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

• Inflammatory Fibrosarcoma of Small Bowel
• Inflammatory Fibrosarcoma of Small Intestine
• Plasma Cell Granuloma of Small Bowel

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

• Inflammatory Myofibroblastic Tumor of Small Intestine is a very rare, mostly benign tumor of the small bowel (small intestine). 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 Inflammatory Myofibroblastic Tumor of Small Intestine is generally unknown, though it may be associated with genetic mutations. There are also no well-established risk factors for this tumor type
• The signs and symptoms are generally non-specific, but may include abdominal pain, nausea, and general malaise. Complications, such as tumor recurrence on partial removal, is known to take place
• The mainstay of treatment is a surgical excision that can be curative. The prognosis of Inflammatory Myofibroblastic Tumor of Small Intestine is generally good on tumor removal, but some tumors are known to recur and even metastasize (rare cases)

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

• Inflammatory Myofibroblastic Tumor of Small Bowel is typically seen in young adults and children, including in newborns and infants
• Very few cases of IMT of Small Intestine have been recorded in the medical literature
• Both males and females are affected, though females are affected more than males
• 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 Small Intestine? (Predisposing Factors)

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

The cause of development of Inflammatory Myofibroblastic Tumor of Small Intestine 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 Small Intestine?

The signs and symptoms depend on the size of the tumor. Specific signs and symptoms of Inflammatory Myofibroblastic Tumor of Small Intestine are generally not observed. The presentations are based upon the site of tumor in the small intestine and may include:

• Small tumors usually do not cause any symptoms. But, occasionally they may become painful, if they compress surrounding structure
• Abdominal cramps or abdominal pain that may be acute
• Mild abdominal tenderness
• Nausea
• Indigestion; constipation; irregular bowel movement
• Malabsorption
• Fever, weakness and night sweats may be present
• Diarrhea, mostly in children

The tumor size may range from 4-8 cm. The common location for IMT in the small intestine is the peritoneal folds (mesentery and omentum, which are the fats connected to the small intestine). The mesentery and omentum is the most common extrapulmonary site following the lungs.

How is Inflammatory Myofibroblastic Tumor of Small Intestine Diagnosed?

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

• Complete evaluation of family (medical) history, along with a thorough physical examination
• X-ray of the abdomen and pelvic region
• Barium enema X-ray
• CT or MRI scan of the abdomen and pelvic region
• Abdominal ultrasound scan
• Upper GI endoscopy: An endoscopic procedure is performed using an instrument called an endoscope, which consists of a thin tube and a camera. Using this technique, the radiologist can have a thorough examination of the insides of the gastrointestinal tract
• 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 Small Intestine?

Some potential complications of Inflammatory Myofibroblastic Tumor of Small Intestine include:

• They can mimic bowel cancer and cause considerable emotional stress in the affected individual
• Intussusception: When one part of the intestine pushes itself into another part of the intestine, causing obstruction of the bowel
• Recurrence of the tumor after treatment, especially due to partial removal of IMT, which is seen in about 25-30% of the cases
• Growth retardation in children, in some cases, due to malnutrition

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 Small Intestine Treated?

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

• Surgical removal of the entire tumor is the preferred method of 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 , if there is a local invasion, or a distant metastasis is noted (in very rare cases)
• Radiation therapy and chemotherapy (used following a surgery) are not found to be very useful treatment tools
• Occasionally, some tumors are known to disappear over time, without any treatment

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

How can Inflammatory Myofibroblastic Tumor of Small Intestine be Prevented?

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

What is the Prognosis of Inflammatory Myofibroblastic Tumor of Small Intestine?

• An early diagnosis and prompt treatment of Inflammatory Myofibroblastic Tumor of Small Intestine 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
• On a complete excision and removal, IMT of Small Intestine is generally not known to recur. However, some tumors are known to recur on an incomplete removal, or can even metastasize (occasionally)

Additional and Relevant Useful Information for Inflammatory Myofibroblastic Tumor of Small Intestine:

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

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