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Case Reports
. 2018 Dec 15;57(24):3575-3580.
doi: 10.2169/internalmedicine.0657-17. Epub 2018 Aug 10.

A Mass Filling the Right Atrium: Primary Cardiac Rhabdomyosarcoma

Akihisa Kimura  1 , Masaki Tsuji  1 , Toshiaki Isogai  1 , Kenichiro Nagata  1 , Ken Kato  1 , Motoyuki Hisagi  2 , Takahiro Nonaka  2 , Mikio Ninomiya  2 , Takahiro Kiriu  3 , Hiroyuki Tanaka  1 , Tamotsu Tejima  1
Affiliations
Case Reports

A Mass Filling the Right Atrium: Primary Cardiac Rhabdomyosarcoma

Akihisa Kimura et al. Intern Med. .

Abstract

A 43-year-old woman presented with worsening shortness of breath and lower-extremity edema. Echocardiography and computed tomography showed obstruction of blood flow due to a mass filling the right atrium. Emergency surgery was performed for circulatory failure. Primary cardiac rhabdomyosarcoma was diagnosed based on a histological examination. The patient died about two months after the diagnosis despite surgical excision and radiation therapy. The poor prognosis may have resulted from the grossly incomplete removal of the tumor and chemotherapy intolerance. We herein report a case of primary cardiac rhabdomyosarcoma filling the right atrium and offer possible reasons for the poor prognosis.

Keywords: combined modality approach; primary cardiac tumor; prognosis; rhabdomyosarcoma.

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Figures

Figure 1.
Figure 1.
Transesophageal echocardiography (mid-esophageal view 90°) revealed a movable isoechoic mass filling the right atrium. LA: left atrium, M: mass, RA: right atrium, SVC: superior vena cava
Figure 2.
Figure 2.
Contrast-enhanced computed tomography on admission showed a mass extending from the right atrium (arrow) (A) to the inferior vena cava (arrow) (B).
Figure 3.
Figure 3.
The tumor was attached to the inferior wall of the right atrium near the inferior vena cava (A). The excised tumor had a smooth gray surface and a size of 70×50×30 mm (B). RA: right atrium, RV: right ventricle, T: tumor
Figure 4.
Figure 4.
Atypical spindle-shaped cells were dense around vessels on Hematoxylin and Eosin staining (A). The tumor was negative for cytokeratin AE1/AE3 (B) and positive for vimentin, desmin, and myogenin, as is characteristic of rhabdomyosarcoma (C-E).
Figure 5.
Figure 5.
Contrast-enhanced computed tomography showed the reemergence of the tumor in the right atrium to the inferior vena cava (arrow) (A), the enlargement of thrombi in the inferior vena cava (arrow) (B), and the emergence and growth of a lung-metastatic tumor (arrow) (C).
Figure 6.
Figure 6.
Fluorodeoxyglucose-positron emission tomography revealed the recurrent cardiac tumor extending from the right atrium to the inferior vena cava (arrow) and the metastatic tumor in the right lung (arrow).
See this image and copyright information in PMC

References

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