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 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 5  |  Issue : 2  |  Page : 119-121

Cardiac myxoma with glandular elements: An unusual histologic variant with a brief review of the literature


1 Department of Pathology, North Eastern Indira Gandhi Regional Institute of Health and Medical Sciences, Shillong, Meghalaya, India
2 Department of Cardiothoracic and Vascular Surgery, North Eastern Indira Gandhi Regional Institute of Health and Medical Sciences, Shillong, Meghalaya, India

Date of Submission14-Apr-2019
Date of Decision13-May-2019
Date of Acceptance04-Jun-2019
Date of Web Publication19-Aug-2019

Correspondence Address:
Dr. Biswajit Dey
Department of Pathology, North Eastern Indira Gandhi Regional Institute of Health and Medical Sciences, Shillong - 793 018, Meghalaya
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jpcs.jpcs_29_19

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  Abstract 


Cardiac myxoma with glandular elements is an unusual histologic variant of cardiac myxoma and constitutes 2%–5% of all cardiac myxomas. Here, we report a case of cardiac myxoma with glandular elements in a 34-year-old male, who presented with right-sided hemiparesis following a cardiovascular attack 2 months back. Echocardiography revealed a large right atrial mass measuring 3.5 cm × 3.5 cm × 2 cm. Under cardiopulmonary bypass, left atrial mass was excised. Histopathological and immunohistochemical examination of the mass confirmed the diagnosis of cardiac myxoma with glandular elements. It is important to recognize this variant to avoid misdiagnosis of metastatic adenocarcinoma due to its rarity as well as due to its unusual morphological features.

Keywords: Adenocarcinoma, echocardiography, myxoma


How to cite this article:
Dey B, Raphael V, Khonglah Y, Jyoti PK. Cardiac myxoma with glandular elements: An unusual histologic variant with a brief review of the literature. J Pract Cardiovasc Sci 2019;5:119-21

How to cite this URL:
Dey B, Raphael V, Khonglah Y, Jyoti PK. Cardiac myxoma with glandular elements: An unusual histologic variant with a brief review of the literature. J Pract Cardiovasc Sci [serial online] 2019 [cited 2019 Sep 15];5:119-21. Available from: http://www.j-pcs.org/text.asp?2019/5/2/119/264625




  Introduction Top


Primary cardiac tumors are rare, accounting for 0.0017%–0.03% in autopsy series and most often represented by a myxoma in 50% of cases in the adult population.[1] Cardiac myxoma with glandular elements is a rare entity, constituting 2%–5% of all cardiac myxomas.[2],[3] First described by Anderson and Dmytryk in 1946, cardiac myxoma with glandular elements is histologically characterized by a classical cardiac myxoma with benign glandular elements.[3],[4] The diagnosis of cardiac myxoma is usually straightforward; however, cardiac myxoma with glandular elements may be misdiagnosed due to its rarity as well as due to unusual morphological features. We report a case of cardiac myxoma with glandular elements in a 34-year-old male, who presented with right-sided hemiparesis.


  Case Report Top


A 34-year-old male presented with right-sided hemiparesis following a cardiovascular attack 2 months back. There was no history of fever or myalgia. Family history was unremarkable. Cardiovascular examination revealed murmurs in the mitral area. On neurological examination, the patient had right-sided hemiplegia with left facial weakness.

Echocardiography revealed a large right atrial mass suggestive of intracavitary thrombi or blood clot with moderate mitral regurgitation and mild stenosis [Figure 1]a. Under cardiopulmonary bypass, left atrial mass was excised and sent for histopathological examination. Grossly, the mass was globular, measuring 3.5 cm × 3.5 cm × 2 cm [Figure 1]b. On cut section, it was solid, gray-white in color with gelatinous areas. Microscopic examination showed lipidic/stellate cells embedded in a loose myxoid stroma [Figure 1]c. There were thin- and thick-walled blood vessels. Also seen were glandular structures lined by cuboidal to columnar cells with mucin droplets and thin basophilic secretions in the lumen. These glandular components were found near the attachment and occupied around 5% of the total area of the myxoma. Staining with histochemical stain Alcian blue-periodic acid–Schiff (AB-PAS) at pH 2.5 showed apical positivity highlighting the acidic mucin. On immunohistochemistry, the glandular elements were positive for pan-cytokeratin, CK7, and epithelial membrane antigen (EMA) [Figure 1]d. CK20, CDX2, thyroid transcription factor-1 (TTF-1), calretinin, and prostate-specific antigen (PSA) were negative in the glandular elements. The stellate cells were positive for CD34, CD31, and calretinin. The stellate cells were negative for pan-cytokeratin. A final diagnosis of cardiac myxoma with glandular elements was made.
Figure 1: (a) Echocardiography showing left atrial mass. (b) Gross photomicrograph shows a gelatinous mass. (c) Histopathology showing irregular glands lined by columnar cells with mucin droplets in a myxomatous stroma (H and E, ×40). (d) Glandular elements positive for CK7 (Immunohistochemical, ×10)

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The postoperative period was uneventful, and the patient was discharged on the 7th postoperative day with advice of regular follow-up.


  Discussion Top


Cardiac myxoma is generally considered to be a mesenchymal neoplasm.[3] The most common sites are the left atrium (75%), followed by the right atrium (18%), the right and left ventricles (3% in each), and the valves (1%).[1] Multiple myxomas are also known and represent 5% of the cases.[1] The clinical manifestations of cardiac myxoma depend on the tumor location, size, mobility, and consistency.[2] The patients usually present with cardiac symptoms due to intracardiac flow obstruction by the tumor.[1] About 30%–40% of patients also suffer from embolization to other organs such as the central nervous system, kidney, spleen, and extremities.[5] In the present case, the patient had an embolic phenomenon, resulting in right-sided hemiparesis.

The majority of the cardiac myxomas present sporadically; however, they can be associated with familial syndromes such as Carney's complex.[6] Those associated with familial syndromes have a younger age, higher frequency of multifocal locations, and recurrence.[6] Cardiac myxoma with glandular elements is mostly sporadic and mainly occurs in the left atrium of adults with a female preponderance.[5] A few cases of cardiac myxoma with glandular elements have been reported in the pediatric age group.[6]

Echocardiography is the most commonly used methodology for the diagnosis of cardiac myxomas.[7] However, the echocardiographic diagnosis of myxomas is not always easy and straightforward.[7] The echocardiographic differential diagnoses of cardiac myxomas include metastatic tumors, intracardiac thrombi, and vegetations.[7] In the present case, the preoperative echocardiographic diagnosis was intracardiac thrombus or blood clot. The pointers for glandular differentiation in a cardiac myxoma include the presence of internal irregularities, heterogeneity, or cystic areas.[2] Both computed tomography and magnetic resonance imaging can differentiate myxomas from other intracavitary cardiac masses such as thrombi and vegetations.[2]

Cardiac myxoma is a neoplasm composed of stellate to plump, cytologically bland, and mesenchymal cells embedded within a myxoid stroma.[1] Cardiac myxoma with glandular elements has benign glandular elements in addition to the classical features of cardiac myxoma.[3] These well-formed glandular structures are lined by columnar cells with variable mucin-secreting goblet cells, and these structures typically constitute <15% of the tumor mass.[2],[8] The histogenesis of the glandular elements is enigmatic. It is hypothesized that these glandular elements arise due to divergent differentiation of subendocardial lepidic cells or from the progressive differentiation of lipidic cells of myxoma into glands.[6] Some also opine that they arise from the remnants of entrapped foregut rests.[6] Positivity with AB-PAS stain at pH 2.5 suggests the presence of acidic mucin favoring colonic epithelial differentiation.[3] Immunohistochemically, these glandular elements are positive for pan-cytokeratin, CK7, CAM5.2, EMA, and carcinoembryonic antigen (CEA) while negative for CK20 and CDX2.[3],[8] Mesenchymal cells or endothelial cells do not show immunoreactivity for pan-cytokeratin, CK7, EMA, or CEA.[3] In the present case, the glandular elements were positive for pan-cytokeratin, CK7, EMA, and CEA while negative for CK20. These immunohistochemical (IHC) findings suggest that the presence of these glandular elements is due to epithelial differentiation toward coelomic epithelium from the undifferentiated precursor cells of the subendocardium.[3],[7] Unlike the stellate cells, calretinin was positive in the glandular elements suggesting that the glandular elements are unlikely to arise from the mesothelial cells.[3]

The important differential diagnosis of cardiac myxoma with glandular elements is metastatic adenocarcinoma, which is one of the most common metastatic malignancies to the heart.[8]

This becomes important in patients who have a history of adenocarcinoma.[6] The points which differentiate cardiac myxoma with glandular elements from metastatic adenocarcinoma are that the former lacks cytologic atypia, no mitotic activity, and the absence of necrosis.[6],[8] In cases, where metastasis is suspected, organ-specific IHC markers such as TTF-1, calretinin, CK20, CDX2, PSA, and gross cystic disease fluid protein-15 may be done. Although in the present case, there was no history of malignancy, organ-specific IHC markers were done to rule out metastasis from an occult primary. TTF-1 (to rule out lung adenocarcinoma), calretinin (to rule out mesothelioma), PSA (to rule out prostatic adenocarcinoma), and CK20 and CDX2 (to rule out colorectal adenocarcinoma) were done, and all these markers were negative in the present case.

Complete surgical excision is the treatment of choice for cardiac myxoma or glandular cardiac myxoma.[3] Although cardiac myxoma with glandular elements usually has an excellent prognosis, cases with recurrence or metastasis have been reported.[8] Therefore, long-term surveillance with echocardiography is warranted.[8]

Cardiac myxoma with glandular elements is an unusual histologic variation of cardiac myxoma with a benign course. The glandular elements are considered to arise as a result of epithelial differentiation toward coelomic epithelium from the undifferentiated pluripotent subendocardial cells. It is important to recognize this uncommon entity to avoid misdiagnosis of metastatic adenocarcinoma.

Declaration of patient consent

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

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.

Ethics clearance

As per institute's guidelines, ethical clearance not required for case reports. However the authors certify that they have obtained all appropriate patient consent forms. In the form the patient has given his/her consent for his/her images and other clinical information to be reported in the journal. The patient understand that his/her name and initials will not be published and due efforts will be made to conceal his/her identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Aiello VD, de Campos FP. Cardiac myxoma. Autops Case Rep 2016;6:5-7.  Back to cited text no. 1
    
2.
Flint N, Siegel RJ, Bannykh S, Luthringer DJ. Bi-atrial cardiac myxoma with glandular differentiation: A case report with detailed radiologic-pathologic correlation. Eur Heart J Case Rep 2018;2:yty045.  Back to cited text no. 2
    
3.
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.  Back to cited text no. 3
[PUBMED]  [Full text]  
4.
Anderson WA, Dmytryk ET. Primary tumor of the heart containing epithelium-like elements. Am J Pathol 1946;22:337-49.  Back to cited text no. 4
    
5.
Nath D, Arava S, Ray R, Bhoje AK, Saxena R, Chaudhary SK. Familial biatrial cardiac myxoma with glandular elements: A rare entity with review of literature. Indian J Pathol Microbiol 2017;60:568-70.  Back to cited text no. 5
[PUBMED]  [Full text]  
6.
Mallick SR, Das P, Shukla B, Kothari S, Devagourou V, Ray R. Right atrial myxoma with glandular differentiation: A rare entity in pediatric age group. Ann Pediatr Cardiol 2010;3:159-62.  Back to cited text no. 6
    
7.
Park H, Jo S, Cho YK, Kim J, Cho S, Kim JH, et al. Differential diagnosis of a left atrial mass after surgical excision of myxoma: A remnant or a thrombus? Korean Circ J 2016;46:875-8.  Back to cited text no. 7
    
8.
Zhang M, Ding L, Liu Y, Xue L. Cardiac myxoma with glandular elements: A clinicopathological and immunohistochemical study of five new cases with an emphasis on differential diagnosis. Pathol Res Pract 2014;210:55-8.  Back to cited text no. 8
    


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