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obsolete colonic ganglioneuroma

Description

Ganglioneuromas: A Rare Benign Tumor

Ganglioneuromas (GNs) are rare, benign tumors that originate from the undifferentiated neural crest cells of the autonomic nervous system. They are characterized by a proliferation of ganglion cells, nerve fibers, and glial cells.

Types of Colonic Ganglioneuromas

There are three types of colonic GN lesions:

  • Polypoid GNs: These are benign tumors that appear as polyps in the colon.
  • Ganglioneuromatous polyposis (GP): This is a rare condition characterized by multiple polypoid GNs throughout the colon.
  • Diffuse ganglioneuromatosis (DG): This is a rare condition where GNs are found throughout the entire colon.

Incidence and Epidemiology

Due to their rarity, the incidence of colonic ganglioneuroma is not known. Less than 100 cases have been documented in medical literature. A 10-year retrospective search of the pathology database at our institution identified eight cases of colonic GNs [8].

Clinical Presentation

Ganglioneuromas are typically asymptomatic and may be discovered incidentally during a colonoscopy or other imaging studies. They can occur at any age, but most cases are reported in adults.

Treatment and Management

The treatment for ganglioneuroma is usually surgical removal of the tumor. In some cases, a polypoid GN may be removed endoscopically.

References:

  • [1] A rare case of colonic ganglioneuroma was reported in a 51-year-old Caucasian male.
  • [3] Three types of colonic GN lesions exist: polypoid GNs, ganglioneuromatous polyposis, and diffuse ganglioneuromatosis.
  • [8] A 10-year retrospective search of the pathology database at our institution identified eight cases of colonic GNs.

Note: The references provided are based on the context information retrieved from a search engine.

Signs and Symptoms

Common Signs and Symptoms

Colonic ganglioneuromas, although rare, can present with a range of clinical manifestations. The symptoms may vary depending on the size, location, and subtype of the lesions.

  • Abdominal pain: A common presenting symptom, which can be caused by the growth of the tumor or its impact on surrounding tissues [9][13].
  • Bleeding: Hematochezia (rectal bleeding) is a possible sign, especially if the lesion is located in the rectum or sigmoid colon [10][11].
  • Constipation: Some patients may experience constipation due to the growth of the tumor or its impact on bowel function [6][12].
  • Diarrhea: In some cases, diarrhea can be a symptom, possibly due to the tumor's effect on bowel motility [7][13].
  • Ileus: A rare but possible sign, which occurs when there is a temporary halt in the normal movement of food through the intestines [9][11].
  • Weight loss: Some patients may experience weight loss due to malabsorption or other factors related to the tumor's growth [8][10].

Less Common Signs and Symptoms

In addition to the above symptoms, some patients with colonic ganglioneuromas may also experience:

  • Obstruction: A rare but possible sign, which occurs when the tumor grows large enough to block the normal flow of food through the intestines [9][11].
  • Megacolon: In some cases, the tumor can cause a significant dilation of the colon, leading to megacolon with intestinal obstruction [8][13].

Asymptomatic Cases

It's worth noting that many patients with colonic ganglioneuromas may be asymptomatic, and the lesions are often detected during screening colonoscopies.

Additional Symptoms

Diagnostic Tests

Based on the provided context, it appears that there are several diagnostic tests that can be used to identify a colonic ganglioneuroma.

Imaging Studies

  • CT scan of the chest, abdomen, and pelvis: This test can help identify any potential tumors or abnormalities in the chest, abdomen, and pelvis. [3]
  • MRI scan of the chest and abdomen: Similar to the CT scan, an MRI scan can also be used to visualize the internal organs and detect any potential issues. [3]

Colonoscopy

  • Colonoscopy is a procedure that allows doctors to visually examine the inside of the colon and rectum using a flexible tube with a camera and light on the end. This test can help identify polyps, tumors, or other abnormalities in the colon. [2, 5, 8]

Blood and Urine Tests

  • Blood tests may be done to determine if the tumor is producing hormones or other chemicals. [14]
  • Urine tests may also be performed to check for any abnormal substances that could indicate the presence of a ganglioneuroma.

Biopsy

  • A biopsy, which involves taking a sample of tissue from the affected area, can be used to confirm the diagnosis of a colonic ganglioneuroma. [4, 9]

It's worth noting that while these tests can help identify a colonic ganglioneuroma, further genetic testing may also be recommended to screen for associated syndromes (Cowden's disease, von Recklinghausen disease or MEN type IIb). [1]

Treatment

Differential Diagnosis

The differential diagnosis of colonic ganglioneuromas (GNs) includes various benign and malignant tumors that can mimic the appearance and characteristics of GNs.

Benign Tumors:

  • Neurofibromas: These are common benign neoplasms affecting the soft tissues, which can be found in the colon. They are often associated with neurofibromatosis type 1 (NF type 1) [3][5].
  • Schwannomas: These are rare, benign tumors that arise from Schwann cells and can be found in the colon. They lack ganglion cells and are a differential diagnosis for GNs [10].
  • Lipomas: These are benign tumors composed of fat tissue that can occur in the colon.
  • Leiomyomas: These are benign smooth muscle tumors that can be found in the colon.

Malignant Tumors:

  • Adenocarcinoma: This is a type of cancer that originates from glandular cells and can mimic the appearance of GNs [7].
  • Gastrointestinal stromal tumor (GIST): This is a rare, malignant tumor that arises from the interstitial cells of Cajal and can be found in the colon.
  • Neuroblastoma: This is a type of cancer that originates from immature nerve cells and can be found in the adrenal glands or other parts of the body, including the colon [8].

Other Conditions:

  • Hirschsprung's disease: This is a congenital condition characterized by the absence of ganglion cells in the distal colon, which can mimic the appearance of GNs.
  • Inflammatory bowel disease (IBD): This is a chronic condition that causes inflammation and damage to the lining of the colon, which can be mistaken for GNs.
  • Intestinal tuberculosis: This is an infectious disease caused by Mycobacterium tuberculosis that can affect the colon and mimic the appearance of GNs.

It's essential to note that definitive diagnosis of colonic ganglioneuroma can only be made through pathological and histological examination, which shows the presence of ganglion cells mixed with proliferation of Schwann cells [12].

Additional Information

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