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Abstract
Uterine leiomyosarcoma (ULMS) is a rare malignancy arising from the myometrial smooth muscle wall, and cardiac metastases are extremely rare. Metastasis to the heart is a very unusual finding, and atrial metastasis is even rarer. Here, we report a case of a 45 year old woman who presented with dyspnea and pleural effusion and had a significant history of hysterectomy done for ULMS. Magnetic resonance imaging revealed a left atrial mass, which was resected and revealed to be a metastasis of leiomyosarcoma on histopathology and immunohistochemistry. Metastatic ULMS may rarely present as a left atrial mass with acute clinical presentation. Detailed clinical history and accurate diagnosis are vital for further management.
Keywords: Left atrial mass, metastasis, right lung mass, uterine leiomyosarcomas
How to cite this article: Introduction 
Uterine leiomyosarcoma (ULMS) is a rare entity and accounts for 1% to 2% of malignancies in postmenopausal women.[1] It is a highly aggressive tumor with high predilection for metastasis to the lung, liver, and less frequently to the bone and brain. Symptomatic cardiac metastasis is exceptionally rare. Cardiac metastasis may progress to complications such as heart failure and shock, if not diagnosed early in ULMS patients.[2] Very few cases of ULMS with atrial metastasis have been reported in the literature. Here, we report the case of a 45-year-old woman with a history of ULMS diagnosed 7 years back who presented with left atrial metastasis leading to pericardial and pleural effusion.
Case Report A 45-year-old female presented to the emergency department with complaints of progressive shortness of breath associated with progressive edema of both lower extremities. The patient was dyspneic with a respiratory rate of 23, SpO2 92%, and a blood pressure of 170/110 mmHg. There was no history of chest pain, nausea, diaphoresis, dizziness, fever, or chills associated with her shortness of breath. There was no associated cough, abdominal pain, diarrhea, or emesis. The patient gave a history of hysterectomy 7 years before the current presentation. At that time, she was evaluated for a large mass in the uterus, and a hysterectomy was performed. The specimen sample was sent to a tertiary-center pathology department, and histopathology revealed the uterine mass to be a low-grade leiomyosarcoma.
On physical examination, significant distant heart sounds, mild abdominal distention, and bilateral lower limb edema were noticed. Echocardiography showed large hemodynamically significant pericardial effusion with both right ventricular and right atrial collapse. A computed tomography (CT) scan showed a large pericardial effusion, with a small right pleural effusion, and anterior cardiac wall density. Positron emission tomography–computed tomography (PET-CT) scan showed significant uptake in the left atrial mass and right lung mass [Figure 1].
Figure 1: (a) Sagittal reformatted contrast-enhanced CT chest image shows a large hypodense mass lesion in the left atrium (black arrow). (b) Coronal reformatted PET-CT image shows a large mass lesion in the left atrium with significant fluorodeoxyglucose (FDG) uptake (black arrow) and metastatic lesion in the right lung with FDG uptake (white arrow). (c) Gross specimen shows a well-encapsulated lesion with a gray-white cut surface. (d) Microscopy shows spindle to pleomorphic hyperchromatic nuclei with atypical mitosis (black arrow) (hematoxylin and eosin, 40×)The early clinical diagnosis was left atrial myxoma. The patient underwent cardiac surgery for resection of the atrial mass. Operative findings revealed a large tumor with a broad-based attachment to the medial wall of the left atrium. Grossly, the atrial mass measured about 7.5 × 6.8 × 3.6 cm and was smooth and nodular in appearance with gray-white shiny areas on the cut surface. Histopathology sections of the atrial mass showed a lesion composed of spindle cells arranged in fascicles showing moderate to marked nuclear pleomorphism. Areas of tumor necrosis and atypical mitoses were present with hot areas showing >10 mitoses/10 high-power fields. Focal myxoid areas were also seen. Immunohistochemistry was performed, and the lesional cells were revealed to be Desmin and smooth muscle actin (SMA) positive and Ki-67 showing high index, consistent with high-grade leiomyosarcoma [Figure 1] and [Figure 2]. The patient was referred to the medical oncology and radiotherapy department for further appropriate chemotherapy and radiotherapy and was subsequently discharged.
Figure 2: (a) Section shows fascicles of tumor cells having spindle hyperchromatic nuclei with indistinct eosinophilic cytoplasm (hematoxylin and eosin, 10×). (b) Tumor cells show focal positive for SMA. (c) Strong positivity for Desmin. (d) Ki-67 showing high proliferative index, about 20% to 25% Discussion ULMS is a rare malignant mesenchymal neoplasm arising from smooth muscle cells of the myometrium. It constitutes 1% to 2% of malignant uterine tumors; however, highly aggressive potential often leads to unforeseen clinical course.[1] The most common sites for metastasis include the lung, peritoneum, bones, and liver. ULMS commonly metastasize through the hematogenous route. Survival is primarily dependent on the metastasis, with a 5-year survival rate of 91.2% and 41.2% for nonmetastatic and metastatic ULMS, respectively.[3] A 5-year disease-specific survival of ULMS is determined by factors such as age, race, pathological stage, mitotic index, and lymphovascular invasion.[4]
The first case was published by Rosenblatt and Featherston[5] in 1960. This case series detected six cases as primary ULMS out of the total 2,000 cases of metastatic cardiac tumors studied.[5] In our case, the patient presented with cardiac metastasis after 7 years of hysterectomy, whereas another study showed cardiac metastasis after 16 years of primary resection.[6] Cardiac metastasis may also be diagnosed on autopsy with significant history of uterine mass.[7] ULMS spreads to the heart and lung via the hematogenous route, and it may present with an evidence of vascular spread in the form of multiple intravenous tumor foci.[8] Suggestive cardiac symptoms along with the utility of echocardiography and PET-CT scan are helpful to rule out metastasis.[9],[10] On imaging, metastasis may show variable anatomic distribution in the heart, which may be extensive involving multiple chambers and blood vessels,[11] single chamber involvement,[12] tricuspid valve infiltration,[13] or interventricular septal involvement.[14] Cardiac metastasis of ULMS may show variable clinical presentation as reported by Barış et al.[15] and Karass et al.,[16] where the patients presented with acute coronary syndrome and hypertrophic obstructive cardiomyopathy, respectively. Histology of the tumor reveals spindle cells with eosinophilic cytoplasm and marked nuclear atypia. Frequent mitosis and necrosis is often present. Immunohistochemistry (IHC) shows positivity for smooth muscle actin and Desmin.[13],[17]
Surgical resection is the preferred treatment for the intracardiac spread of ULMS. In cases where surgery is not possible or in advanced cases, additional treatment with chemotherapy and/or radiotherapy is proposed. Reconstructive surgery might be performed in case of a cardiac wall defect.[18],[19]
In recent literature, very few cases have been reported.[20],[21] Taking into consideration the metastatic potential of ULMS, this case has been presented to show the rare metastasis to the heart. In our case, the patient had an atrial mass and the differential diagnosis included were primary cardiac tumors such as atrial myxoma, intracardiac clot, or any metastasis. Echocardiography is the easiest and most widely available method to assess the cardiac chambers. Preoperative thoracic imaging (CT scan or radiography) is also suggested during the initial diagnostic workup. Pathological diagnosis with IHC confirmation assists in excluding other mesenchymal tumors [Table 1].[10],[13],[20]
Table 1: Literature search of cardiac metastasis from uterine leiomyosarcoma (ULMS) Conclusion We presented this case to indicate the aggressiveness of ULMS and its potential for heart metastasis, albeit very rare. Thus, the clinicians treating patients must be aware of the propensity of these tumors for metastasis, sometimes to unexpected sites. In patients showing cardiac symptoms with a past history of leiomyosarcoma, we must recognize the utility of echocardiography and CT scan for diagnosis along with histopathological and IHC confirmation with cognizance of the need to carry out surgical ablation for better survival chance.
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References 
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CheckDOI: 10.4103/ijpm.ijpm_577_21
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