European Journal of Echocardiography 2007 8(5):406-407; doi:10.1016/j.euje.2006.07.002
Copyright © 2006, The European Society of Cardiology
Primary cardiac rhabdomyosarcoma causing obstruction to the right ventricular outflow
Alberto Bouzas-Mosquera*,
Xacobe Flores-Rios and
Guillermo Aldama
Cardiology Department, Juan Canalejo Hospital, As Xubias 84, 15006, A Coruña, Spain
Received 20 June 2006; received in revised form 26 June 2006; accepted after revision 15 July 2006.
aboumos{at}canalejo.org
* Corresponding author. Tel.: +34 981178184; fax: +34 981178258.
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Abstract
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A 72-year-old man presented with worsening shortness of breath
and a systolic murmur. Transthoracic and transoesophageal echocardiograms
revealed a huge mass (70
x30mm) obliterating the right ventricular
outflow tract, with a peak transpulmonary gradient of 128mmHg.
Pathological examination confirmed the mass to be a primary
cardiac rhabdomyosarcoma originating from the pulmonary valve.
Keywords: Primary cardiac tumours
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Case report
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A 72-year-old male was referred to our institution with complaints
of worsening dyspnoea and exercise intolerance during the preceding
3 months. At admission he was haemodynamically stable, and a
systolic murmur was detected in the left parasternal border.
Transthoracic echocardiogram showed striking signs of right
ventricular overload, with severe enlargement and hypertrophy
of the right ventricle (RV) and remarkable displacement of the
interventricular septum toward the left ventricle. At the pulmonary
valve level, a huge mass (70
x30mm) was found to be filling and
obstructing the right ventricular outflow tract (RVOT). A transoesophageal
echocardiogram showed the mass to be attached to the pulmonary
valve, extending to the RVOT and proximal portion of the pulmonary
trunk over a wide base (
Fig. 1). The peak gradient across
the pulmonary valve, assessed by continuous wave Doppler, was
128mmHg (
Fig. 2).
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Figure 1 Transoesophageal echocardiogram showing a giant mass taking up the pulmonary valve and extending to the right ventricular outflow tract and pulmonary trunk (mid oesophageal AV short axis view). AV=aortic valve; RV=right ventricle; T=tumour.
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Surgical intervention was planned but, before the procedure
could be carried out, the patient experienced an episode of
syncope, followed, a few hours later, by resting dyspnoea and
diaphoresis, with progressive hypotension and desaturation that
could not be overcome, and he died. The anatomopathological
study disclosed a solid cardiac tumour arising from the pulmonary
valve (
Fig. 3) and the histologic diagnosis was a moderately
differentiated primary cardiac rhabdomyosarcoma. No evidence
of peripheral metastasis was noted.
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Discussion
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Cardiac rhabdomyosarcomas are rare tumours
1 accounting for about
4% of primary cardiac neoplasms, although this prevalence has
not been unanimously reported; in a large series of 75 primary
cardiac sarcomas, none of them were found to be a rhabdomyosarcoma.
2
Rhabdomyosarcomas have no predilection for a single cardiac chamber and may obstruct one or more valve orifices. Nevertheless, very few cases of sarcomas originating from the pulmonary valve have been reported.3 These tumours may grow to a large size within the lumen and may end up obstructing the RVOT, leading to RV overload, dilation and failure. Additionally, portions of the tumour may dislodge and embolize into the pulmonary arteries.
The prognosis is very poor; surgical intervention is often futile, and most patients die within the first year after diagnosis; nevertheless, surgery is usually necessary to elucidate the diagnosis and alleviate symptoms.
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References
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- Hui K.S., Green L.K., Schmidt W.A. Primary cardiac rhabdomyosarcoma: definition of a rare entity. Am J Cardiovasc Pathol (1988) 2(1):19–29.[Medline]
- Burke A.P., Cowan D., Virmani R. Primary sarcomas of the heart. Cancer (1992 January 15) 69(2):387–395.[CrossRef]
- Schroder S., Walker T., Greschniok A., Herdeg C., Karsch K.R., Ziemer G. Primary cardiac leiomyosarcoma originating from the pulmonary valve. Case report and review of the literature. J Cardiovasc Surg (Torino) (2001 February) 42(1):53–56.[Medline]
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