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Leiomyosarcoma of Blood Vessel

Last updated Oct. 27, 2018

Approved by: Maulik P. Purohit MD, MPH

Leiomyosarcoma (LMS) is a rare type of connective tissue cancer, accounting for 5-10% of all soft tissue sarcomas (a type of cancer).

What are the other Names for this Condition? (Also known as/Synonyms)

  • Leiomyosarcoma of Vascular Origin
  • LMS of Blood Vessel
  • LMS of Vascular Origin

What is Leiomyosarcoma of Blood Vessel? (Definition/Background Information)

  • Leiomyosarcoma (LMS) is a rare type of connective tissue cancer, accounting for 5-10% of all soft tissue sarcomas (a type of cancer)
  • It was once believed that leiomyosarcomas originated from small, benign, smooth muscle tumors, known as leiomyomas. The occurrence of a malignant tumor from a leiomyoma is now believed to be extremely rare
  • Leiomyosarcoma occurs in the muscles that are not voluntarily controlled, known as smooth muscles. Due to the bounty of smooth muscle throughout the body, any individual is susceptible to LMS, although the elderly are more prone to the condition
  • There are currently no established risk factors, causes, or preventive methods for Leiomyosarcoma of Blood Vessel
  • These cancers appear as painless growth in the blood vessels and the signs and symptoms also depend upon the tumor location. The complications for Leiomyosarcoma of Blood Vessel are dependent upon the stage of the cancer along with the method of treatment
  • Treatment for the condition is mainly through surgery and supplementary treatments. The prognosis of Leiomyosarcoma of Blood Vessel depends on the cancer stage

Who gets Leiomyosarcoma of Blood Vessel? (Age and Sex Distribution)

  • In general, women are two times more likely to have leiomyosarcomas
  • Typically, the condition first appears in elderly adults who are in their 50’s and 60’s
  • People of all races and ethnicities are equally prone to Leiomyosarcoma of Blood Vessel
  • There are no known geographical localizations; this cancer type is found worldwide

What are the Risk Factors for Leiomyosarcoma of Blood Vessel? (Predisposing Factors)

While there are no well-established risk factors for Leiomyosarcoma of Blood Vessel, there are a few leading theories:

  • Certain inherited genetic traits are believed to increase the risk
  • High-dose radiation exposure is believed to increase the risks of leiomyosarcoma
  • Immunocompromised patients infected by Epstein-Barr virus seem to be predisposed to LMS. The reason for this is not understood, yet there seems to be a definite correlation between the viral infection and the arising of multiple, synchronized leiomyosarcomas

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Leiomyosarcoma of Blood Vessel? (Etiology)

  • Currently, there are no known causes for Leiomyosarcoma (LMS) of Blood Vessel
  • As smooth muscles are found widely throughout the body, any individual is susceptible to LMS. However, due to the rarity of the cancer, it is difficult to determine what exactly leads to the formation of Leiomyosarcoma of Blood Vessel
  • Based on clinical data, Leiomyosarcoma of Blood Vessel is more likely to arise from lower pressure veins than high pressure arteries. The cause of this preference is not known at the moment

What are the Signs and Symptoms of Leiomyosarcoma of Blood Vessel?

Signs and symptoms of Leiomyosarcoma of Blood Vessel include:

  • It appears as enlarging, painless tumor masses
  • Tumors in the small blood vessels do not show signs of constriction or compression
  • Tumors in the large blood vessels can produce:
    • Edema or a leaking of fluids out of the vessels into the surrounding areas
    • Numbness from compression of local nerves
    • Abdominal pain
    • Chest discomfort
    • Jaundice from high levels of bilirubin: This is a side effect of a tumor located in the inferior vena cava (one of the major veins of the abdominal cavity)         
    • Shortness of breath

How is Leiomyosarcoma of Blood Vessel Diagnosed?

A diagnosis of Leiomyosarcoma of Blood Vessel may be made by using the following resources:

  • Preliminary examination composed of:
    • Complete physical examination
    • Evaluation  of medical (and family) history        
  • Initial diagnosis that is made by:
    • Plain radiographs of the suspected area to provide quick visualization of ‘potential’ Leiomyosarcoma of Blood Vessel
    • An MRI scan of LMS can provide a view of the tumor’s effect on adjacent structures such as the nerves, bones, and other vascular structures
    • Chest CT scans allow the physicians to check for the presence of any metastasis to the chest and other adjacent regions
    • Angiography can be performed to provide a visualization of the internal space of blood vessels         
  • A biopsy may be necessary to determine, if the tumor present is a leiomyosarcoma, or a different soft tissue sarcoma. In the tissue biopsy procedure, the physician removes a sample of the tissue and sends it to the laboratory for a histopathological examination. The pathologist examines the biopsy under a microscope and arrives at a definitive diagnosis after a thorough evaluation of the clinical and microscopic findings, as well as by correlating the results of special studies on the tissues (if required)

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Leiomyosarcoma of Blood Vessel?

The complications of Leiomyosarcoma of Blood Vessel may occur for a variety of reasons. These may include:

  • The rarity of the condition may cause a delayed diagnosis, leading to metastasis. The metastasis rates for Leiomyosarcoma of Blood Vessel have been reported to be higher than for other leiomyosarcomas
  • Location of the tumor may also adversely impact adjoining/surrounding structures, such as the nerves and joints, leading to discomfort or a loss of feeling
  • Surgery is the most common method of treatment, though it may not be always possible to use surgical procedures. Location and stage of Leiomyosarcoma of Blood Vessel may prohibit surgery, due to operational risks or difficulty of access
  • Recurrence of LMS after treatment

How is Leiomyosarcoma of Blood Vessel Treated?

The treatment of Leiomyosarcomas of Blood Vessel differs from one individual to another. It depends on the tumor stage, tumor size, location, histological grade, and presence or absence of metastasis. Tumors are staged from I to IV, with IV being the most progressed, which has spread throughout the body.

  • Surgery is the most common treatment for leiomyosarcoma. Surgery aims to remove the tumor along with a portion of the tissue surrounding it. The surrounding tissue is removed to increase the likelihood of complete tumor removal, since some tumor cells may have drifted locally
  • Other than surgery, LMS provides a treatment challenge due to the observed resistance to chemotherapy and radiation therapy
  • Currently, clinical trials are being held to see if secondary treatment of chemotherapy and radiotherapy are capable of aiding tumor growth suppression

In addition to traditional adjuvant therapies, the following techniques are currently being investigated:

  • Immunotherapy aims to stimulate the patient’s immune system to recognize and destroy the cancer cells. It includes:
    • Antigen vaccines
    • DNA vaccines
    • Viral therapy
    • Gene therapy          

Once treatment is complete, it is recommended that the patient schedule regular check-ups, based on recommendations of the specialist(s) treating them.

How can Leiomyosarcoma of Blood Vessel be Prevented?

There are currently no known methods of preventing Leiomyosarcoma of Blood Vessel.

What is the Prognosis of Leiomyosarcoma of Blood Vessel? (Outcomes/Resolutions)

Prognosis for Leiomyosarcoma of Blood Vessel depends on the stage of the leiomyosarcoma upon diagnosis.

  • Many patients with metastatic or locally advanced tumors are not recommended for surgery and may be referred for clinical trials for experimental treatment options
  • Among the individuals that received surgery, a report stated that less than 50% with complete macroscopic excision did not experience any recurrence of leiomyosarcoma. The risk of fatalities from disease progression was also avoided in such individuals who underwent surgical treatment procedures

Additional and Relevant Useful Information for Leiomyosarcoma of Blood Vessel:

  • Although leiomyosarcomas are rare cancer forms, there are many online discussion groups, local groups, and sarcoma centers available to provide help and support
  • The most common type of leiomyosarcoma that accounts for 50% of the cases occurs in the smooth muscles of the retroperitoneal area. The retroperitoneal area is found in front of the spine, behind the membrane lining the abdominal cavity, known as the peritoneum

What are some Useful Resources for Additional Information?

References and Information Sources used for the Article:

Helpful Peer-Reviewed Medical Articles:

Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: July 26, 2015
Last updated: Oct. 27, 2018