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 Table of Contents  
CASE REPORT
Year : 2016  |  Volume : 4  |  Issue : 3  |  Page : 100-103

Glandular myxoma of left atrium: An uncommon tumor


1 Department of Pathology, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra Pradesh, India
2 Department of Cardiovascular Thoracic Surgery, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra Pradesh, India
3 Department of Pathology, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra PDepartment of Cardiovascular Thoracic Surgery, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra Pradeshradesh, India

Date of Web Publication16-Sep-2016

Correspondence Address:
Rashmi Patnayak
Department of Pathology, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2321-449x.190743

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  Abstract 

In the present day, there is an overall increase in the incidence of various tumors of the body though tumors of the heart are not common. Metastatic tumors of the heart are more common than primary tumors. Among primary tumors of the heart, myxoma is the most common tumor. However, glandular differentiation in myxoma is rare. Hereby, we present a rare case report of glandular differentiation in cardiac myxoma of the left atrium.

Keywords: Benign tumors of heart, cardiac myxoma, cardiac tumors, glandular myxoma


How to cite this article:
Divya E, Rukhmangadha N, Patnayak R, Chandra A, Murthy HP. Glandular myxoma of left atrium: An uncommon tumor. Heart India 2016;4:100-3

How to cite this URL:
Divya E, Rukhmangadha N, Patnayak R, Chandra A, Murthy HP. Glandular myxoma of left atrium: An uncommon tumor. Heart India [serial online] 2016 [cited 2020 Jul 7];4:100-3. Available from: http://www.heartindia.net/text.asp?2016/4/3/100/190743


  Introduction Top


Primary tumors of the heart are rare with an incidence of 0.0017-0.33% in autopsy series. [1] Cardiac myxomas account for about half of the cardiac tumors in the elderly. They commonly occur in females during third to fifth decade. They are rarely seen in children. Myxomas can arise anywhere in the heart, but the common location is left atrium. [2] Myxoma with glandular differentiation is rare. In literature, glandular myxomas account for 5% among cardiac myxomas. [3]


  Case report Top


A 52-year-old male patient presented with chief complaints of breathlessness on exertion for the past 6 months and cough with expectoration and associated chest pain since 1 month. There was no past history of diabetes mellitus, hypertension, tuberculosis, bronchial asthma, or epilepsy. Routine hematological and biochemical investigations were within normal limits. Systemic examination including cardiovascular system and respiratory system did not reveal any abnormality. A large left atrial myxoma of size 5.9 cm × 3.5 cm protruding across mitral valve was noted in two-dimensional echocardiogram (ECHO) [Figure 1]. There was mild tricuspid regurgitation with normal left ventricular function. He underwent coronary angiography which was done under local anesthesia using modified seldinger technique, and the impression was given as normal epicardial coronaries. Later, he underwent left atrial myxoma excision. During surgery, the myxoma was found to be originated from the free wall of the left atrium. The myxoma was resected en bloc, and the iatrogenic atrial wall defect was repaired with an autologous pericardial patch. The specimen is received in the department of pathology in 10% formalin. The myxoma was grossly a gray-brown, gelatinous soft tissue mass measuring 7 cm × 5 cm × 3.5 cm with a gray-white stalk. On cut section, it was gray-brown, solid, slimy with focal gray-white to yellowish areas [Figure 2] and [Figure 3]. The sections were stained by hematoxylin and eosin stain, perls and Alcian blue periodic acid Schiff (ALPAS) stains. Microscopic examination revealed flattened lining epithelium beneath which were seen round, oval, elongated to spindle-shaped cells with scant to eosinophilic cytoplasm. These cells were seen surrounding blood vessels and as individual cells in myxomatous background. Stalk showed benign looking glands with basally placed nucleus and apical mucin with extensive stromal fibrosis [Figure 4],[Figure 5],[Figure 6],[Figure 7] and [Figure 8]. Perls stain showed hemosiderin pigment deposition and Gamna Gandy bodies [Figure 9]. ALPAS stain demonstrated mucin positivity [Figure 10]. The diagnosis was given as atrial myxoma with glandular differentiation. The patient is doing good after 2 years following surgery.
Figure 1: Two-dimensional echocardiogram showing large dumbbell-shaped myxoma protruding across mitral valve of size 10 cm × 8 cm × 5 cm

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Figure 2: Gray-brown gelatinous soft tissue mass measuring 7 cm 5 cm 3.5 cm with a gray-white stalk

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Figure 3: Cut section is gray-brown, solid, slimy with focal gray-white to yellowish areas

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Figure 4: Myxomatous areas with few round, oval to spindle cells (H and E, ×40)

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Figure 5: Myxomatous lesion lined by flattened epithelium with occasional spindle cells and hemosiderin pigment (H and E, ×20)

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Figure 6: Extensive hemosiderin pigment deposition that is Gamna Gandy bodies in a myxoid background (H and E, ×10)

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Figure 7: Stalk showing glands lined by columnar cells with intervening smooth muscle bundles and congested capillaries (H and E, ×10)

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Figure 8: Glands lined by columnar epithelium with basally placed nucleus. No nucleomegaly (H and E, ×20)

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Figure 9: Hemosiderin deposition (perls, ×20)

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Figure 10: Extensive mucin positivity (Alcian blue periodic acid Schiff, ×20)

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  Discussion Top


Primary heart tumors are mostly benign. Cardiac myxomas are the most common heart tumors. Myxomas were first described in 1845. After a long time, Anderson et al. reported epithelial differentiation of myxoma in 1946. So far in the literature till 2007, 39 cases of glandular myxoma were reported. [4]

Most of the cardiac myxomas occur sporadically, while 5-10% show familial inheritance which occur as a part of Carney complex [5] which was first described in 1985. It is an X-linked autosomal dominant disorder characterized by cardiac myxomas, mucosal and cutaneous myxomas, osteochondromyxoma, spotty skin pigmentation, myxomatous tumors of the breast, ductal adenoma of the breast, blue naevi, endocrine overactivity, and other tumors. However, in our case, it is sporadic.

Patients of cardiac myxoma present with nonspecific signs and symptoms. They include palpitation, shortness of breath, syncope and heart murmurs based on the location of the tumor and vary with size, shape, and length of the pedicle. [6] In our case, patient had shortness of breath. Most sensitive and specific imaging modality preoperatively is ECHO. [7] The characteristic morphological feature in cardiac myxoma is stellate or spindle-shaped "myxoma cells" lying in a myxoid background. [6]

Cell of origin for cardiac myxomas is still not well established. They were initially considered as an organizing thrombus. Now they are believed to arise from mesenchymal cells. These are subendocardial reserve or lepidic cells, which have divergent differentiation. [8] Hypothesis behind the glandular differentiation in myxoma include: (1) Divergent differentiation of subendocardial lepidic cells, (2) the possibility of entrapped foregut rests or, (3) progressive differentiation of myxoma into glands. Previously Linder et al. described myxomas with glandular differentiation as congenital endodermal heterotopia of atrioventricular node. [9] However, this hypothesis does not hold true due to the absence of any other element. It is difficult to prove or disprove any of the above hypotheses.

Glandular component of myxoma contains well-formed glands lined by cuboidal to columnar epithelium which is seen mostly in the base or stalk region as seen in our case. [6] It is important to identify this condition and has to be differentiate from metastatic adenocarcinoma. The latter condition shows atypical glands with frequent mitosis, including abnormal forms and areas of necrosis. In this case, history of the patient and ultrasound evaluation did not reveal any possible primary. The glands are lined by columnar cells without nucleomegaly or stratification and without cytological atypia confined to stalk. There was extensive mucin positivity. Furthermore, there were no glandular structures inside the wall of other cardiac chambers, and abdominal ultrasound was normal which rule out the possibility of metastatic adenocarcinoma.

Treatment of choice for atrial myxoma is surgical excision. Patients are generally asymptomatic after surgery. As glandular differentiation is rare, scant data are available regarding prognosis. Recurrence and metastasis have been reported in literature. [10] Hence, long-term follow-up and serial ECHO evaluation are necessary in young and familial cases.

To conclude glandular differentiation in a cardiac myxoma though rare, it is important to recognize this entity, as it leads to misdiagnosis of mucin secreting adenocarcinoma.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Tasdemir A, Canoz O, Tasdemir K. Cardiac myxoma with glandular component. Erciyes Med J 2011;33:341-4.  Back to cited text no. 1
    
2.
Reynen K. Cardiac myxomas. N Engl J Med 1995;333:1610-7.  Back to cited text no. 2
    
3.
Pucci A, Gagliardotto P, Zanini C, Pansini S, di Summa M, Mollo F. Histopathologic and clinical characterization of cardiac myxoma: Review of 53 cases from a single institution. Am Heart J 2000;140:134-8.  Back to cited text no. 3
    
4.
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.  Back to cited text no. 4
    
5.
Bertherat J. Carney complex (CNC). Orphanet J Rare Dis 2006;1:21.  Back to cited text no. 5
    
6.
Liu Y, Xu ML. Cardiac myxoma with glandular component: Case report. Chin Med J (Engl) 2006;119:174-6.  Back to cited text no. 6
    
7.
Mügge A, Daniel WG, Haverich A, Lichtlen PR. Diagnosis of noninfective cardiac mass lesions by two-dimensional echocardiography. Comparison of the transthoracic and transesophageal approaches. Circulation 1991;83:70-8.  Back to cited text no. 7
    
8.
Thornton CM, Walsh MY. Glandular differentiation in cardiac myxomata. Ir J Med Sci 1993;162:95-7.  Back to cited text no. 8
    
9.
Linder J, Shelburne JD, Sorge JP, Whalen RE, Hackel DB. Congenital endodermal heterotopia of the atrioventricular node: Evidence for the endodermal origin of so called mesotheliomas of the atrioventricular node. Hum Pathol 1985;10:736-9.  Back to cited text no. 9
    
10.
Imai Y, Taketani T, Maemura K, Takeda N, Harada T, Nojiri T, et al. Genetic analysis in a patient with recurrent cardiac myxoma and endocrinopathy. Circ J 2005;69:994-5.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10]



 

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