A comprehensive diagnostic approach to primary cardiac tumors: a case report

    Authors

    Keywords

    myxoid pleomorphic liposarcoma, pericardial effusion, primary cardiac tumor

    DOI

    https://doi.org/10.15836/ccar2025.161

    Full Text

    **Introduction:** Primary cardiac tumors are a rare entity, accounting for 0.3-0.7% of all cardiac tumors. Only 25% of primary cardiac tumors are malignant, and of these, 75% are sarcomas. Malignant primary cardiac tumors typically affect young patients and have poor prognosis, with a one-year survival rate of around 10% (1, 2). **Case report:** 35-year-old female, without any known previous comorbidities, was admitted with suspected pericarditis. The patient had experienced flu-like symptoms one month prior to admission, and her symptoms of fever (up to 38.1°C) and chest pain with inspiration began a few days before admission. Initial laboratory work-up revealed anemia (hemoglobin 99 g/L), slightly elevated leukocytes (11.3x10^9/L), and elevated CRP (249 ng/L), with normal troponin levels. Chest X-ray revealed an enlarged heart silhouette, suggestive of pericardial effusion, and small bilateral pleural effusions. Initial echocardiography showed a large circumferential pericardial effusion (maximum of 3 cm) and a large mass infiltrating the right ventricle (67x23 mm), along with a smaller mass infiltrating the left ventricle (**Figure 1**). Given the suspicion of a cardiac tumor, detailed radiological diagnostics were performed. Multislice spiral computed tomography (MSCT) showed a hypovascular tumor infiltrating the right ventricle with characteristics suggestive of sarcoma (**Figure 2**). One of the branches of the right coronary artery was infiltrated by the tumor. Magnetic resonance imaging revealed a large (7.3x5.3x7.5 cm) expansive, inhomogeneous mass between the pericardial layers, infiltrating the right ventricular wall, and showing radiological characteristics of sarcoma. The pericardium was thickened, with dense pericardial effusion (**Figure 3**). Multiple intracardiac echocardiography and MSCT-guided biopsies were performed. The final histopathological diagnosis was myxoid pleomorphic liposarcoma, an extremely rare and aggressive subtype of liposarcoma with a predilection for the mediastinum, particularly affecting young adults (2). Staging with positron emission tomography did not show signs of metastatic dissemination. Given the high surgical risk, the multidisciplinary team recommended chemotherapy with Adriamycin and Ifosfamide as the first-line treatment but later cardiectomy and implantation of two HeartMate3 ventricular assist devices as form of total artificial heart was performed. FIGURE 1. Echocardiography showing a large mass infiltrating the right ventricular wall. FIGURE 2. Multislice spiral computed tomography of the tumor. FIGURE 3. Magnetic resonance imaging of the tumor. **Conclusion:** Although primary cardiac tumors are extremely rare, a comprehensive diagnostic approach using multiple imaging modalities is essential in cases of suspicion.

    Literature

    1. Leja MJ, Shah DJ, Reardon MJ. Primary cardiac tumors. Tex Heart Inst J. 2011;38(3):261–2. https://pubmed.ncbi.nlm.nih.gov/21720466/
    2. Dermawan JK, Hwang S, Wexler L, Tap WD, Singer S, Vanderbilt CM, et al. Myxoid pleomorphic liposarcoma is distinguished from other liposarcomas by widespread loss of heterozygosity and significantly worse overall survival: a genomic and clinicopathologic study. Mod Pathol. 2022 November;35(11):1644–55. https://doi.org/10.1038/s41379-022-01107-6
    Cardiologia Croatica
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    A comprehensive diagnostic approach to primary cardiac tumors: a case report

    Extended Abstract
    Issue5-6
    Published
    Pages161-162
    PDF via DOIhttps://doi.org/10.15836/ccar2025.161
    myxoid pleomorphic liposarcoma
    pericardial effusion
    primary cardiac tumor

    Authors

    Dubravka Šipuš*ORCIDUniversity Hospital Centre Zagreb, Zagreb, Croatia
    Ana Maria SliškovićORCIDUniversity Hospital Centre Zagreb, Zagreb, Croatia
    Richard MatasićORCIDUniversity Hospital Centre Zagreb, Zagreb, Croatia
    Davor RadićORCIDUniversity Hospital Centre Zagreb, Zagreb, Croatia
    Petra Angenbrandt BeloševićORCIDUniversity Hospital Centre Zagreb, Zagreb, Croatia
    Eduard MargetićORCIDUniversity Hospital Centre Zagreb, Zagreb, Croatia
    Vedran VelagićORCIDUniversity Hospital Centre Zagreb, Zagreb, Croatia
    Ivo PlanincORCIDUniversity Hospital Centre Zagreb, Zagreb, Croatia
    Maja ČikešORCIDUniversity Hospital Centre Zagreb, Zagreb, Croatia
    Boško SkorićORCIDUniversity Hospital Centre Zagreb, Zagreb, Croatia
    Dora FabijanovićORCIDUniversity Hospital Centre Zagreb, Zagreb, Croatia
    Ana MataićORCIDUniversity Hospital Centre Zagreb, Zagreb, Croatia
    Davorin HercegORCIDUniversity Hospital Centre Zagreb, Zagreb, Croatia
    Hrvoje GašparovićORCIDUniversity Hospital Centre Zagreb, Zagreb, Croatia
    Davor MiličićORCIDUniversity Hospital Centre Zagreb, Zagreb, Croatia
    Daniel LovrićORCIDUniversity Hospital Centre Zagreb, Zagreb, Croatia
    Dejan DošenORCIDUniversity Hospital Centre Zagreb, Zagreb, Croatia

    *Correspondence email: dubravka.sipus@gmail.com

    Full Text

    Introduction: Primary cardiac tumors are a rare entity, accounting for 0.3-0.7% of all cardiac tumors. Only 25% of primary cardiac tumors are malignant, and of these, 75% are sarcomas. Malignant primary cardiac tumors typically affect young patients and have poor prognosis, with a one-year survival rate of around 10% (1, 2).

    Case report: 35-year-old female, without any known previous comorbidities, was admitted with suspected pericarditis. The patient had experienced flu-like symptoms one month prior to admission, and her symptoms of fever (up to 38.1°C) and chest pain with inspiration began a few days before admission. Initial laboratory work-up revealed anemia (hemoglobin 99 g/L), slightly elevated leukocytes (11.3x10^9/L), and elevated CRP (249 ng/L), with normal troponin levels. Chest X-ray revealed an enlarged heart silhouette, suggestive of pericardial effusion, and small bilateral pleural effusions. Initial echocardiography showed a large circumferential pericardial effusion (maximum of 3 cm) and a large mass infiltrating the right ventricle (67x23 mm), along with a smaller mass infiltrating the left ventricle (Figure 1). Given the suspicion of a cardiac tumor, detailed radiological diagnostics were performed. Multislice spiral computed tomography (MSCT) showed a hypovascular tumor infiltrating the right ventricle with characteristics suggestive of sarcoma (Figure 2). One of the branches of the right coronary artery was infiltrated by the tumor. Magnetic resonance imaging revealed a large (7.3x5.3x7.5 cm) expansive, inhomogeneous mass between the pericardial layers, infiltrating the right ventricular wall, and showing radiological characteristics of sarcoma. The pericardium was thickened, with dense pericardial effusion (Figure 3). Multiple intracardiac echocardiography and MSCT-guided biopsies were performed. The final histopathological diagnosis was myxoid pleomorphic liposarcoma, an extremely rare and aggressive subtype of liposarcoma with a predilection for the mediastinum, particularly affecting young adults (2). Staging with positron emission tomography did not show signs of metastatic dissemination. Given the high surgical risk, the multidisciplinary team recommended chemotherapy with Adriamycin and Ifosfamide as the first-line treatment but later cardiectomy and implantation of two HeartMate3 ventricular assist devices as form of total artificial heart was performed.

    FIGURE 1. Echocardiography showing a large mass infiltrating the right ventricular wall.

    FIGURE 2. Multislice spiral computed tomography of the tumor.

    FIGURE 3. Magnetic resonance imaging of the tumor.

    Conclusion: Although primary cardiac tumors are extremely rare, a comprehensive diagnostic approach using multiple imaging modalities is essential in cases of suspicion.

    Literature

    1. 1.
      Leja MJ, Shah DJ, Reardon MJ. Primary cardiac tumors. Tex Heart Inst J. 2011;38(3):261–2.PubMed
    2. 2.
      Dermawan JK, Hwang S, Wexler L, Tap WD, Singer S, Vanderbilt CM, et al. Myxoid pleomorphic liposarcoma is distinguished from other liposarcomas by widespread loss of heterozygosity and significantly worse overall survival: a genomic and clinicopathologic study. Mod Pathol. 2022 November;35(11):1644–55.DOI