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

• Cardiac Inflammatory Fibrosarcoma
• Cardiac Inflammatory Pseudotumor
• Plasma Cell Granuloma of Heart

What is Inflammatory Myofibroblastic Tumor of Heart? (Definition/Background Information)

• Inflammatory Myofibroblastic Tumor of Heart is a very rare, mostly benign tumor of the heart. They can grow to large sizes and generally occur in younger populations
• Inflammatory myofibroblastic tumor (IMT) is generally considered as a benign tumor with aggressive behavior (low-grade tumor), which can occur anywhere in the body
• The cause of Cardiac Inflammatory Myofibroblastic Tumor is generally unknown. There are also no well-established risk factors for this tumor type
• The tumor may be located at any location in the heart. The signs and symptoms are generally non-specific, but may include breathing difficulties, chest pain, and dizziness. Complications, such as sudden cardiac death, is known to occur
• The mainstay of treatment is a surgical excision that can be curative. The prognosis is generally good on tumor removal, but some tumors are known to recur and rarely they can even metastasize

Who gets Inflammatory Myofibroblastic Tumor of Heart? (Age and Sex Distribution)

• Inflammatory Myofibroblastic Tumor of Heart is typically seen in young adults and children, including in newborns and infants
• Very few cases (less than 100) have been recorded in the medical literature
• Both males and females are affected and no gender preference is seen
• All races and ethnic groups are at risk for the condition

Note: Most inflammatory myofibroblastic tumors are found in the lung, which is the most common site of the tumor. In such cases, these tumors are frequently diagnosed in middle-aged adults. However, in contrast, the average age of presentation for extrapulmonary IMT (IMT outside the lung) is around 10 years.

What are the Risk Factors for Inflammatory Myofibroblastic Tumor of Heart? (Predisposing Factors)

• Presently, the specific risk factors for Cardiac Inflammatory Myofibroblastic Tumor are unknown or unidentified

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 Inflammatory Myofibroblastic Tumor of Heart? (Etiology)

The cause of development of Inflammatory Myofibroblastic Tumor of Heart is generally unknown.

• Some research scientists believe that the cause of the condition is mostly due to genetic mutations, which results in tumor formation. In over 40% of the tumors, ALK gene mutation has been observed
• Some believe that the inflammatory myofibroblastic tumor is the result of an inflammatory reactive process and that it is not a true tumor
• It is also believed by some researchers that the tumor may arise due to viral infections caused by human herpes virus 8 (HHV8) or Epstein-Barr virus (EBV)

What are the Sign and Symptoms of Inflammatory Myofibroblastic Tumor of Heart?

Specific signs and symptoms of Inflammatory Myofibroblastic Tumor of Heart are generally not observed. The presentations are based upon the site of tumor in the heart and may include:

• Chest pain
• Difficulty breathing, palpitations
• Fainting
• Large tumors can cause obstruction of blood flow in the heart

The tumor is mostly seen to form in the right ventricle outflow tract, but can be found anywhere in the heart. Large tumor sizes (averaging 8 cm) have been observed.

How is Inflammatory Myofibroblastic Tumor of Heart Diagnosed?

A diagnosis of Inflammatory Myofibroblastic Tumor of Heart may be undertaken using the following tests and exams:

• Complete evaluation of family (medical) history, along with a thorough physical examination; including examination of the heart (with special emphasis to signs such as abnormal heart sounds)
• CT or MRI scan of the heart
• Electrocardiogram (EKG): It is used to measure the electrical activity of the heart, in case of arrhythmias
• Echocardiography: This procedure uses sound waves to create a motion picture of the heart movement
  • It can help show the size and shape of the heart and how well the chambers and valves are working
  • It can also help visualize the tumors, especially the large tumors; 1 mm and lesser-sized tumors may not be visible though
• Doppler ultrasound: Sound waves are used to measure the speed and direction of blood flow
• Tissue biopsy of the tumor:
  • A tissue biopsy of the nodule is performed and sent to a laboratory for a pathological examination. A pathologist 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. Examination of the biopsy under a microscope by a pathologist is considered to be gold standard in arriving at a conclusive diagnosis
  • Biopsy specimens are studied initially using Hematoxylin and Eosin staining. The pathologist then decides on additional studies depending on the clinical situation
  • Sometimes, the pathologist may perform special studies, which may include immunohistochemical stains, molecular testing, and very rarely, electron microscopic studies to assist in the diagnosis

Note: Inflammatory Myofibroblastic Tumors are very rare. Due to this, it typically causes diagnostic challenges to the pathologist while trying to establish an accurate 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 Inflammatory Myofibroblastic Tumor of Heart?

Some potential complications of Inflammatory Myofibroblastic Tumor of Heart include:

• Recurrence of the tumor after treatment, which is seen in about 25-30% of the cases
• Heart dysfunction due to large tumors
• Heart attack that can be fatal
• Sudden death due to the tumor is known to occur

IMTs are considered to be low-grade tumors and in a majority of individuals, they do not show any malignancy (95% or more cases). However, in about 5% of the individuals, the tumor can undergo a malignant transformation (called Malignant Inflammatory Myofibroblastic Tumor). In such cases, metastasis has been observed that may even result in fatal outcomes.

How is Inflammatory Myofibroblastic Tumor of Heart Treated?

Inflammatory Myofibroblastic Tumor of Heart may be treated through the following measures:

• Surgical removal of the entire tumor is the preferred treatment method
• In young children, if the tumors cannot be surgically removed, then corticosteroid administration is found to be beneficial
• Occasionally, some tumors are known to disappear over time, without any treatment
• Chemotherapy may help, if the condition recurs , if there is a local invasion, or a distant metastasis is noted (in very rare cases)

Observation and periodic checkups to monitor the condition is recommended following treatment.

How can Inflammatory Myofibroblastic Tumor of Heart be Prevented?

Presently, there are no specific methods or guidelines to prevent Inflammatory Myofibroblastic Tumor of Heart.

What is the Prognosis of Inflammatory Myofibroblastic Tumor of Heart? (Outcomes/Resolutions)

• An early diagnosis and prompt treatment of Inflammatory Myofibroblastic Tumor of Heart generally yields better outcomes than a late diagnosis and delayed treatment
• The prognosis on timely surgical removal of the tumor is generally good. Some tumors are known to spontaneously regress and disappear
• Some IMTs are known to be locally aggressive and some can recur following treatment. Some tumors are even known to metastasize (occasionally)

Additional and Relevant Useful Information for Inflammatory Myofibroblastic Tumor of Heart:

The following article link will help you understand other cancers and benign tumors:

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

The following article link will help you understand other heart conditions:

http://www.dovemed.com/healthy-living/heart-center/