Abstract

Case Report

Intravenous Leiomyomatosis of the Uterus with Intracardiac Extension

Tomas Reyes-del Castillo*, Minerva I Hernandez-Rejon, Jose L Ruiz-Pier, Mario Peñaloza-Guadarrama, Carlos E Merinos-Avila, Cristina Juarez-Cabrera, Pedro A del Valle-Maldonado, Sofia Ley-Tapia and Valentín Gonzalez-Flores

Published: 22 July, 2025 | Volume 9 - Issue 1 | Pages: 003-007

Background: Intravascular Leiomyomatosis (IVL) is an often misdiagnosed rare benign mesenchymal tumor characterized by the presence of vascular extension and invasion of smooth muscle cells in a serpiginous-like pattern, first originating in uterine smooth muscle cells. Its growth pattern can involve both ovarian veins, the inferior vena cava, and even reach the right atrium/ventricle in 45% of the cases. The incidence has been reported to be 0.25 to 0.40% of patients with uterine leiomyoma, with about 300 cases reported in the literature. Also, since the tumor is hormone-dependent, most affected individuals are premenopausal women in middle age. Optimal treatment for IVL is complete surgical removal with hysterectomy and oophorectomy, independent of stage. The most frequent perioperative complications are hemorrhage due to tumoral hypervascularization, embolism, and the usual laparotomy complications. We present the case of a 51-year-old female with IVL stage 3 with complete single-stage surgical resolution.

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References

  1. Mathey MP, Duc C, Huber D. Intravenous leiomyomatosis: Case series and review of the literature. Int J Surg Case Rep. 2021;85:106257. Available from: http://dx.doi.org/10.1016/j.ijscr.2021.106257
  2. Qu Y, Chen L, Guo S, Liu Y, Wu H. Genetic liability to multiple factors and uterine leiomyoma risk: A Mendelian randomization study. Front Endocrinol (Lausanne). 2023;14:1133260. Available from: http://dx.doi.org/10.3389/fendo.2023.1133260
  3. Styer AK, Rueda BR. The epidemiology and genetics of uterine leiomyoma. Best Pract Res Clin Obstet Gynaecol . 2016;34:3–12. Available from: http://dx.doi.org/10.1016/j.bpobgyn.2015.11.018
  4. Ordulu Z, Nucci MR, Dal Cin P, Hollowell ML, Otis CN, Hornick JL, et al. Intravenous leiomyomatosis: An unusual intermediate between benign and malignant uterine smooth muscle tumors. Mod Pathol. 2016;29(5):500–10. Available from: https://doi.org/10.1038/modpathol.2016.36
  5. Shi T, Shkrum MJ. A case report of sudden death from intracardiac leiomyomatosis. Am J Forensic Med Pathol. 2018;39(2):119–22. Available from: http://dx.doi.org/10.1097/PAF.0000000000000377
  6. Ma G, Miao Q, Liu X, Zhang C, Liu J, Zheng Y, et al. Different surgical strategies of patients with intravenous leiomyomatosis. Medicine (Baltimore). 2016;95(37):e4902. Available from: http://dx.doi.org/10.1097/md.0000000000004902
  7. Low G, Rouget AC, Crawley C. Case 188: Intravenous leiomyomatosis with intracaval and intracardiac involvement. Radiology. 2012;265(3):971–5. Available from: https://doi.org/10.1148/radiol.12111246
  8. Wang MX, Menias CO, Elsherif SB, Segaran N, Ganeshan D. Current update on IVC leiomyosarcoma. Abdom Radiol (NY). 2021;46:5284–96. Available from: https://doi.org/10.1007/s00261-021-03256-9
  9. Ordulu Z, Chai H, Peng G, McDonald AG, De Nictolis M, Garcia-Fernandez E, et al. Molecular and clinicopathologic characterization of intravenous leiomyomatosis. Mod Pathol. 2020;33(9):1844–60. Available from: http://dx.doi.org/10.1038/s41379-020-0546-8
  10. Law YJ, Hines EM, Frahm-Jensen G. Images in vascular medicine: Asymptomatic stage IV intravascular leiomyomatosis – A diagnostic difficulty. Vasc Med. 2024;29(2):223–4. Available from: https://doi.org/10.1177/1358863x231211330
  11. Bender LC, Mitsumori LM, Lloyd KA, Stambaugh LE 3rd. AIRP best cases in radiologic-pathologic correlation: Intravenous leiomyomatosis. Radiographics. 2011;31(4):1053–8. Available from: http://dx.doi.org/10.1148/rg.314115013
  12. Kawakami S, Sagoh T, Kumada H, Kimoto T, Togashi K, Nishimura K, et al. Intravenous leiomyomatosis of uterus: MR appearance. J Comput Assist Tomogr. 1991;15(4):686–9. Available from: http://dx.doi.org/10.1097/00004728-199107000-00030
  13. Zheng T, Huang C, Xia Q, He W, Liu Y, Ye H. Intravenous leiomyomatosis in the inferior vena cava and right atrium with pulmonary benign metastasizing leiomyoma secondary to a pelvic arteriovenous fistula: A case report and literature review. Cardiovasc Pathol. 2024;73:107685. Available from: https://www.sciencedirect.com/science/article/pii/S1054880724000814
  14. Fasih N, Prasad Shanbhogue AK, Macdonald DB, Fraser-Hill MA, Papadatos D, Kielar AZ, et al. Leiomyomas beyond the uterus: Unusual locations, rare manifestations. Radiographics. 2008;28(7):1931–48. Available from: http://dx.doi.org/10.1148/rg.287085095
  15. Yin S, Shi P, Han J, Li H, Ren A, Ma L, et al. Pathological and molecular insights into intravenous leiomyomatosis: An integrative multi-omics study. J Transl Med. 2025;23(1):229. Available from: http://dx.doi.org/10.1186/s12967-024-05919-9

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