Familial biatrial cardiac myxoma with glandular elements: A Rare entity with review of literature

Devajit Nath; Sudheer Arava; Ruma Ray; Amol Kumar Bhoje; Rachit Saxena; Shiv Kumar Chaudhary

doi:10.4103/IJPM.IJPM_211_16

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CASE REPORT

Year : 2017 | Volume : 60 | Issue : 4 | Page : 568-570

Familial biatrial cardiac myxoma with glandular elements: A Rare entity with review of literature

Devajit Nath¹, Sudheer Arava¹, Ruma Ray¹, Amol Kumar Bhoje², Rachit Saxena², Shiv Kumar Chaudhary²
¹ Department of Pathology, All India Institute of Medical Sciences, New Delhi, India
² Department of Cardiothoracic and Vascular Surgery, All India Institute of Medical Sciences, New Delhi, India

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Date of Web Publication

12-Jan-2018

Abstract

Cardiac myxomas are benign neoplasm of the heart with an incidence of 0.3%. Glandular cardiac myxomas are very rare and accounts for less than 3% of all cardiac myxomas. Here, we report a case of familial glandular cardiac myxoma in a 35 year old male who complained of exertional dyspneoa and weakness of right side of body on clinical presentation. Associated features of Carney's complex were not present. Family history revealed presence of cardiac myxoma in younger brother and sister. Transthoracic echocardiography detected biatrial myxoma. Excision of both lesions was done under cardiopulmonary bypass. Histopathology confirmed myxoma with glandular elements. Postoperative course was uneventful.

Keywords: Biatrial, familial, glandular cardiac myxoma

How to cite this article:
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

How to cite this URL:
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 [serial online] 2017 [cited 2023 Sep 30];60:568-70. Available from: https://www.ijpmonline.org/text.asp?2017/60/4/568/222960

Introduction

Myxomas are the most common primary cardiac tumors with an overall incidence of 0.3%.^[1] Most common site is in the left atrium (LA) (75%) followed by right atrium (RA) (25%). Rarely, they may also occur in the ventricles, aorta, pulmonary artery, and inferior vena cava. Biatrial myxomas are extremely rare and commonly seen in familial conditions.^[2] Clinical signs and symptoms depend on the site and size of the tumor. Most common complications include obstruction to the blood flow, thrombus formation and even sudden cardiac death. The treatment of choice is surgical excision. Glandular cardiac myxomas arising from two different heart chambers are extremely rare with only one reported case in the literature. Due to its rarity, the present case has been discussed with review of literature.

Case Report

A 35-year-old male presented with a history of weakness in the right side of the body and dyspnea on exertion for the last 3 months without any history of syncope, fever, or myalgia. Past history revealed stroke 1 year back with right-sided hemiplegia. Family history revealed elder brother having stroke with recurrent LA myxoma for which he underwent open heart surgery twice. Screening of younger brother and two sisters revealed LA mass in one sister. Thorough clinical search for carney complex was negative. Cardiovascular examination revealed murmurs in the mitral and tricuspid area. Neurological examination revealed right-sided hemiplegia with left facial weakness. Electrocardiography recordings and chest radiograph were normal.

Echocardiography showed a large RA mass with moderate tricuspid regurgitation and LA mass with moderate mitral regurgitation and mild stenosis. Biventricular function was normal. Noncontrast computed tomography scan head showed an old left middle cerebral artery territory infarct.

Under cardiopulmonary bypass, biatrial mass was excised and sent for histopathological examination. On gross examination, they were gelatinous in appearance with LA mass measuring 3.5 cm × 2 cm × 1 cm, while the RA mass measuring 3 cm × 2 cm × 2 cm. Microscopically [Figure 1], both the tumor showed stellate/lepidic cells in a loose myxoid stroma. Some of these cells were arranged in solid cords and vague vascular channels. In addition, glandular component consisting of irregularly shaped glands lined by columnar epithelial cells with focal mucin production was also noted without any evidence of pleomorphism or nuclear atypia. Immunohistochemically, the stellate cells were immunopositive for CD34, CD31, calretinin and were negative for pan cytokeratin. The glandular elements were diffusely positive for pan cytokeratin, CK7, CK19, epithelial membrane antigen (EMA), and carcinoembryonic antigen (CEA) while negative for CK20, thyroid transcription factor-1, chromogranin, and synaptophysin. Proliferative (MIB-1 labeling index) index of the tumor was approximately 2%. Based on the above histological and immunohistochemical features, a diagnosis of glandular cardiac myxoma was made. Echocardiogram, done during immediate postoperative period and before the discharge, showed no residual tumor. Thorough search for any occult malignancy was negative. The patient recovered well without any postoperative complications and discharged after 6 days. Postoperative period till date after 1 year is uneventful.

Figure 1: Glandular cardiac myxoma: (a) Intraoperative right atrial myxoma with a pedicle (b) Excised biatrial myxomas. Surface is globular and shows dark brown hemorrhagic discoloration. (c) Microscopy showed the presence of benign glandular elements with goblet cells (d) Alcian blue-periodic acid-Schiff stain showed the presence of intracellular mucin (arrow) (e) Glandular elements showing strong immunopositivity for cytokeratin

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Discussion

Multiple intracardiac myxomas constitute <5%^[1] and most of these are familial_. The origin of cardiac myxoma is thought to be from remnants of sub endocardial vasoformative reserve cells or multipotential primitive mesenchymal cells in the fossa ovalis and the surrounding endocardium.

Glandular cardiac myxoma is characterized by classical myxoma with benign glandular elements.^[2] It is mostly sporadic and mainly occurs in the LA of adults with female predominance.[3]A knowledge regarding the glandular structures in myxoma is important and failure to do so may lead to an erroneous diagnosis of secondary mucin secreting adenocarcinoma.^[4]

Approximately 7% of cardiac myxomas are familial or part of the syndrome with complex abnormalities that consist of myxomas in other locations (breast or skin), spotty skin pigmentation (lentigines, pigmented nevi, or both) and endocrine over activity (pituitary adenoma, primary pigmented nodular adrenocortical disease or testicular tumor involving the endocrine component).^[5] In contrast to sporadic myxoma, familial or syndromic myxoma tumors tend to occur in younger individuals are more often multiple in location and are more likely to have postoperative recurrences, probably reflecting their multicentric nature. As we could not demonstrate any clinical syndromic manifestations in our case, it should be categorized as familial nonsyndromic benign glandular cardiac myxoma.

Clinical presentation of cardiac myxoma depends on the location and size of the tumor.^[6] Cardiac symptoms in over 50% of cases are due to tumor causing valve stenosis or obstruction.^[7] Other most common presentation is embolization to central nervous system, kidney, spleen and extremities.

Immunohistochemically, the stellate cells show positivity for calretinin and negativity for cytokeratin. On special histochemical stains, the glandular structures show positivity for epithelial, neutral or acidic mucin and immunohistochemically, they show positivity for epithelial markers such as cytokeratin, EMA and CEA.

Glandular myxomas are considered as a histologically benign cardiac tumor. Recurrence and malignant transformation are extremely rare like any other myxomas.^[8] Therefore, in any case of multiple cardiac myxoma, especially in young individuals with familial history, it is mandatory to advice long-term regular postoperative follow-up and interval echocardiography to see any evidence of early recurrences. Sporadic myxomas have good prognosis with 1%–3% recurrence rate; however, familial myxomas have 10% recurrence with the development of another tumor in a different location within 5 years of postoperative period.^[9] Surgical wide resection of the myxoma with excision of adjacent normal cardiac tissue is the mainstay of treatment in these type of cases to prevent recurrences in the future.

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

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2.	Peachell JL, Mullen JC, Bentley MJ, Taylor DA. Biatrial myxoma: A rare cardiac tumor. Ann Thorac Surg 1998;65:1768-9.
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4.	Namura O, Saitoh M, Moro H, Watanabe H, Sogawa M, Nishikura K, et al. Acase of biatrial multiple myxomas with glandular structure. Ann Thorac Cardiovasc Surg 2007;13:423-7.
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8.	Nina VJ, Silva NA, Gaspar SF, Rapôso TL, Ferreira EC, Nina RV, et al. Atypical size and location of a right atrial myxoma: A case report. J Med Case Rep 2012;6:26.
9.	Uppin SG, Jambhekar N, Puri A, Kumar R, Agarwal M, Sanghvi D, et al. Bone metastasis of glandular cardiac myxoma mimicking a metastatic carcinoma. Skeletal Radiol 2011;40:107-11.