|
|
CASE REPORT |
|
Year : 2022 | Volume
: 10
| Issue : 3 | Page : 168-170 |
|
Giant biatrial myxomas: A rare cardiac tumor
Sanjeev Asotra1, Sarita Asotra2, Shivani Rao3, Meena Rana1
1 Department of Cardiology, I.G.M.C, Shimla, Himachal Pradesh, India 2 Department of Pathology, I.G.M.C, Shimla, Himachal Pradesh, India 3 Department of Cardiology, RML Hospital, New Delhi, India
Date of Submission | 15-Aug-2022 |
Date of Decision | 06-Oct-2022 |
Date of Acceptance | 07-Oct-2022 |
Date of Web Publication | 14-Dec-2022 |
Correspondence Address: Sanjeev Asotra Room No. 308, Department of Cardiology, I.G.M.C. Shimla, Himachal Pradesh India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/heartindia.heartindia_37_22
Primary cardiac tumors constitute around 10% of all tumors of the heart. Atrial myxomas are the most common tumors of the heart, constituting around 50%–70% of all tumors. Most of them arise from the left atrium but multiple-site involvement is seen in 5%. Biatrial myxomas are rare and present in around 2.5% of patients. Myxomas can have varied symptoms. We present a case of biatrial myxoma that underwent successful surgical resection.
Keywords: Atria, cardiac surgery, cardiac tumor, echocardiography, myxoma
How to cite this article: Asotra S, Asotra S, Rao S, Rana M. Giant biatrial myxomas: A rare cardiac tumor. Heart India 2022;10:168-70 |
Introduction | |  |
Myxomas are most common primary tumors of heart constituting around 10% of all cardiac tumors. Left atrium is the common site involving around 50 -70%. Biatrial Myxomas are extremely rare. We report a case of large biatrial myxoma in a young male who underwent successful surgical correction.
Case Report | |  |
A 31-year-old male presented with a 6-month history of low-grade irregular fever, exertional dyspnea, and palpitations. The patient was started on antitubercular drugs for the past 2 months by a local physician due to a high erythrocyte sedimentation rate (ESR) and low-grade fever without any relief. Patients denied any history of cardiac illness in the past. His mother had undergone cardiac surgery for left atrial myxoma 1 year back. He was hemodynamically stable with a pulse rate of 96 beats/min. Lungs were clear and cardiovascular examination revealed mid-diastolic rumble with tumor plop. Chest X-ray revealed mild Pulmonary venous hypertension (PVH). Laboratory investigation showed hemoglobin of 13 g/dl and normal blood counts with an ESR of 65. Liver, kidney function, and coagulation profile were normal. An ultrasonography scan of the abdomen was normal. Transthoracic echocardiography (TTE) revealed biatrial mobile masses attached to the interatrial septum protruding across the tricuspid and mitral valve during diastole. The right atrial mass was 9.4 cm × 4.7 cm and the left atrial mass was 5.1 cm × 2.4 cm. The tricuspid valve color Doppler showed mild TR with a gradient of 38. There was evidence of mild pericardial effusion [Figure 1]. | Figure 1: Echocardiogram showing two large masses protruding into ventricles across both AV valves
Click here to view |
Systemic examination did not reveal any feature suggestive of the Carneys complex. Her younger sister underwent screening echocardiography which was found to be normal. The patient underwent surgery through a biatrial approach. There was a large right atrial myxomatous mass of size 10 cm × 8 cm with a 15 mm stalk and it was almost filling the whole cavity of the right atrium. The left atrial myxomatous mass was 6 cm × 3 cm in size; it was directly attached to the undersurface of the interatrial septum. Both the tricuspid and mitral valves were normal. Both myxomas were excised along with wide excision of the interatrial septum and the interatrial septum was repaired with a pericardial patch. The patient had an uneventful recovery and follow-up echocardiography at 6 months was normal. Histopathology of excised masses revealed multiple round, stellate, and spindle cells with abundant eosinophilic cytoplasm embedded in an abundant myxoid stroma. Scattered giant and plasma cells were seen along with foci of hemorrhage, and no mitosis or atypia was seen [Figure 2]. Immunohistochemistry revealed positive for CD34 and vimentin. | Figure 2: Slide showing multiple round, stellate, and spindle cells with abundant eosinophilic cytoplasm embedded in an abundant myxoid stroma
Click here to view |
Discussion | |  |
Myxomas are the most common cardiac tumors accounting for almost 50% of all primary cardiac tumors. The majority of myxomas arise from the left atrium and are usually attached to the interatrial septum at the fossa ovalis. About 18%–20% of them arise from the right atrium and 5% can have origin from multiple sites in the heart.[1],[2] Biatrial myxomas are very rare and found in 2.5% of all myxomas; they usually arise from the mirror image region of both sides of the interatrial septum, often representing growth in both sides directly from the common septum.[3],[4],[5],[6] Seven percent of myxomas can present as heritable disorders with spotty pigmentation of the skin, naevi, and endocrinopathy as Carneys complex.[7] Myxomas can have varied clinical presentations depending on the hemodynamic effect of the tumor, symptoms related to embolism, or constitutional symptoms. They can present with pyrexia of unknown origin as in our patient due to the release of inflammatory cytokines like interleukin-6 from the tumor.[8] Some may present asymptomatically with incidental echocardiographic detection.
The majority of myxomas are benign but can have malignant potential suggested by the reoccurrence of the tumor, local invasion, extension from the heart, and distant metastasis.[9] TTE is useful in the assessment of tumor dimension, site of attachment, shape, location, and its associated complication. Transesophageal echocardiography may be better than TTE in the assessment of tumor dimension and its attachment.[10],[11]
Cardiac myxomas are generally curable by surgical resection of the tumor. Surgical excision can be approached through the right atrial or biatrial approach but reoccurrence can occur at the site of the original tumor or other sites.[12] Reoccurrence is seen in around 3% of isolated cases of myxomas and 22% of cases in the Carneys complex and generally occurs within 3–4 years.[13] Meticulous follow-up is required postoperatively.
Conclusion | |  |
Giant biatrial myxomas are rare tumors. Clinical presentation of these may present a diagnostic challenge at times. Transesophageal or TTE is the imaging modality of choice. Surgical resection is generally curative. The reoccurrence rate is very low. Patients should be followed up with serial echocardiography.
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 initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
Ethical approval
Institutional ethical committee permission has been obtained.
Authors' contributions
All authors have contributed equally in every aspect of case and manuscript preparation.
References | |  |
1. | McAllister HA Jr., Hall RJ, Cooley DA. Tumors of the heart and pericardium. Curr Probl Cardiol 1999;24:57-116. |
2. | Reynen K. Cardiac myxomas. N Engl J Med 1995;333:1610-7. |
3. | Peachell JL, Mullen JC, Bentley MJ, Taylor DA. Biatrial myxoma: A rare cardiac tumor. Ann Thorac Surg 1998;65:1768-9. |
4. | O'Brien-Connors M. Biatrial myxoma: Rare incidence in cardiac surgery. Can J Cardiovasc Nurs 2004;14:21-3. |
5. | Namura O, Saitoh M, Moro H, Watanabe H, Sogawa M, Nishikura K, et al. A case of biatrial multiple myxomas with glandular structure. Ann Thorac Cardiovasc Surg 2007;13:423-7. |
6. | Vijan V, Vuppture A, Nair C. An unusal case of biatrial myxoma in a young female. Case Rep Cardiol 2016:1-3. [doi.org/10.1155/2016/3545480]. |
7. | Rothacker D, Kerber C. Carney complex. Clinical, pathological and genetic features in two generations of a family. Pathologe 2008;29:294-300. |
8. | Linares JA, Toyas C, Lacambra I, Ballester C. Fever of unknown origin and jaundice as the clinical presentation of myxoma of the right atrium. Rev Esp Cardiol 2008;61:220-2. |
9. | Odim J, Reehal V, Laks H, Mehta U, Fishbein MC. Surgical pathology of cardiac tumors. Two decades at an urban institution. Cardiovasc Pathol 2003;12:267-70. |
10. | Srivastava R, Hsiung MC, Fadel A, Nanda NC. Transesophageal echocardiographic demonstration of biatrial myxoma. Echocardiography 2004;21:187-8. |
11. | Doig JC, Ray S, Srikrishna SV, Hilton CJ, McComb JM. Transthoracic and transoesophageal echocardiography in the diagnosis and management of a biatrial myxoma. Eur Heart J 1993;14:1719-20. |
12. | Stevens LM, Lapierre H, Pellerin M, El-Hamamsy I, Bouchard D, Carrier M, et al. Atrial versus biatrial approaches for cardiac myxomas. Interact Cardiovasc Thorac Surg 2003;2:521-5. |
13. | Mendoza C, Bernstein E, Ferreira A. Multiple recurrences of nonfamilial cardiac myxomas: A report of two cases. Tex Heart Inst J 2007;34:236-9. |
[Figure 1], [Figure 2]
|