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      A complex clinical case of intracardiac leiomyomatosis

      case-report

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          Abstract

          Background

          Intracardiac leiomyomatosis (ICL) is an uncommon condition characterized by the proliferation of intravascular tissue within the veins, leading to the development of tumor emboli. This can pose a significant threat to life when the tumor invades cardiac structures. The diagnostic process for this condition is complex and presents considerable challenges.

          Case presentation

          We report a case of a 38-year-old female patient whose pulmonary artery computed tomography (CT) revealed low density structure in the branches of the pulmonary artery. Echocardiography revealed a mobile tumor within the right heart chambers and pulmonary trunks as well as characteristic thickening of the ventricular septum consistent with hypertrophic cardiomyopathy (HCM). Magnetic resonance imaging (MRI) revealed a mass in the right anterior uterine wall, extending to the inferior vena cava (IVC) and right iliac vein. Post-surgery histopathological analysis confirmed a diagnosis of intravenous leiomyomatosis (IVL).

          Conclusions

          When IVL affects the heart, echocardiography is the best diagnostic tool for detecting the disease. CT and MRI are essential in identifying the location and extent of the tumor, as well as in evaluating prognosis.

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          Most cited references16

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          Different surgical strategies of patients with intravenous leiomyomatosis

          Guotao Ma, Qi Miao, Xingrong Liu … (2016)
          Abstract Intravenous leiomyomatosis (IVL) is a rare benign tumor. The study aimed to assess outcomes of patients treated surgically for IVL. Between November 2002 and January 2015, 76 patients were treated for IVL. The stage of IVL was evaluated preoperatively by echocardiography and enhanced computerized tomography (CT) scan, and graded into 4 stages according to intravascular tumor progression. We recorded age, lower limb edema before surgery, surgical parameters, and hospitalization expenses. Patients were followed up every 6 months and tumor recurrence was assessed by CT and ultrasound. Patients were followed up for a mean of 4.5 ± 2.5 years (range 1–13 years) and there was no operative, hospital, or long-term mortality or were lost to follow-up. The rate of lower extremity edema, amount of blood loss, postoperative transfusion, length of intensive care unit (ICU) stay, postoperative hospitalization, and hospitalization expenses differed significantly between patients at different presurgery stages. Tumors recurred in 4 of 7 patients with stage I IVL that opted for surgery that preserved the ovaries and uterus. No recurrence was observed in patients graded stage II or more, in all of which the uterus and ovaries were removed. Recurrence was observed in only 4 of 76 cases of IVL, all of whom opted for surgery that spared the ovaries and uterus. Different surgical strategies should be decided based on the staging to completely remove the tumor and ensure the safety of patients. Removal of both ovaries is necessary for inhibiting tumor growth and avoiding recurrence.
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            [Introduction of WHO classification of tumours of female reproductive organs, fourth edition].

            Zhaohui Lu, Jie Chen (2014)
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              Mesenchymal tumors of the uterus. V. Intravenous leiomyomatosis. A clinical and pathologic study of 14 cases.

              Michael H. Norris, T H Parmley (1975)
              Fourteen examples of intravenous leiomyomatosis in the FIP files were studied. Growth beyond the uterus occurred in 10 of the 14 examples, largely in vessels in the broad ligament and in uterine and iliac veins. Metastasis to the heart occurred in 1 example. The study provides evidence that is consistent with both theories of origin of intravenous leiomyomatosis; namely, that it may arise either from the wall of veins within the myometrium or be a result of unusually extensive vascular invasion from a leiomyoma of the myometrium. Intravenous leiomyomatosis should be distinguished from a leiomyoma with vascular invasion by arbitrarily limiting the designation of leiomyoma with vascular invasion to neoplasms in which the vascular growth is a microscopic finding and confined within the leiomyoma. Criteria are given for distinguishing intravenous leiomyomatosis from endometrial stromal sarcoma, leiomyosarcoma, leiomyoma with vascular invasion, and leiomyomatosis peritonealis disseminata.
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                Author and article information

                Contributors
                Xiuzhang Lv: lxz_echo@163.com
                Journal
                Journal ID (nlm-ta): BMC Cardiovasc Disord
                Journal ID (iso-abbrev): BMC Cardiovasc Disord
                Title: BMC Cardiovascular Disorders
                Publisher: BioMed Central (London )
                ISSN (Electronic): 1471-2261
                Publication date (Electronic): 17 February 2025
                Publication date PMC-release: 17 February 2025
                Publication date Collection: 2025
                Volume: 25
                Electronic Location Identifier: 105
                Affiliations
                [1 ]Department of Ultrasound, Beijing Chao Yang Hospital, Capital Medical University, ( https://ror.org/013xs5b60) No. 8 Gongren Tiyuchang Nanlu, Chaoyang District, Beijing, 100020 China
                [2 ]Department of Radiology, Beijing Chao Yang Hospital, Capital Medical University, ( https://ror.org/013xs5b60) No. 8 Gongren Tiyuchang Nanlu, Chaoyang District, Beijing, 100020 China
                Article
                Publisher ID: 4561
                DOI: 10.1186/s12872-025-04561-7
                PMC ID: 11831790
                PubMed ID: 39962397
                SO-VID: f6db7cc8-75ab-447b-b829-59aa0f18162c
                Copyright © © The Author(s) 2025
                License:

                Open Access This article is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License, which permits any non-commercial use, sharing, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if you modified the licensed material. You do not have permission under this licence to share adapted material derived from this article or parts of it. The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by-nc-nd/4.0/.

                History
                Date received : 11 September 2024
                Date accepted : 10 February 2025
                Categories
                Subject: Case Report
                Custom metadata
                issue-copyright-statement © BioMed Central Ltd., part of Springer Nature 2025

                ScienceOpen disciplines: Cardiovascular Medicine
                Keywords: intracardiac leiomyomatosis,echocardiography,hypertrophic cardiomyopathy
                Data availability:
                ScienceOpen disciplines: Cardiovascular Medicine
                Keywords: intracardiac leiomyomatosis, echocardiography, hypertrophic cardiomyopathy

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