Indian Journal of Pathology and Microbiology

: 2017  |  Volume : 60  |  Issue : 3  |  Page : 312-

Cardiac myxomas with glandular elements

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

Correspondence Address:
Pradeep Vaideeswar
Department of Pathology (Cardiovascular and Thoracic Division), Seth GS Medical College, Parel, Mumbai - 400 012, Maharashtra

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

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Vaideeswar P. Cardiac myxomas with glandular elements. Indian J Pathol Microbiol [serial online] 2017 [cited 2020 Aug 4 ];60:312-312
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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]


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