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Case Reports
. 2016 Jul 7;11(1):95.
doi: 10.1186/s13019-016-0490-3.

A case report of primary cardiac myxofibrosarcoma presenting with severe congestive heart failure

Kosuke Ujihira  1 , Akira Yamada  2 , Naritomo Nishioka  2 , Yutaka Iba  2 , Ryushi Maruyama  2 , Katsuhiko Nakanishi  2 , Ai Shimizu  3 , Kanako C Hatanaka  3 , Tomoko Mitsuhashi  3 , Toshiya Shinohara  4 , Hatsue Ishibashi Ueda  5
Affiliations
Case Reports

A case report of primary cardiac myxofibrosarcoma presenting with severe congestive heart failure

Kosuke Ujihira et al. J Cardiothorac Surg. .

Abstract

Background: Primary cardiac sarcomas are extremely rare. Furthermore, the myxofibrosarcomas are one of the rarest forms of cardiac sarcomas, and its prognosis is known to be quite poor.

Case presentation: This is a case of a 23-year-old man who presented with acute severe congestive heart failure caused by almost complete obstruction of the mitral valve due to a large left atrial tumor. The patient required endotracheal intubation before his arrival to the hospital, and underwent an emergent surgical excision of the tumor. The tumor had a complex shape and originated from the orifice of the right upper pulmonary vein. Because the tumor seemed to extend over most of the surface of the left atrium, it seemed impossible to reconstruct the left atrium had we done a complete transmural resection. Instead, we carefully peeled the tumor leaving the outer layer of the left atrial wall. We applied cryoablation to the attached site, in order to prevent a recurrence of the tumor. The pathology report revealed that the tumor was a myxofibrosarcoma, and it seemed to originate from the heart. The patient received radiation therapy after the surgery and continues to be alive and well after 1-year, without apparent recurrence.

Conclusions: Cardiac myxofibrosarcoma can cause acute, severe left-sided heart failure. Non-transmural atrial wall resection with cryoablation might be effective for patients with cardiac myxofibrosarcomas with extensive atrial attachment.

Keywords: Cardiac tumor; Cryoablation; Heart failure; Myxofibrosarcoma.

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Figures

Fig. 1
Fig. 1
Pre-operative CT image. An enhanced CT scan showed lung congestion and intra-atrial tumor occupying the entire left atrial cavity
Fig. 2
Fig. 2
Pre-operative trans-esophageal echocardiogram images. The mobile tumor contracted inside the left atrium during a systolic phrase (a) and expanded across the mitral valve (b) into the left ventricular during a diastolic phrase (c)
Fig. 3
Fig. 3
Macroscopic views of the excised tumor. The tumor had three differentiated bodies. a, A thin and fragile tumor widely attached to the posterior left arial wall. b, A solid and club-like yellow-white colored tumor with myxoid area. c, A cyst with smooth surface
Fig. 4
Fig. 4
Histological images. a, An examination with hematoxylin and eosin technique showed spindle-shaped cells with myxoid background. b, Most of the tumor cells showed spindle-shaped, but some cells were round in shape. c, Focally a hypercellular lesion was found. d, The tumor cells were weakly immunnoreactive for calponin
See this image and copyright information in PMC

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