Epstein-Barr Virus-Associated Smooth Muscle Tumors

Epstein-Barr Virus-Associated Smooth Muscle Tumors

Article
Diseases & Conditions
Cancer & Benign Tumors
+2
Contributed byMaulik P. Purohit MD MPHSep 10, 2018

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

  • Epstein-Barr Virus Infection-Associated Smooth Muscle Tumors
  • Smooth Muscle Tumors caused by EBV
  • Smooth Muscle Tumors caused by Epstein-Barr Virus

What are EBV-Associated Smooth Muscle Tumors? (Definition/Background Information)

  • Epstein-Barr Virus-Associated Smooth Muscle Tumors are rare tumors that originate from the smooth muscles and are associated with Epstein-Barr virus (EBV)
  • They usually occur in immunocompromised individuals and can arise anywhere in the body. There may be multiple tumors too; but, this does not indicate that the tumors have metastasized (spread to various body regions)
  • The treatment of EBV-Associated Smooth Muscle Tumors includes treating the health condition causing the tumor formation and surgical excision of the tumors
  • The prognosis of EBV-Associated Smooth Muscle Tumors depends upon factors such as health status of the individual and the underlying condition causing the tumors 

Who gets EBV-Associated Smooth Muscle Tumors? (Age and Sex Distribution)

  • Epstein-Barr Virus-Associated Smooth Muscle Tumors are typically observed in individuals (of any age) with poor immune system of either sex (both male and female genders are affected)
  • However, there is a slight male predominance (3:2 male-female ratio)
  • There is no geographical, racial, or ethnic preference noticed

What are the Risk Factors for EBV-Associated Smooth Muscle Tumors? (Predisposing Factors)

The risk factors for Epstein-Barr Virus-Associated Smooth Muscle Tumors include:

  • HIV infection and AIDS
  • Immunosuppression after organ transplant, such as liver, kidney, heart, and lungs
  • Any health condition causing a weak immune system for prolonged periods
  • Cancer treatment using chemotherapy drugs

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 EBV-Associated Smooth Muscle Tumors? (Etiology)

  • The exact cause of Epstein-Barr Virus-Associated Smooth Muscle Tumors development is unknown, but it is related to Epstein-Barr virus infection
  • They are known to occur in individuals with poor immune system that occurs from various health conditions 

What are the Signs and Symptoms of EBV-Associated Smooth Muscle Tumors?

The signs and symptoms of Epstein-Barr Virus-Associated Smooth Muscle Tumors include:

  • These tumors can appear anywhere on the body; some appear after nearly 3 years after an organ transplant
  • Most common tumor locations include the soft tissues of the lungs and liver
  • Multiple tumors may form in different body parts at the same time (called multifocal tumors). The presence of such tumors should not be considered as a metastasis
  • In some, these tumors behave in a benign fashion; while in others, they may be aggressive
  • Rarely, the presence of such tumors may be the first symptoms of AIDS
  • Some smooth muscle tumors may not present any significant signs and symptoms

How are EBV-Associated Smooth Muscle Tumors Diagnosed?

The diagnosis of Epstein-Barr Virus-Associated Smooth Muscle Tumors may involve:

  • Complete physical examination with thorough evaluation of medical history
  • CT, MRI scans of the affected regions
  • Tissue biopsy: A tissue biopsy is performed and sent to a laboratory for a pathological examination, who examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis
  • Differential diagnosis, to eliminate other tumor types is considered, before arriving at a definitive diagnosis

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 EBV-Associated Smooth Muscle Tumors?

The possible complications of Epstein-Barr Virus-Associated Smooth Muscle Tumors include:

  • Cosmetic issues causing stress in some individuals
  • Some deep-seated tumors may affect the function of the organs where they develop
  • Injury to blood vessels, tissues, nerves, and organs during surgery to remove EBV-Associated Smooth Muscle Tumors
  • Recurrence of the tumor after surgery, especially in cases where it is incompletely removed

How are EBV-Associated Smooth Muscle Tumors Treated?

Following are the treatment methods adopted for Epstein-Barr Virus-Associated Smooth Muscle Tumors:

  • Asymptomatic tumors may not require any treatment and may be placed under observation; a ‘wait and watch’ approach may be adopted by the healthcare provider
  • Treatment of the underlying condition: Treating the underlying immunosuppression may not bring improvement in the tumor state. Some studies have shown that improving the immune status may help in treatment
  • Chronic steroidal therapy to decrease inflammation, especially in the throat and tonsils
  • A simple surgical excision and removal of the entire tumor is normally sufficient treatment
  • Follow-up care with regular screening and check-ups are important

How can EBV-Associated Smooth Muscle Tumors be Prevented?

Current medical research has not established a way of preventing Epstein-Barr Virus-Associated Smooth Muscle Tumors.

What is the Prognosis of EBV-Associated Smooth Muscle Tumor? (Outcomes/Resolutions)

  • The prognosis of Epstein-Barr Virus-Associated Smooth Muscle Tumors depends upon the underlying condition of the patient, such as the severity of AIDS, severity of immunosuppression, etc.
  • These tumors by themselves do not cause any mortality; mortality usually occurs from the primary health condition causing poor immunity
  • Periodic follow-up check-ups with screening may be required for a long time period

Additional and Relevant Useful Information for EBV-Associated Smooth Muscle Tumors:

The following DoveMed website link is a useful resource for additional information:

https://www.dovemed.com/diseases-conditions/cancer/

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On the Article

Maulik P. Purohit MD MPH picture
Approved by

Maulik P. Purohit MD MPH

Assistant Medical Director, Medical Editorial Board, DoveMed Team

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