Keywords
cardiac tumor - cardiac myxoma
Introduction
Tumors of the heart are rare with a frequency of 0.017 and 0.28% in autopsy series.
Myxoma is the most common cardiac tumor, accounting for more than 70% of all cardiac
neoplasms.[1] Only a few cases of biatrial myxoma or biatrial recurrence of cardiac myxoma have
been reported and they generally describe a single tumor reaching both atria.[2] In this study, we found two independently growing atrial myxomas.
We report on a 56-year-old female patient who was referred to our center with diagnosis
of a biatrial tumor mass in 2012. She had an extensive hospital record with Leiden
factor V mutation, Leriche syndrome, tumor of unknown dignity of the uterus and the
breasts, and history of removal of left atrial cardiac myxoma in 2008. Embolic Leriche
syndrome had manifested as a consequence of a left atrial myxoma which was removed
in a second step. During the first surgery in 2008, both atria were inspected, and
a left atrial tumor mass was found, originating from the left upper pulmonary vein
outlet. It had been excised with a 1-cm resection margin and the defect reconstructed
using a Gore Tex (W.L. Gore & Associates, Inc., Newark, Delaware, United States) patch.
Histology had confirmed cardiac myxoma with a tumor-free resection margin. There was
neither a family history of cardiac myxoma nor signs of a carney complex.
We performed a complete tumor staging including transesophageal echocardiography ([Fig. 1]) and computed tomography ([Fig. 2]). The left atrial tumor prolapsed into the left ventricle; there was no infiltration
of surrounding structures, so a metastasis of the gynecologic tumors seemed unlikely.
Finally, we decided to urgently remove the biatrial tumor mass to prevent further
complications such as pulmonary embolism or stroke.
Fig. 1 Transthoracic echocardiography: four-chamber view showing the biatrial tumor mass.
Fig. 2 Computed tomography: four-chamber view showing the biatrial tumor mass.
Because it was a redo surgery, the extracorporeal circulation was connected to the
right axillary artery and the femoral vein, before we gained surgical access via median
sternotomy. An additional cannula for venous drainage was connected to the superior
caval vein, thus achieving complete bicaval bypass. The heart was arrested with cold
blood cardioplegia. Opening the right atrium, we found it was completely filled with
a 6-cm dark blue, polypous pedunculated tumor mass. The left atrium was opened through
the interatrial septum. Inside we found a 7-cm, round, pedunculated tumor mass ([Fig. 3]). Tumors were not connected and unlike the finding during the first surgery in 2008,
the tumors originated from different parts of the interatrial septum and were not
in touch with the old resection area. Both tumors were completely resected, including
the adjacent parts of the interatrial septum ([Fig. 4]). Atria were reconstructed using bovine pericardium.
Fig. 3 Intraoperative picture of both myxomas in situ.
Fig. 4 Both tumors after resection.
The postoperative course was uneventful and the patient was referred to our gynecology
unit for further treatment. Histology revealed cardiac myxoma without signs of malignancy.
Resection margins were tumor free. For the further treatment course, we recommended
echocardiographic checkups on a regular basis to timely diagnose another recurrence
of cardiac myxoma.
Discussion
The risk for recurrence of cardiac myxoma is thought to be approximately 1 to 3% for
sporadic tumors and 12 to 22% for familial and complex myxomas; mostly recurrent myxomas
appear within the first 4 years after primary surgery.[3]
In our case, the cause for the recurrence of the myxoma remains unclear. There were
no typical signs of a carney complex-like skin lesions or hyperpigmentation. Different
etiologies of myxoma recurrence including malignant metastasis are discussed in the
literature.[3]
[4]
[5] If the reason for the recurrence was an inadequate surgical resection at first surgery,
then the question remains why tumor recurrence happened in both atria. A possible
explanation might be spreading of tumor cells during the first surgery or a multilocalized
tumor that has started growing after the first surgery and had not been detected.
As the etiology of myxoma recurrence is unclear, all patients after surgical removal
of cardiac myxoma should receive routine echocardiographic checkups.
Even in patients with complex multimorbidity, removal of cardiac tumors is possible
with remarkable postoperative outcome. In our case, surgical removal of the tumor
was necessary both in curative and palliative intent. Only with the cardiac problem
solved, curative treatment of the gynecologic tumor became possible.
