Indian Journal of Pathology and Microbiology
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Year : 2017  |  Volume : 60  |  Issue : 3  |  Page : 312
Cardiac myxomas with glandular elements

Department of Pathology (Cardiovascular and Thoracic Division), Seth GS Medical College, Mumbai, Maharashtra, India

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Date of Web Publication22-Sep-2017

How to cite this article:
Vaideeswar P. Cardiac myxomas with glandular elements. Indian J Pathol Microbiol 2017;60:312

How to cite this URL:
Vaideeswar P. Cardiac myxomas with glandular elements. Indian J Pathol Microbiol [serial online] 2017 [cited 2021 Jul 23];60:312. Available from: https://www.ijpmonline.org/text.asp?2017/60/3/312/215384

Myxomas are considered to be the most common primary benign cardiac tumors. They are essentially composed of stellate to plump “myxoma” or “lepidic” cells amidst a myxoid background and are often characteristically located on the left side of the interatrial septum, i.e., typical cardiac myxomas (CM).[1] Similar tumors located at other sites within the heart are termed as atypical myxomas.[2] At any given location, the tumors have a solid or papillary configuration. Although the first case had been described in 1845,[3] the histogenesis is still nebulous, and the immunohistochemical profile is still uncertain. The majority of the CMs (over 90%) occur sporadically or in isolation, and such nonsyndromic tumors are seen chiefly in women with a mean age of 50 years. However, in developing countries, the tumors present at a younger age group, usually in the fourth decade.[4] On the other hand, syndromic CMs are seen in a setting of the Carney's complex, where the patients are younger and the tumors may be multiple and/or recurrent with associated noncardiac benign tumors. The common histopathological features include arrangement of the lepidic cells in the form of rings, cords, or nests with associated secondary/degenerative changes in the form of fibrosis, fresh or old hemorrhages, calcification, Gamna-Gandy bodies, and even osseous metaplasia. Uncommon elements that are identified include clusters of hematopoietic cells,[1] thymic rests with even origin of ectopic thymoma,[5] and glandular elements, as reported in this issue.[6]

The incidence of such glandular variants of CM is very low and in a recent publication by Yuan, 100 cases published till 2013 were reviewed.[7] The occurrence of these elements had no predilection for the clinical setting (sporadic or familial), location (typical or atypical), and gross morphological type (solid or papillary). In majority of the cases, the glandular spaces are located toward the site of attachment to the interatrial septum, which gives credence to the notion that these structures may represent foregut remnants. The glandular epithelia are immunohistochemically positive for enteric markers, as highlighted by the current report,[6] and in addition, heterotopias have also been reported in the heart in the absence of myxomatous background. Immunohistochemical characterization is important as myxoma cells with cytoplasmic vacuolation can exhibit pseudoglandular pattern.[8] Papillary myxomas, owing to their very nature, are known to embolize. When such tumors exhibit glandular differentiation, the embolized fragments can also contain glands, mimicking metastatic adenocarcinoma at extracardiac sites.[9],[10] Hence, the recognition of myxoma in the background becomes crucial. It should, however, be noted that the epithelial elements can show nuclear atypia and occasional atypical mitoses or can transform truly into an adenocarcinoma.[11]

   References Top

Vaideeswar P, Butany JW. Benign cardiac tumors of the pluripotent mesenchyme. Semin Diagn Pathol 2008;25:20-8.  Back to cited text no. 1
Vaideeswar P, Gupta R, Mishra P, Lanjewar C, Raut A. Atypical cardiac myxomas: A clinicopathologic analysis and their comparison to 64 typical myxomas. Cardiovasc Pathol 2012;21:180-7.  Back to cited text no. 2
Reynen K. Cardiac myxomas. N Engl J Med 1995;333:1610-7.  Back to cited text no. 3
Aggarwal SK, Barik R, Sarma TC, Iyer VR, Sai V, Mishra J, et al. Clinical presentation and investigation findings in cardiac myxomas: New insights from the developing world. Am Heart J 2007;154:1102-7.  Back to cited text no. 4
Miller DV, Tazelaar HD, Handy JR, Young DA, Hernandez JC. Thymoma arising within cardiac myxoma. Am J Surg Pathol 2005;29:1208-13.  Back to cited text no. 5
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. 6
  [Full text]  
Yuan SM. Glandular cardiac myxoma: Case report with literature review. Folia Morphol (Warsz) 2014;73:374-82.  Back to cited text no. 7
Goldman BI, Frydman C, Harpaz N, Ryan SF, Loiterman D. Glandular cardiac myxomas. Histologic, immunohistochemical, and ultrastructural evidence of epithelial differentiation. Cancer 1987;59:1767-75.  Back to cited text no. 8
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.  Back to cited text no. 9
Moiyadi AV, Moiyadi AA, Sampath S, Kalpana SR, Mahadevan A, Shankar SK, et al. Intracranial metastasis from a glandular variant of atrial myxoma. Acta Neurochir (Wien) 2007;149:1157-62.  Back to cited text no. 10
Berger MD, Schneider J, Ballmer PE, Eckhardt BP, Dommann-Scherrer C. Mucin-producing adenocarcinoma arising in an atrial myxoma. Ann Diagn Pathol 2013;17:104-7.  Back to cited text no. 11

Correspondence Address:
Pradeep Vaideeswar
Department of Pathology (Cardiovascular and Thoracic Division), Seth GS Medical College, Parel, Mumbai - 400 012, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJPM.IJPM_448_17

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