Inflammatory Myofibroblastic Tumor of Lymph Node

Inflammatory Myofibroblastic Tumor of Lymph Node

Article
Diseases & Conditions
Cancer & Benign Tumors
+2
Contributed byKrish Tangella MD, MBAOct 12, 2018

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

  • Inflammatory Fibrosarcoma of Lymph Node
  • Inflammatory Pseudotumor of Lymph Node
  • Plasma Cell Granuloma of Lymph Node

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

  • Inflammatory Myofibroblastic Tumor of Lymph Node is a very rare, benign tumor involving the lymph nodes of the body. Usually only one lymph node (or a group of lymph nodes in the region) is affected by the tumor
  • The tumor releases biochemicals which causes enlargement of other lymph nodes and systemic signs and symptoms. This may lead to enlarged lymph nodes in the body, due to the lymphatic system being affected
  • 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 Inflammatory Myofibroblastic Tumor of Lymph Node is generally unknown, though the involvement of genetic mutations is observed in some cases. There are also no well-established risk factors for this tumor type
  • The signs and symptoms may include enlarged lymph nodes that may be painless and low-grade fever. Complications, such as recurrence of IMT on incomplete removal, is known to occur
  • The mainstay of treatment is a surgical excision that can be curative. The prognosis of Inflammatory Myofibroblastic Tumor of Lymph Node is generally good on tumor removal, but some tumors are known to recur after a certain period of time

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

  • Inflammatory Myofibroblastic Tumor of Lymph Node is typically seen in adults; children are not commonly affected by this tumor type
  • Very few cases of IMT of Lymph Node 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 Lymph Node? (Predisposing Factors)

  • Presently, the specific risk factors for Inflammatory Myofibroblastic Tumor of Lymph Node 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 Lymph Node? (Etiology)

The cause of development of Inflammatory Myofibroblastic Tumor of Lymph Node 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 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 Lymph Node?

The signs and symptoms depend on the size of the tumor; small tumors usually do not cause any symptoms. The specific signs and symptoms of Inflammatory Myofibroblastic Tumor of Lymph Node may include:

  • Lymphadenopathy or enlarged lymph nodes that may involve many lymph nodes at any location in the body
  • The swollen lymph nodes are generally painless
  • Breathing difficulties
  • Swelling of hands and feet
  • Thrombosis (blood clot in a blood vessel)
  • Pancytopenia, which includes the following 3 conditions, may be present:
    • Anemia causing pale skin, due to lower levels of red blood cells
    • Thrombocytopenia due to decreased platelets, which can cause bleeding disorders and easy bruising
    • Leucopenia or reduced white blood cells
  • Fever, loss of appetite

The tumor has been generally known to involve the inguinal (groin), axillary (armpit) and cervical (neck) lymph nodes.

How is Inflammatory Myofibroblastic Tumor of Lymph Node Diagnosed?

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

  • Complete evaluation of family (medical) history, along with a thorough physical examination
  • CT or MRI scan of the affected region
  • Lymph node biopsy: A lymph node biopsy is performed and sent to a laboratory for a pathological examination. The 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
  • The lymph node biopsy may be performed through any of the following procedures:
    • Fine needle aspiration biopsy (FNAB): A device called a cannula is used to extract tissue or fluid from the lymph nodes
    • Lymph node core biopsy of the enlarged lymph node
    • Lymph node open biopsy of the enlarged lymph node

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 Lymph Node?

Some potential complications of Inflammatory Myofibroblastic Tumor of Lymph Node include:

  • They can mimic cancer and cause considerable emotional stress in the affected individual
  • In some individuals, an enlarged liver and/or spleen may be observed
  • Recurrence of the tumor after treatment, especially due to partial removal of IMT

Even though, the tumors are considered to be low-grade, they are not known to undergo malignant transformations.

How is Inflammatory Myofibroblastic Tumor of Lymph Node Treated?

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

  • Surgical removal of the entire tumor is the preferred method of treatment
  • Occasionally, some tumors are known to disappear over time, without any treatment
  • In young children, if the tumors cannot be surgically removed, then corticosteroid administration is found to be beneficial
  • Chemotherapy may help if the condition recurs, or if there is a local invasion
  • Observation and periodic checkups to monitor the condition is recommended

How can Inflammatory Myofibroblastic Tumor of Lymph Node be Prevented?

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

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

  • An early diagnosis and prompt treatment of Inflammatory Myofibroblastic Tumor of Lymph Node generally yields better outcomes than a late diagnosis and delayed treatment
  • The prognosis on timely surgical removal of the tumor is generally good. On a complete excision and removal, IMT of Lymph Node is generally not known to recur
  • Some tumors are known to spontaneously regress and disappear. But, some tumors are known to recur following their incomplete removal

Additional and Relevant Useful Information for Inflammatory Myofibroblastic Tumor of Lymph Node:

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

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

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Krish Tangella MD, MBA picture
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Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team

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