What is appendiceal (appendix) cancer?


Appendiceal cancer (appendix cancer or cancer of the appendix) is a rare type of cancer that starts in the cells of the appendix, a small pouch attached to the large intestine. Because it often doesn’t cause early symptoms, it’s sometimes discovered by accident during surgery for other conditions like appendicitis.
Common related conditions
Metastatic Colorectal Cancer Colon Adenoma Colorectal Adenocarcinoma Colorectal Cancer Gastrointestinal Carcinoid Tumor

Causes of appendiceal cancer

The exact cause of appendiceal cancer isn’t always clear. It develops when cells in the appendix grow and divide uncontrollably, forming a tumor.

  • Can arise from different types of tumors (like carcinoid tumors, mucinous neoplasms and adenocarcinomas)
  • Some tumors produce mucin, which can lead to pseudomyxoma peritonei (a buildup of jelly-like material in the abdomen)
  • May be linked to genetic mutations or inherited syndromes, though this is rare

Risk factors for appendiceal cancer

Though uncommon, certain factors may increase the likelihood of developing appendiceal cancer.

  • Being over 50, though some types can occur earlier
  • Personal or family history of gastrointestinal or colorectal cancers
  • Having certain genetic conditions, like Lynch syndrome
  • Inflammatory bowel disease (IBD), such as Crohn’s or ulcerative colitis

Symptoms of appendiceal cancer

Many people with appendiceal cancer don’t notice symptoms right away. When they do appear, they can mimic other abdominal issues.

  • Pain in the lower right abdomen (may resemble appendicitis)
  • Abdominal bloating or swelling
  • Changes in bowel habits
  • Nausea or vomiting
  • Unintended weight loss
  • Feeling of fullness even after eating small amounts

Diagnosing appendiceal cancer

Since appendiceal cancer is rare and often found accidentally, diagnosis usually begins during or after surgery.

  • Imaging tests such as CT scans or ultrasounds to identify masses or mucin
  • Blood tests to check for tumor markers (e.g., CEA, CA-125)
  • Surgical exploration or appendectomy may reveal the tumor
  • Pathology testing of the appendix after removal confirms cancer type

Treatment of appendiceal cancer

Treatment depends on the tumor type, size, stage, and whether it has spread.

  • Surgical removal of the appendix (appendectomy)
  • Right hemicolectomy (removal of part of the colon) if cancer has spread
  • Cytoreductive surgery + HIPEC (heated chemotherapy) for mucinous tumors or PMP
  • Chemotherapy, particularly if cancer is more aggressive or widespread
  • Regular imaging and follow-up for monitoring

 

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