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Unusual survival time of primary cardiac sarcoma of the right ventricle.
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MedLine Citation:
PMID:  21731808     Owner:  NLM     Status:  PubMed-not-MEDLINE    
Abstract/OtherAbstract:
Primary cardiac sarcomas are rare tumors with unfavorable prognosis. We report a 69-year-old male with a right ventricular mass diagnosed as primary malignant cardiac sarcoma with unexpected long survival of 16.5 months.
Authors:
Khaled M Buresly; Abdul Mohammed Shukkur; Babu Uthaman
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Heart views : the official journal of the Gulf Heart Association     Volume:  12     ISSN:  0976-5123     ISO Abbreviation:  Heart Views     Publication Date:  2011 Jan 
Date Detail:
Created Date:  2011-07-06     Completed Date:  2011-07-14     Revised Date:  2013-05-29    
Medline Journal Info:
Nlm Unique ID:  101316474     Medline TA:  Heart Views     Country:  Qatar    
Other Details:
Languages:  eng     Pagination:  35-8     Citation Subset:  -    
Affiliation:
Department of Cardiology, Chest Diseases Hospital, Kuwait.
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

Full Text
Journal Information
Journal ID (nlm-ta): Heart Views
Journal ID (publisher-id): HV
ISSN: 1995-705X
ISSN: 0976-5123
Publisher: Medknow Publications Pvt Ltd, India
Article Information
© Heart Views
open-access:
Print publication date: Season: Jan-Mar Year: 2011
Volume: 12 Issue: 1
First Page: 35 Last Page: 38
ID: 3123515
PubMed Id: 21731808
Publisher Id: HV-12-35
DOI: 10.4103/1995-705X.81552

Unusual Survival Time of Primary Cardiac Sarcoma of the Right Ventricle
Khaled M. Bureslyaff1
Abdul Mohammed Shukkuraff1
Babu Uthamanaff1
Department of Cardiology, Chest Diseases Hospital, Kuwait
Correspondence: Address for correspondence: Dr. A. M. Shukkur, Department of Cardiology, Chest Diseases Hospital, Kuwait. E-mail: shukkur@hotmail.com

INTRODUCTION

Primary cardiac sarcomas are rare malignant tumors with poor prognosis for survival ranging from 6 to 12 months. The prevalence of primary cardiac sarcoma in autopsy series is 0.001–0.28%.[1] In one of the series, the incidence was reported to range from 0.0017 to 0.019%.[2, 3] The usual sites are the atrium, ventricle, pericardium or great vessels.[4] The therapeutic options include surgery, chemotherapy and radiotherapy alone or in combination.[5]


CASE PRESENTATION

A 69-year-old male with diabetes mellitus presented with epigastric pain and weight loss for 3 months. Examination revealed low grade fever and markedly raised jugular venous pressure. Chest radiograph demonstrated cardiomegaly and small bilateral pleural effusions. Transthoracic echocardiography demonstrated large pericardial effusion with cardiac tamponade. He underwent emergency pericardiocentesis and 1060 ml of hemorrhagic fluid was aspirated.

Percutaneous pericardial biopsy was done to look for specific etiology. After pericardial biopsy, a 4-F pigtail catheter was left in the pericardial cavity for further aspiration. Exactly 5600 ml of hemorrhagic pericardial fluid was aspirated during the following 2 weeks. Pericardial fluid Gram stain and culture were negative for pyogenic or tuberculosis infection. Cytology did not reveal any malignant etiology. Repeat echocardiographic imaging revealed a large solid mass (7 × 10 × 8 cm) involving the right ventricular inflow, the body of the right ventricle and right ventricular outflow tract postero-medially. The mass appeared relatively fixed with possible myocardial infiltration [Figure 1ac]. Cardiac catheterization and hemodynamic assessment showed biventricular heart failure. Right ventricular angiogram demonstrated a filling defect in the postero-inferior aspect of the right ventricle. Biopsy taken from that area using a bioptome revealed primary cardiac sarcoma of the malignant fibrous histiocytoma sub-group. He was offered tumor resection with chemotherapy but he did not accept. Initial computed tomography (CT) scan did not reveal any extracardiac tumor or metastasis. Echocardiographic evaluation at 6 and 8 months interval revealed enlarging tumor mass. The last echocardiogram demonstrated a huge right ventricular tumor measuring 20 × 10 cm, filling the entire right ventricular cavity and infiltrating through the right ventricular wall into the liver [Figure 2a and b]. He also had small pericardial effusion, bilateral pleural effusions, ascites as well as hepatic and splenic metastasis. He continued to refuse any treatment and expired at home 16.5 months after the initial presentation.


DISCUSSION

Cardiac sarcomas are usually asymptomatic until advanced, or present after variable periods of symptoms that are often nonspecific ranging from a few weeks to several months.[6] They manifest by pericardial effusion with tamponade, local invasion causing arrhythmias, obstruction to blood flow and interference with valve function, embolic phenomena, or systemic symptoms of dyspnea, fever, malaise and weight loss. In one of the published series, 29% of patients had pericardial effusion at presentation.[3]

Our patient had massive pericardial effusion with tamponade at the initial presentation. He underwent emergency pericardiocentesis and the repeat echocardiography could demonstrate the tumor mass in the right ventricle. Dyspnea is the commonest symptom in 60%, followed by chest pain in 28%, congestive heart failure in 28%, palpitations in 24%, fever in 14%, myalgia in 10% and embolism in 5%.[1] Although in adults cardiac tumors are mostly benign, 25% can be malignant and one-third of these are angiosarcomas.[79] Cardiac sarcoma is a rapidly growing tumor with local invasion and systemic metastases which result in dismal prognosis. Therefore, early recognition and characterization of their pathoanatomy is crucial. In previous studies, metastases were present in 66–89% of patients at the time of diagnosis.[1012] Burke et al,[13] reported significantly improved survival period of 12 months for patients with primary cardiac angiosarcoma who received treatment, while for the untreated, the median survival was only 3 months. Mean survival for most sarcomas is about 9–11 months. Median survival ranges from 24 months after resection versus 10 months with either incomplete or no resection.[2] This case report highlights an unusual clinical course of survival for 16.5 months despite being without treatment, leading to local infiltration and distant metastasis.


CONCLUSION

This is a case of infiltrating cardiac sarcoma with unexpected survival for 16.5 months without definitive therapy. Although the patient presented with nonspecific symptoms and cardiac tamponade, echocardiography was extremely useful to diagnose the cardiac sarcoma and to document its progress.


Notes

Source of Support: Nil

Conflict of Interest: None declared.

REFERENCES
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2. Centofanti P,Di Rosa E,Dersorsola L,Dato GM,Patane F,LaTorre M,et al. Primary cardiac neoplasms: Early and late results of surgical treatment in 91 patientsAnn Thorac SurgYear: 19996812364110543485
3. Shanmugam G. Primary cardiac sarcomaEur J Cardiothorac SurgYear: 2006299253216675225
4. Engelen M,Bruch C,Hoffmeier A,Kersting C,Stypmann J. Primary left atrial angiosarcoma mimicking severe mitral valve stenosisHeartYear: 200527913
5. Erpolat OP,Icli F,Dogan OV,Gokaslan G,Akmansu M,Erekul S,et al. Primary cardiac angiosarcoma: A case reportTumoriYear: 200894892719267115
6. Hoffmeier A,Scheld HH,Tjan TD,Schneider M,Kerber S,Schmidt T,et al. Ex situ resection of primary cardiac tumorsThorac Cardiovasc SurgYear: 2003519910112730820
7. Chidel MA,Macklis RM. Perez C,Brady LW,Halperin EC,Schmidit –Illrich RKTumors of the heart and great vesselsPrinciples and practice of radiation oncologyYear: 2004PhiladelphiaLippincott Williams and Wilkins130514
8. Sakaguchi M,Minato N,Katayama Y,Nakashima A. Cardiac angiosarcoma with right atrial perforation and cardiac tamponadeAnn Thorac Cardiovasc SurgYear: 20062145816702940
9. Oshima K,Ohtaki A,Kano M,Motoi K. Primary cardiac angiosarcoma associated with cardiac tamponade case reportJpn Circ JYear: 199963822410553930
10. Sorlie D,Myhre ES,Stalsberg H. Angiosarcoma of the heart unusual presentation and survival after treatmentBr Heart JYear: 1984519476537794
11. Patrianakos AP,Parthenakis FI,Drositis I,Nyktari E,Vardas PE. Primary heart angiosarcomaHellenic J CardiolYear: 200748364518196659
12. Santo K,Dandekar U. Primary right atrial angiosarcomaAsian Cardiovasc Thorac AnnYear: 200816490118984761
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Figures

[Figure ID: F1]
Figure 1a 

2-Dimensional echocardiographic imaging (apical 4 chamber view) demonstrating dilated right side of the heart with most of the right ventricular cavity occupied by the tumor mass



[Figure ID: F2]
Figure 1b 

2D apical 4 chamber view showing forward tricuspid flow through the residual RV cavity



[Figure ID: F3]
Figure 1c 

2D echocardiogram parasternal short axis view showing the tumor mass at the right ventricular outflow and inflow



[Figure ID: F4]
Figure 2a 

Subcostal echocardiographic view demonstrating the tumor mass extending outside the right ventricle infiltrating into the liver



[Figure ID: F5]
Figure 2b 

Subcostal echocardiographic view showing spleen an echogenicity suggesting splenic infiltration



Article Categories:
  • Case Report

Keywords: Echocardiography, metastasis, pericardial effusion, primary cardiac sarcoma, right ventricular mass.

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