaluation of pleural masses. Chest Surg Clin North Am. 1994;4:431–450[Medline]




This Article
Right arrow Abstract Freely available
Right arrow Full Text (PDF)
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to Personal Folders
Right arrow Download to citation manager
Right arrow Permission Requests
Citing Articles
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Skopin, I.I.
Right arrow Articles by Sazonenkov, M.A.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Skopin, I.I.
Right arrow Articles by Sazonenkov, M.A.
Related Collections
Right arrow Cardiac - other

HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
ANN THORAC SURG ASIAN CARDIOVASC THORAC ANN EUR J CARDIOTHORAC SURG
J THORAC CARDIOVASC SURG ICVTS ALL CTSNet JOURNALS