Primary epithelioid leiomyosarcoma of left atrium: Pathologist clinches it all

Primary cardiac neoplasms are rare with an estimated prevalence of 0.001%–0.2%. Cardiac tumors are mostly benign and myxomas are the commonest. Malignant tumors are rare in which metastasis and infiltration from the adjacent malignant tumor is more common than the primary ones. Amongst the primary malignant tumors sarcomas are the commonest. Primary epithelioid leiomyosarcoma of the heart is extremely rare. Here, we present a case of a 67-year-old female patient who was admitted with acute onset of pulmonary edema; on investigation, the patient was found to have left atrial mass. A provisional diagnosis of large left atrial myxoma with left ventricular inflow obstruction was made. Biopsy taken showed features of leiomyosarcoma which was confirmed by immunohistochemistry. Most of the patients with cardiac sarcoma have a high-grade tumor at the time of diagnosis with distant metastasis in the lungs (35.7%), lymph node (14.2%) followed by liver (7.14%) and they potent a poor prognosis. At present, only a few hundred primary cardiac sarcomas have been reported, most of which are based on autopsy series. Primary cardiac epithelioid leiomyosarcoma is a rare entity that can pose a diagnostic dilemma. However, atypical presentation of “atrial myxoma” being the common differential diagnosis. Histopathological evaluation is confirmatory to diagnose such a lesion. The pathologist should be aware of such an entity which can be attended by morphological evaluation including immunohistochemistry.