Neuroma of Appendix

Neuroma of Appendix

Article
Digestive Health
Diseases & Conditions
+1
Contributed byKrish Tangella MD, MBAAug 17, 2023

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

  • Appendiceal Neuroma
  • Fibrous Obliteration of Appendix
  • Neuronal Hyperplasia of Appendix 

What is Neuroma of Appendix? (Definition/Background Information)

  • Neuroma of Appendix is the most common (mostly benign) tumor in the appendix that arises due to a proliferation of nerve cells. The appendix is a small blind pouch of the colon, located at the beginning of the colon (large intestine)
  • When these changes occur only in the mucosal surfaces (lining of the appendix), and does not affect the lumen of the appendix, it is called an intramucosal neuroma
  • When Neuroma of Appendix is examined under the microscope by a pathologist, the lumen of the appendix is occupied by a mixture of spindle cells, hypertrophied nerve bundles, fat cells (adipocytes) and collagen bundles
  • Earlier, the tumor was diagnosed as a Fibrous Obliteration of Appendix. In this condition, the lumen of the appendix is fibrosed (i.e., obliterated). However, the presence of the tumor does not increase the chance for a malignancy
  • Neuroma of Appendix is seen among a wide age range, though mostly in elderly adults. It may be misdiagnosed as a severe manifestation of inflammatory bowel disease or acute/chronic appendicitis
  • The signs and symptoms depend upon the size of the tumors and it may include abdominal pain/discomfort and blood in stool. Many Neuroma of Appendix tumors are found incidentally while examining the individual for other medical conditions, or following an appendectomy
  • Typically, a surgical excision of Neuroma of Appendix with its entire removal is the treatment of choice. The prognosis is excellent with its complete removal in a majority of cases

Who gets Neuroma of Appendix? (Age and Sex Distribution)

  • Neuroma of Appendix is a common type of mesenchymal (non-epithelial) tumor in the appendix. In some studies, 27% of appendix specimens examined in a laboratory presented with neuromas
  • The tumor may be seen in individuals of a wide age group, but it is usually noted in older adults (higher prevalence rate with increasing age). Appendiceal Neuromas are rare in children
  • Both males and females can be affected
  • No specific ethnic or racial preference is seen

What are the Risk Factors for Neuroma of Appendix? (Predisposing Factors)

  • Currently, no definitive risk factors are reported for Neuroma of Appendix
  • Also, no direct involvement of inflammatory bowel disease (IBD) with Appendiceal Neuromas are noted 

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 Neuroma of Appendix? (Etiology)

The exact cause and mechanism of Neuroma of Appendix formation is unknown.

  • Some researchers believe that it may be due to chronic infection leading to fibrosis
  • The lymphoid tissue of the appendix is replaced by fibrous tissue nerve

What are the Signs and Symptoms of Neuroma of Appendix?

In a majority of individuals, Neuroma of Appendix does not cause any significant symptoms. They may be detected incidentally during imaging studies that are performed for other health condition. It is reported that the tumor condition is often diagnosed when the appendix is removed for acute appendicitis.

In some individuals, the following signs and symptoms of Neuroma of Appendix may be present that include:

  • Symptoms that are similar to acute appendicitis such as the following:
    • Abdominal pain that first originates around the umbilicus and then gradually moves towards a point on the right lower quadrant of the abdomen, called the McBurney’s point
    • Sometimes, the pain can occur anywhere in the upper or lower abdomen, back, or rectum
    • Nausea and vomiting
    • In some, fever, shivering, and chills may be noted
    • Loss of appetite
    • Constipation or diarrhea
    • Occasionally, painful urination and severe cramps can occur
  • Presence of a true tumor is very rarely noted. In many cases, the entire lumen is involved and destroyed (obliterative fibrosis)
  • In a few cases, the superficial layers, the mucosa and submucosa, are involved and no lumen is obstructed
  • Swollen abdomen
  • The location of the neuroma is mostly the center of the appendix

How is Neuroma of Appendix Diagnosed?

In many, Neuroma of Appendix is diagnosed incidentally during imaging studies undertaken for some other medical conditions. The diagnosis may involve the following tests and procedures:

  • Physical exam and evaluation of medical history
  • Complete blood count (CBC)
  • Basic metabolic panel 
  • X-ray of abdomen
  • Ultrasound or CT scan of abdomen
  • Screening colonoscopy: A colonoscopy is a test that allows the physician to look at the inner lining of the colon and rectum. A typical colonoscopy involves using a thin, flexible tube (called a colonoscope), with an attached video camera, to view the colon and rectum

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. If it is indeed a polyp, a distinct appearance is noted by the pathologist. 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: A differential diagnosis may be necessary to eliminate certain tumors, such as carcinoid tumor, presenting similar signs and symptoms.

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 Neuroma of Appendix?

The complications of Neuroma of Appendix may include:

  • Presence of severe pain
  • Rupture/perforation of the appendix
  • Damage to the muscles, vital nerves, and blood vessels, during surgery
  • Post-surgical infection at the wound site is a potential complication

How is Neuroma of Appendix Treated?

The treatment measures for Neuroma of Appendix may include the following.

  • A complete surgical resection of the tumor via the following procedures:
    • Open appendectomy: It involves making a deep incision on the lower abdomen and removing the appendix from the surrounding abdominal organs
    • In laparoscopic approach, a surgeon makes 3 or 4 small incisions for insertion of the camera and the laparoscopic instruments and the appendix is cut and removed
  • Post-operative care is important: A minimum activity level is to be ensured, until the surgical wound heals

Follow-up care with regular screening may be recommended by the healthcare provider.

How can Neuroma of Appendix be Prevented?

Current medical research has not established a method of preventing Neuroma of Appendix.

What is the Prognosis of Neuroma of Appendix? (Outcomes/Resolutions)

The prognosis of Neuroma of Appendix is generally excellent with adequate treatment.

Additional and Relevant Useful Information for Neuroma of Appendix:

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

Krish Tangella MD, MBA picture
Approved by

Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team

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