Plexiform Fibromyxoma of Stomach

Plexiform Fibromyxoma of Stomach

Article
Digestive Health
Diseases & Conditions
+1
Contributed byMaulik P. Purohit MD MPHNov 20, 2018

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

  • Gastric Plexiform Angiomyxoid Myofibroblastic Tumor
  • Gastric Plexiform Fibromyxoma
  • Plexiform Angiomyxoid Myofibroblastic Tumor of Stomach

What is Plexiform Fibromyxoma of Stomach? (Definition/Background Information)

  • Plexiform Fibromyxoma of Stomach is a highly-uncommon benign tumor of the stomach. It is a recently noted tumor type that is reported almost only in the stomach
  • The cause of tumor is unknown and no risk factors are currently described. Plexiform Fibromyxoma of Stomach can occur in both children and adults
  • Small-sized tumors are not known to cause any significant symptoms, but larger tumors may lead to abdominal discomfort and compression of adjoining organs. Some tumors are known to grow into the duodenum (part of the small intestine)
  • A complete surgical removal of the lesion results in a cure. The prognosis of Plexiform Fibromyxoma of Stomach is good in a majority of cases

Who gets Plexiform Fibromyxoma of Stomach? (Age and Sex Distribution)

  • Plexiform Fibromyxoma of Stomach is a very rare tumor that is present in both children and adults, affecting a wide age range of population
  • Both males and females are affected and no gender preference is observed
  • No racial or ethnic predilection is observed

What are the Risk Factors for Plexiform Fibromyxoma of Stomach? (Predisposing Factors)

  • Currently, no definitive risk factors for Plexiform Fibromyxoma of Stomach are known

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s 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 Plexiform Fibromyxoma of Stomach? (Etiology)

The exact cause of Plexiform Fibromyxoma of Stomach is unknown.

What are the Signs and Symptoms of Plexiform Fibromyxoma of Stomach?

Some small-sized Plexiform Fibromyxomas of Stomach may not cause any significant symptoms and are detected incidentally. In others, the following signs and symptoms may be noted:

  • Abdominal discomfort and pain may be noted
  • Most tumors are small-sized, while some may grow to large sizes
  • The tumors are commonly located in the stomach region, closer to the duodenum (pyloric and antrum region)
  • Some may grow into the duodenum too and obstruct the stomach outlet
  • In some cases, blood in vomit from tumor ulceration may be noted
  • Rarely, nausea, anemia, and unexplained weight loss

How is Plexiform Fibromyxoma of the Esophagus Diagnosed?

A diagnosis of Plexiform Fibromyxoma of Stomach would involve:

  • Complete physical exam with evaluation of medical history
  • Ultrasound scan of the stomach/abdomen: It is a non-invasive procedure that uses high frequency sound waves to produce real-time images
  • Abdominal CT scan: It is a noninvasive procedure that provides more details of soft tissues, blood vessels, and internal organs
  • Stool sample analysis
  • 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
  • Chromoendoscopy can help detect small-sized tumors; small tumors can also be detected using narrow band imaging technique
  • Endoscopic ultrasonography: During this procedure, fine needle aspiration biopsy (FNAB) can be performed on the affected area. This is good technique for tumor detection including tumor invasion parameters, and whether nearby lymph nodes are affected
  • Exploratory laparoscopy (diagnostic laparoscopy) may be required, if gastrointestinal symptoms are present. In this procedure, the abdomen is examined using a minimally-invasive technique, and a tissue biopsy and tissue for culture obtained

Although the above modalities can be used to make an initial diagnosis, a tissue biopsy of the tumor is necessary to make a definitive diagnosis to begin treatment. The tissue for diagnosis can be procured in multiple different ways which include:

  • Fine needle aspiration (FNA) biopsy of the tumor: A FNA biopsy may not be helpful, because one may not be able to visualize the different morphological areas of the tumor and the tumor may be misdiagnosed. Hence, a FNA biopsy as a diagnostic tool has certain limitations, and an open surgical biopsy is preferred
  • Core biopsy of the tumor
  • Open biopsy of the tumor

Tissue biopsy:

  • A tissue biopsy of the tumor 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: In some cases, the tumor may be misdiagnosed as gastrointestinal Stromal tumor (GIST).

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 Plexiform Fibromyxoma of Stomach?

The complications of Plexiform Fibromyxoma of Stomach are normally rare; but in some cases, large tumors may cause the following complications:

  • Obstruction of the gastric passageway
  • Rarely, tumor recurrence following its surgical removal
  • Damage to the muscles, vital nerves, and blood vessels, during surgery
  • Post-surgical infection at the wound site is a potential complication

How is Plexiform Fibromyxoma of Stomach Treated?

Due to the benign nature of Plexiform Fibromyxoma of Stomach, small-sized tumors do not generally require any treatment. However, they may be removed to confirm the diagnosis.

  • In some cases, the healthcare provider may recommend a ‘wait and watch’ approach for small-sized tumors, after diagnosis of the tumor is confirmed
  • A complete surgical resection of the tumor is usually curative. It is normally undertaken when significant symptoms are observed

How can Plexiform Fibromyxoma of Stomach be Prevented?

Currently, no known preventive methods exist for Plexiform Fibromyxoma of Stomach.

What is the Prognosis of Plexiform Fibromyxoma of Stomach? (Outcomes/Resolutions)

The prognosis for individuals with Plexiform Fibromyxoma of Stomach is generally good in a majority of cases.

Additional and Relevant Useful Information for Plexiform Fibromyxoma of Stomach:

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

http://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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