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Year : 2021  |  Volume : 10  |  Issue : 1  |  Page : 26-28

Multiple recurrences in a nonfamilial cardiac myxoma

1 Cardiovascular Research Center, Tabriz University of Medical Sciences, Tabriz, Iran
2 Cardiovascular Research Center, Kerman University of Medical Sciences, Kerman, Iran

Date of Submission27-Jan-2021
Date of Decision04-Apr-2021
Date of Acceptance08-Apr-2021
Date of Web Publication29-Jun-2021

Correspondence Address:
Dr. Maryam Chenaghlou
Cardiovascular Research Center, Tabriz University of Medical Sciences, Tabriz
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/rcm.rcm_7_21

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Cardiac myxomas are the most common primary cardiac tumors, with an estimated incidence of 0.5 per million individuals annually. Although surgical resection is curative however, postoperative tumor recurrence has been reported, especially in familial and complex type of myxoma. Literature reviews reveal only a few cases of recurrence in sporadic cardiac myxoma. Herein we have reported a case of sporadic cardiac myxoma, with multiple recurrences after surgical resection. The possibility of repeated recurrence of cardiac myxomas demonstrates the importance of regular echocardiographic surveillance after surgical resection in order to detection of recurrence even in non-familial and non-syndromic cases. Further evaluations, including genetic analysis of patients with recurrent cardiac myxomas, are warranted to investigate the exact nature of these tumors.

Keywords: Cardiac myxoma, sporadic, tumor recurrence

How to cite this article:
Salehi R, Mirtajaddini M, Chenaghlou M, Faridi L. Multiple recurrences in a nonfamilial cardiac myxoma. Res Cardiovasc Med 2021;10:26-8

How to cite this URL:
Salehi R, Mirtajaddini M, Chenaghlou M, Faridi L. Multiple recurrences in a nonfamilial cardiac myxoma. Res Cardiovasc Med [serial online] 2021 [cited 2022 Dec 9];10:26-8. Available from: https://www.rcvmonline.com/text.asp?2021/10/1/26/319791

  Introduction Top

Cardiac myxoma is the most frequent primary cardiac tumor with benign features, endocardial origin that mainly located in the left atrium (LA).[1] Embolic events, intracardiac obstruction, and constitutional symptoms are the typical triad of myxomas. Recurrence rate of sporadic cardiac myxoma is about 3% while it reaches to 12% and 22% in familial and complex forms, respectively.[2] The second recurrence of cardiac myxoma is exceptionally rare.[3] Sporadic form usually presents in a middle-age woman at the border of fossa ovalis in the inert-atrial septum of LA. The familial form usually represents in a young man with unusual locations and might have multifocal origin. Carney syndrome or the complex myxoma is characterized by myxoma association with multiple neoplasia syndromes, pituitary-related symptoms, and primary pigmented nodular adrenocortical disease.[2] There are few reported cases of the second recurrence of cardiac myxoma in sporadic form. Herein, we present the 2nd recurrence of sporadic cardiac myxoma in a young man, risk factors for recurrence of this tumor, and importance of cardiac surveillance in all patients after resection of the first episode of cardiac myxoma, especially in patients with estimated high risk of recurrence.

  Case Report Top

A 24-year-old male presented with neurologic symptoms as cerebrovascular accident (CVA). Physical examination was unremarkable regarding findings suggestive of CVA. He had not any history of medical illness, and his family history was negative for any disorders. Abnormal laboratory findings were hemoglobin (Hb): 9 mg/dl, hematocrit (Hct): 23%, high C-reactive protein (CRP), and erythrocyte sedimentation rate (ESR): 47. Further evaluation revealed multiple myxomas in all four chambers of the heart and right pulmonic vein. Cardiac surgery was done for removal of all cardiac masses. Pathologic evaluation of tumors confirmed myxoma diagnosis. Two years later, he developed blurred vision and cardiac surgery was performed due to LA myxoma.

Six years later, he admitted with progressive dyspnea and dry cough. The laboratory tests showed Hb of 10.3 mg/dl, HCT of 35.5%, erythrocyte ESR of 50, and CRP of 3 (+). The polymerase chain reaction test for COVID-19 was negative in two separate nasopharyngeal swabs. Echocardiography showed normal left ventricle size with preserved systolic function, normal right ventricle (RV) size with preserved systolic function, fixed heterogeneous large size mass attached to RV apex and lateral wall of RV (size = 20 mm × 17 mm), right atrial (RA) enlargement, large size multilobulated heterogeneous mobile mass in RA attached to junction of inferior vena cava (IVC) to RA (size = 50 mm × 45 mm) protruding to near RV inflow, and tapping of tricuspid valve suggestive of myxoma [[Figure 1] and Online Videos 1 and 2]. Computed tomography (CT) angiography revealed bilateral infarct focus in basal lobes of lungs without evidence of COVID-19. Another findings of CT angiography were soft tissue mass in size of 68 mm × 42 mm in RA and another mass in size of 26 mm × 18 mm in RV apex. Furthermore, ostium of superior and IVC revealed multiple masses.
Figure 1: Transesophageal echocardiography of the patient before the third surgery. Multilobulated heterogeneous mass in right atrium and right ventricle (a and b). Three-dimensional transesophageal echocardiography showing mass in right atrial (c)

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The patient underwent cardiac surgery for the removal of cardiac masses for the third time. Pathologic tissue diagnosis was cardiac myxoma again. The postoperative course was uneventful and the patient discharged in good condition.

  Discussion Top

Our patient had anemia and increased levels of CRP and ESR at first admission. Further evaluation did not reveal any specific cause of these abnormalities. Nonspecific laboratory changes such as leukocytosis, anemia, polycythemia, thrombocytopenia, increased CRP, ESR, and globulin levels have been reported that might be related to interleukin (IL) production from cardiac myxoma leading to autoimmune and inflammatory responses. The most investigated IL probably is IL-6 that could be used as a biomarker of cardiac myxoma in the settings such as tumor recurrence detection.[4] Proposed mechanisms for the recurrence of myxomas are inadequate resection of primary tumor, familial type, totipotent multicentricity, and metastatic forms. Multiple recurrences in the setting of sporadic forms of myxomas could be a genetic nonfamilial predisposition.[3]

Although the recurrent form of cardiac myxoma usually occurs during 4 years, it could be appeared several years after the first surgery. Recurrent myxoma seems to be more common in men unlike the sporadic form. Marked vascularization has been described in a recurrent sporadic form of myxoma, a characteristic that usually presents in malignant tumors. One of the possible mechanisms predisposing to recurrence of tumor might be the presence of large vessels in pedicle, base, and surrounding structures of the tumor that leads to excessive growth and differentiation. Vascular endothelial growth factor (VEGF) released by the tumor cells and macrophages has been shown as the potent angiogenic factor. VEGF might have a pivotal role in angiogenic capability of some invasive forms of cardiac myxomas.[5]

Our patient had not any findings suggestive of marked vascularization of tumor in echocardiography. In a study, factors that have association with tumor recurrence were younger age of patients at the first presentation, the first 10 years after surgery, smaller tumor mass, and ventricular location of the tumor.[6] In another study for determining a risk prediction for recurrence of primary cardiac myxoma after resection, multicentric growth of the myxoma has been shown as a risk factor for recurrence, especially during the first 4 years after surgery.[7]

Our patient was a young man with multicentric and unusual location of cardiac myxoma including ventricular, biatrial, and pulmonic vein origin at first presentation, all indicative of high probability of tumor recurrence. Multipotent mesenchymal cells similar to the primordial cardiac stem cells as the source of myxomas and persistence of these cells might explain the multicentric growth of these tumors in the heart.[8]

Several instances of recurrent forms of cardiac myxoma in familial, syndromic, and even nonfamilial cases besides reported case of asymptomatic recurrence of cardiac myxomas reveal the importance of regular surveillance using echocardiography of these tumors after the first resection.[9]

In conclusion, recurrence in sporadic form of cardiac myxoma is not common, however, it could occur even as multiple recurrences. There is higher probability of tumor recurrence in patients with younger age, multicentric, and unusual location of tumor at first presentation. Surveillance echocardiography has a pivotal role for detecting recurrence in all patients with a history of cardiac myxoma resection, especially in patients at high risk for recurrence.

Ethical clearance

This study was approved by the ethical committee of Tabriz Cardiovascular Medical and Research Center.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Stefanou MI, Rath D, Stadler V, Richter H, Hennersdorf F, Lausberg HF, et al. Cardiac myxoma and cerebrovascular events: A retrospective cohort study. Front Neurol 2018;9:823.  Back to cited text no. 1
Marina K, Vasiliki KE, George S, Vasiliki V, Androniki T, Abraham G, et al. Recurrent cardiac myxoma in a 25 year old male: A DNA study. World J Surg Oncol 2013;11:95.  Back to cited text no. 2
Iskandar ME, Dimitrova K, Geller CM, Hoffman DM, Tranbaugh RF. Complicated sporadic cardiac myxomas: A second recurrence and myxomatous cerebral aneurysms in one patient. Case Rep Surg 2013;2013:642394.  Back to cited text no. 3
Raheem SA, Haji Gh F, Farhan HA, Najam A, Yousif A. Large right atrial myxoma associated with unexpected large abdominal ascites. Int J Med Res Health Sci 2018;7:191-5.  Back to cited text no. 4
Carvalho MS, Andrade MJ, Abecasis J, Gouveia R, Branco L, Neves JP, et al. Understanding cardiac myxoma recurrence: A case report. Rev Port Cardiol 2013;32:239-42.  Back to cited text no. 5
Shah IK, Dearani JA, Daly RC, Suri RM, Park SJ, Joyce LD, et al. Cardiac myxomas: A 50-year experience with resection and analysis of risk factors for recurrence. Ann Thorac Surg 2015;100:495-500.  Back to cited text no. 6
Wang Z, Chen S, Zhu M, Zhang W, Zhang H, Li H, et al. Risk prediction for emboli and recurrence of primary cardiac myxomas after resection. J Cardiothorac Surg 2016;11:22.  Back to cited text no. 7
Jia X, Kohli A, Jarjour J, Chen CJ, Gilmore B, Tabbaa R, et al. Recurrent biatrial myxoma in a 41-year-old woman after left atrial myxoma resection. Tex Heart Inst J 2017;44:402-4.  Back to cited text no. 8
Chen C, Chu H, Tso J, Luthringer DJ, Siegel R. Asymptomatic recurrence of right ventricular myxoma after excision of four-chamber myxoma. CASE (Phila) 2017;1:195-7.  Back to cited text no. 9


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