Vascular Leiomyosarcoma

If you or a loved one has been diagnosed with vascular leiomyosarcoma, you may be scared or concerned and looking for answers.

Consider this an introductory guide to vascular leiomyosarcoma designed to help you understand the basics of the disease so you can ask your doctor informed questions about your prognosis and treatment.

What Is Vascular Leiomyosarcoma?

According to the Liddy Shriver Sarcoma Initiative, it is relatively uncommon for leiomyosarcoma, a rare and aggressive form of connective tissue cancer, to appear directly in your major blood vessels. When it does, however, it is referred to as vascular leiomyosarcoma.

Vascular leiomyosarcoma is so rare, in fact, that there are only a few hundred published reports of its existence. It is believed that vascular leiomyosarcoma mostly appears in low pressure systems like large veins (particularly the inferior vena cava) or the pulmonary artery. Relatively few cases have been discovered in the peripheral arteries.

What Are the Symptoms of Vascular Leiomyosarcoma?

According to the Liddy Shriver Sarcoma Initiative, the symptoms of vascular leiomyosarcoma are entirely dependent on where in your body it the vascular leiomyosarcoma has developed.

If vascular leiomyosarcoma has developed in the inferior vena cava in the supra-hepatic segment, you might develop what is known as Budd-Chiari syndrome.

Budd-Chiari syndrome is accompanied by symptoms such as hepatic segment and jaundice as well as ascites (an accumulation of fluid in the peritoneal cavity that causes abdominal swelling). Please note that abdominal swelling can be a symptom of almost any kind of leiomyosarcoma.

Unfortunately, these types of tumors are typically not surgically resectable, which severely limits treatment options.

Tumors that arise in the inferior vena cava (below the liver) are often accompanied by lower extremity edema (an excess of watery fluid collecting in the cavities or tissues of the body) as well as vague abdominal pain. Generally, the symptoms associated with this form of vascular leiomyosarcoma are defined by the anatomic location of the lesion, as well as by the local vascular physiology and drainage patterns.

A specific type of leiomyosarcoma called arterial leiomyosarcoma can affect the pulmonary artery. These patients often complain of dyspnea and chest discomfort, which are related to obstruction of the arteries. The symptoms here are largely related to the vascular distribution of the affected artery, as well as the existence or lack of collateral blood flow.

Treatment Options for Vascular Leiomyosarcoma

As with other forms of leiomyosarcoma, vascular leiomyosarcoma is typically treated with surgery, as chemotherapy and radiation are of limited use with this particular type of cancer.

However, it is not uncommon for doctors to recommend a course of chemotherapy or radiation before and/or after surgery in order to reduce the size of tumors or to prevent local recurrence.

Most vascular leiomyosarcoma patients undergo complete resection of their tumor, either through ligation, primary or patch repair or expanded polytetrafluoroethylene tube grafting.

A small percentage of patients report severe edema after their operation.

After surgery for vascular leiomyosarcoma, about a third of patients experience local recurrence, and close to half have distant recurrence (another appearance of vascular leiomyosarcoma away from the first location of the disease).

Sadly none of the patients who have had incomplete resections of vascular leiomyosarcoma survived past three years after their surgery.