National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Intravenous leiomyomatosis


Intravenous leiomyomatosis (IVL) is a benign smooth muscle tumor of the uterus that grows within the veins but does not invade the surrounding tissue. IVL usually starts in the veins of the uterus and can extend into the inferior vena cava and ultimately into the right side of the heart, resulting in death The abnormal smooth muscle cells that cause IVL express estrogen and progesterone receptors and tumor growth thus appears to respond to these hormones. Although this is a benign condition, many affected individuals require surgery to remove the excess tissue in the uterus and heart. The exact cause of IVL remains unknown. IVL is rare, with only about 200 cases reported in the medical literature.[1][2][3]
Last updated: 11/1/2013
IVL most often does not cause detectable signs or symptoms. In fact, they may be found by chance during surgery. When symptoms do arise, they can include abnormal uterine bleeding, lower abdominal tenderness, and venous thrombosis. When IVL in the uterus is exposed to venous blood that flows to the heart, it usually grows slowly and may reach the heart undetected. When IVL reaches the heart, it can result in pulmonary embolisms, cardiac failure, fainting, and in some cases, sudden death. Most people do not experience symptoms until the IVL reaches the heart.[2][3][4]
Last updated: 11/1/2013
The mainstay of treatment for IVL is surgery to remove the tumor and its spread throughout the body. The use of anti-estrogen therapy, such as tamoxifen, has also been suggested. Surgery requires the complete removal of the tumor, since incomplete removal may result in a recurrence and hence further surgery or even death. Many affected individuals undergo a hysterectomy; bilateral oophorectomy is also suggested because these tumors are estrogen dependent. Part of a tumor left inside the pelvic veins at the time of hysterectomy can extend towards the right side of the heart, leading to obstruction and other adverse events later in life. The median time between hysterectomy to the diagnosis of IVL with cardiac involvement is 4 years. Once there is cardiac involvement, a patient may require open-heart surgery to remove the IVL from the affected areas.[2][3]
Last updated: 11/1/2013

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • ClinicalTrials.gov lists trials that are related to Intravenous leiomyomatosis. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Intravenous leiomyomatosis. Click on the link to view a sample search on this topic.

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  • I'm hoping to obtain information about intravenous leiomyomatosis.  I have been diagnosed with this disease and had two open-heart surgeries in addition to a hysterectomy. See answer


  1. Smooth muscle: Intravenous leiomyomatosis. Atlas of Genetics and Cytogenetics in Oncology and Haematology. March 2011; http://atlasgeneticsoncology.org/Tumors/IntravLeiomyomID5158.html. Accessed 11/1/2013.
  2. Wu CK et al.. Intravenous leiomyomatosis with intracardiac extension. Internal Medicine. 2009; 48:997-1001. https://www.jstage.jst.go.jp/article/internalmedicine/48/12/48_12_997/_article. Accessed 11/1/2013.
  3. Liu B, Liu C, Guan H, Li Y, Song X, Shen K, Miao Q. leiomyomatosis with inferior vena cava and heart extension. J Vasc Surg. 2009; 50(4):897-902. http://www.ncbi.nlm.nih.gov/pubmed/19560308. Accessed 11/1/2013.
  4. Fukuyama A. et al. A case of uterine leiomyoma with intravenous leiomyomatosis--Histological investigation of the pathological condition. Pathol Oncol Res. 2011; 17:171-174. http://www.springerlink.com/content/d7v52w50427xg730/fulltext.pdf. Accessed 11/1/2013.