| Abstract|| |
The estimated incidence of primary cardiac tumors is extremely rare. Among it, cardiac myxoma represents the most common benign cardiac tumor constituting about 80% of cases. We are presenting a 30–year-old female with large left atrial myxoma. She was presented with severe dyspnea, palpitations, and systolic murmurs. On 2D echocardiography, left atrial mass obstructing mitral flow was noted. On cardiac magnetic resonance imaging, a single, large, mobile pedunculated mass lesion in left atrium attached to inferior interatrial septum with heterogeneous enhancement was noted, which was likely represented to be myxoma. The mass was surgically excised and valve repairing was done. We received large, solid, lobulated, gray white, soft-to-firm mass measuring 4.5 × 4.1 × 2.5 cm, and on microscopy showed cardiac myxoma with cartilaginous differentiation. We are presenting this case for its clinical, imaging, and uncommon histological features.
Keywords: Cardiac tumors, cartilaginous differentiation, mitral regurgitation, myxomas
|How to cite this article:|
Jagtap SV, Salunkhe P, Mane A, Kumbhar S, Mayekar P, Boral S. Cardiac myxoma with cartilaginous differentiation-An uncommon variant presented as mitral stenosis. Indian J Pathol Microbiol 2019;62:599-601
|How to cite this URL:|
Jagtap SV, Salunkhe P, Mane A, Kumbhar S, Mayekar P, Boral S. Cardiac myxoma with cartilaginous differentiation-An uncommon variant presented as mitral stenosis. Indian J Pathol Microbiol [serial online] 2019 [cited 2019 Dec 8];62:599-601. Available from: http://www.ijpmonline.org/text.asp?2019/62/4/599/269064
| Introduction|| |
Primary cardiac tumors are extremely rare and account for 0.0017% to 0.33% at autopsy. In 80% of surgically excised cardiac tumors, most common tumor is cardiac myxoma. Myxomas are intracavitary tumors that are located in left atrium (75%), right atrium (18%), right ventricle (8%), and left ventricle (4%), and rarely in atrioventricular valves (1%). The previous large series have reported cardiac myxoma; however, little is focused on histological findings. Herewith, we are presenting it for its clinical aspect and more emphasis on histological variant.
| Case Report|| |
A 30–year-old female presented to Cardio Vascular and Thoracic Surgery unit with complaints of severe dyspnea, and palpitations since 3 months and history of fever of 4 days. There was no history of hypertension, cough, skin pigmentation, or any abnormal endocrine manifestations. No significant family and past history. On routine investigations, complete blood count, urine analysis, liver function test, and renal function test were within normal limits. Lipid profile was within normal limits. The AntiStreptolysin O titre and Cardiolipin antibody test was negative.
On further investigation, abdominopelvic ultrasonography showed no significant abnormality. Left-sided pleural effusion was noted. On 2D echocardiogram, left ventricular ejection fraction was 55%. Anterior mitral leaflet was mildly thick. Left atrial wall showed a mass obstructing the mitral [Figure 1]a. Moderate mitral regurgitation along with dilated left atrium, right atrium, and right ventricle was noted. Severe tricuspid regurgitation and pulmonary hypertension were noted. On cardiac magnetic resonance imaging showed a large mobile mass lesion in left atrium attached to inferior interatrial septum measuring 4 × 3.4 cm. The lesion was mobile, pedunculated, and enters basal left ventricular cavity in diastole along with mitral valve. It showed heterogeneous enhancement on postcontrast study that likely represented myxoma.
|Figure 1: (a) The 2D echocardiogram showing mass from left atrial wall. (b and c) Excised left atrial mass measuring 4.5 × 4.1 × 3.5 cm|
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Patient underwent left atrial myxoma excision and mitral valve replacement surgery.
We received a single solid, irregular, lobulated, gray white to gray brown, soft tissue mass measuring 4.5 × 4.1 × 3.5 cm with focal papillary configuration [Figure 1]b and [Figure 1]c. Cut surface was glistening soft to firm, gray white, and showed areas of hemorrhages. On microscopy showed a poorly circumscribed lesion composed of bland spindle or stellate shape cells arranged in loose sheets and scattered in an abundant myxoid stroma with capillary vasculature [Figure 2]a and [Figure 2]b. Large areas of hemorrhages, congestion was noted. Areas of cartilaginous differentiation was prominent [Figure 2]c and [Figure 2]d. In some areas, cells were forming tiny aggregates and cord-like arrangement. There was absence of necrosis, mitosis, and pleomorphism. On histopathologically diagnosed as cardiac myxoma with cartilaginous differentiation from excised specimen of left atrium mass.
|Figure 2: (a and b) Photomicrograph showing tumor composed of bland spindle or stellate cells in myxoid stroma (H and E stain 40×). (c and d) Photomicrograph showing tumor with cartilaginous differentiation and areas of hemorrhages (H and E stain, 100×)|
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| Discussion|| |
Cardiac myxomas represent the most common benign cardiac tumor. The origin of cell is considered to be persistent of mesenchymal cells from embryonic residue during septation of the heart, primitive cells from fossa ovalis, cardiomyocyte progenitor cells, etc.
The cardiac myxomas are found in sporadic and familial forms. The familial myxomas are often multiple and usually recurrent. The myxomas can be a single mass as the most common finding but also noted biatrial or multifocal location.
The clinical presentation of patient is variable and depends on tumor size, location, and mobility. Most of them shows manifestation of dyspnea, palpitation, edema of lower limbs, etc., which are cardiac related. Other systemic presentations may be vertigo, syncope, fatigue, hemiparesis, hemiplegia, or manifestation of thromboemboli. The cardiac symptoms in over 50% of cases are due to mass causing valve stenosis or obstruction. Our case is presented as mitral stenosis.
Complications associated with atrial myxomas are cardiac arrhythmias, pulmonary edema, peripheral emboli, blockage of mitral heart valve, etc. Few cases may remain asymptomatic. In our case, she is presented with severe dyspnea and palpitations.
The diagnostic modalities like 2D echocardiography should be the standard method of cardiologic examination of these patients, which could considerably contribute to early diagnosis and treatment of heart myxoma. Coronary angiography and cardiac magnetic resonance imaging may provide specific additional information, which could lead to precise preoperative diagnosis. Postoperatively mass confirmation on histology is essential to find out any other differentiation in tumor.
On microscopy, tumors are variable in size ranging from 1 to 15 cm (mean 5 cm). There are two types of appearance noted: solid/polypoidal-type and papillary-type.
The histological diagnosis of myxomas shows mesenchymal cells arranged in sheets, clusters, or scattered singly. The round, fusiform, or stellate cells having round or oval nuclei of mild eosinophilic cytoplasm. The stroma is myxomatous, rich in collagen, proteoglycan, and elastin with rich fine capillaries. Associated finding of fibrinoid necrosis, hemorrhage, calcification, macrophages, and inflammatory cells have been noted.
On histomorphologically, different types of mesenchymal differentiation are noted in myxomas, which include neural, osseous cartilaginous, vascular, and glandular types. The uncommon elements that are identified include clusters of hematopoietic cells and thymic rests with even origin of ectopic thymoma.,, It is observed that variable fibrosis, calcification, and Gamna–Gandy bodies are noted in myxoma. The nonembolic clinical presentation of myxoma cases were significantly associated with a fibrosis and gamma bodies.
On Periodic Acid–Schiff (PAS) staining, stromal matrix appears purple red, whereas mucous halo appears blue.
The immunohistochemistry markers like S100 protein, cytokeratin, desmin, CD34, CD68, vimentin, calretinin have been shown in various studies. Highly proliferative angiogenic cardiac myxomas on molecular analysis have shown over expression of VEGF, PCNA, IL6, FGF.,
Cardiac myxomas are usually benign; however, occasional cases of malignancy with features of recurrence, local invasion, and extracardiac extension have been rarely reported. The intracardiac recurrence has been noted in 12%–22% of familial and 1%–4% of sporadic cases. The systemic embolization and metastasis from cardiac myxomas to brain and bone have been reported.
The treatment is surgical minimal invasive excision of tumor usually through median sternotomy and cardiopulmonary bypass and valve repairing surgery is the modality of choice. Care should be taken to prevent embolic phenomenon and its complications. Complete surgical excision of myxoma is the only and very successful therapeutic method surgical with excellent prognosis. In follow-up, it is necessary to perform annual echocardiography throughout a patient's life, and particularly in patients with multifocal, atypical, or familial myxomas.
| Conclusion|| |
We are presenting this case of cardiac myxoma with cartilaginous differentiation from excised specimen of left atrium mass for its extreme rarity, clinical, imaging, and uncommon histological features.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
Wold LE, Lie JT. Cardiac myxomas: A clinicopathologic profile. Am J Pathol 1980;101:219-40.
Kumar BV, Abbas AK, Fausto N. Cardiac tumors. In: Kumar BV, Abbas AK, Fausto N, Mitchell R, editors. Robbins Basic Pathology. 8th
ed. Philadelphia: Saunders; 2007. p. 417-8.
Amano J, Kono T, Wada Y, Zhang T, Koide N, Fujimori M, et al
. Cardiac myxoma: Its origin and tumor characteristics. Ann Thorac Cardiovasc Surg 2003;9:215-21.
Swartz MF, Lutz CJ, Chandan VS, Landas S, Fink GW. Atrial myxomas: Pathologic types, tumor location, and presenting symptoms. J Card Surg 2006;21:435-40.
St John Sutton MG, Mercier LA, Giuliani ER, Lie JT. Atrial myxomas: A review of clinical experience in 40 patients. Mayo Clin Proc 1980;55:371-6.
Nath D, Arava S, Ray R, Bhoje AK, Saxena R, Chaudhary SK. Immunohistochemical characterization of glandular elements in glandular cardiac myxoma: Study of six cases. Indian J Pathol Microbiol 2017;60:319-23.
] [Full text]
Miller DV, Tazelaar HD, Handy JR, Young DA, Hernandez JC. Thymoma arising within cardiac myxoma. Am J Surg Pathol 2005;29:1208-13.
Vaideeswar P. Cardiac myxomas with glandular elements. Indian J Pathol Microbiol 2017;60:312.
] [Full text]
Barh D, Kumar A, Chatterjee S, Liloglou T. Molecular features, markers, drug targets and prospective targeted therapeutics in cardiac myxomas. Curr Cancer Drug Targets 2009;9:705-16.
Gosev I, Paic F, Đuric Z, Gošev M, Ivcevic S, Bulic Jakuš F, et al
. Cardiac myxoma the great imitators: Comprehensive histopathological and molecular approach. Int J Cardiol 2013;164:7-20.
Altundag MB, Ertas G, Ucer AR, Durmus S, Abanuz H, Lu TC. Brain metastasis of cardiac myxoma: Case report and review of the literature. J Neurooncol 2005;75:181-4.
Sunil V Jagtap
Professor, Department of Pathology, Krishna Institute of Medical Sciences, Deemed University, Karad - 415 110, Maharashtra
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2]