What Is Appendiceal Cancer?

Appendiceal cancer, also known as appendix cancer, happens when the cells of the appendix mutate and grow out of control and form a tumor. It is a rare condition, and it is typically found during surgery for appendicitis or during an imaging test for an unrelated condition. Small tumors of the appendix tend to not spread, but larger tumors often require more aggressive treatment. The most common form of treatment is surgery.

Your appendix looks like a small tube-like pouch that rests at the bottom right side of your stomach and it is uncertain what its primary role is. Some doctors believe it has something to do with immune function, while others believe it no longer serves a purpose.

Symptoms of Appendiceal Cancer

Symptoms of appendiceal cancer vary between individuals. Some people may exhibit no symptoms, while others may develop symptoms as the cancer spreads. Symptoms may include:

• Appendicitis
• Bloating
• Pain in the abdomen or belly
• Increase in waist size
• Ascites (fluid buildup in the abdomen)
• Nausea or vomiting
• Changes in bowel behaviors
• Early satiety (feelings of fullness)

Sometimes in rare cases, you can develop a condition known as pseudomyxoma peritonei (PMP) alongside appendiceal cancer. Symptoms of PMP may include bloating and abdominal pain.

Causes and Risk Factors of Appendiceal Cancer

What Causes Appendiceal Cancer?

There is no known cause of appendiceal cancer, but cancer develops when cells mutate and grow out of control.

Risk Factors for Appendiceal Cancer

There are several risk factors for appendiceal cancer. Risk factors include:

• Age. This type of cancer can develop at any age, but it is most common in your 50s.
• Biological gender. Females are more common than males to develop this type of cancer.
• Family history. You may have an increased risk of developing appendiceal cancer if there is a family history of this type of cancer.
• Tobacco products. Using tobacco products increases your risk of developing any type of cancer.
• Medical history. Certain medical conditions, such as atrophic gastritis, pernicious anemia, and Zollinger-Ellison syndrome may increase your risk of developing appendiceal cancer.

Types of Appendiceal Cancer

There are various types of appendiceal cancer. The tumors that develop in the appendix can be benign or malignant. The malignant tumors of the appendix are classified into two types, which include appendiceal adenocarcinoma and neuroendocrine tumors.

Appendiceal Adenocarcinoma (Epithelial Tumors)

The most common form of appendix cancer is appendiceal adenocarcinoma. This type of cancer begins in the glandular tissue, which is the tissue that lines your organs. There are different types of appendiceal adenocarcinoma:

• Mucinous adenocarcinoma. This type begins in the lining of your appendix. It releases a component of mucus, known as mucin.
• Colonic-type (nonmucinous) adenocarcinoma. This type develops close to the bottom of your appendix and shares similarities with colon cancer.
• Signet ring cell adenocarcinoma. A rare and aggressive form of appendiceal cancer, named for the signet ring appearance of its cells under a microscope.
• Goblet cell adenocarcinoma. A highly aggressive and rare form of appendiceal cancer, with characteristics of both adenocarcinoma and neuroendocrine tumors.

Appendiceal Neuroendocrine Tumors (Carcinoid Tumors)

This type of cancer affects the neuroendocrine cells, which regulate hormone release. These tumors typically grow slowly and may go undetected for years.

Diagnosis of Appendiceal Cancer

Most cases of appendix cancer are diagnosed incidentally during surgery for an appendectomy or through imaging for unrelated conditions. If cancer is suspected, further testing may include:

• Imaging tests (CT scan or MRI)
• Laparoscopy (a small camera inserted through the abdomen to capture images)
• Biopsy (a small tissue sample examined in a laboratory)
• Blood tests

Treatment Options for Appendiceal Cancer

Surgical Treatments

Surgery is the primary treatment for appendiceal cancer. Procedures may include:

• Appendectomy. Removal of the appendix.
• Hemicolectomy. Removal of part of the colon adjacent to the appendix, often performed for larger carcinoid tumors.
• Cytoreductive surgery. Used for non-carcinoid tumors, removing tumors and surrounding fluid.
• Peritonectomy. Removal of the peritoneum if cancer has spread to the abdominal lining.

Drug Therapies

When surgery alone is insufficient, drug therapies may be recommended, including:

• Chemotherapy. Used when cancer has spread beyond the appendix.
• Hyperthermic intraperitoneal chemotherapy (HIPEC). A heated chemotherapy treatment administered during surgery to target cancer cells in the abdomen.
• Targeted drug therapy. Attacks specific cancer cells while minimizing damage to healthy cells.

Supportive Care

Managing cancer involves both treatment and recovery. Supportive care helps address pain, fatigue, nausea, and emotional or mental well-being. Baptist Health offers resources for pain management and holistic care.

Living with Appendiceal Cancer

If you experience symptoms such as appendicitis, abdominal pain, or bloating, consult your healthcare provider. For those living with appendiceal cancer, worsening or unmanageable symptoms should be reported to your provider for additional care and support.

Why Choose Baptist Health for Appendiceal Cancer Care?

Baptist Health provides comprehensive, patient-centered care for appendiceal cancer, from diagnosis to treatment and recovery. Our experienced oncology team offers advanced surgical options, innovative drug therapies, and personalized supportive care. Call Baptist Health to learn more about our approach to appendiceal cancer care.

Frequently Asked Questions (FAQs)

What is the most aggressive appendix cancer?

The most aggressive forms of appendix cancer are goblet cell adenocarcinoma and signet ring cell adenocarcinoma.

Does appendix cancer spread fast?

Yes. Appendix cancer can spread quickly, particularly if it reaches the lymph nodes.

Can a colonoscopy detect appendix cancer?

Appendix cancer is rarely detected by a colonoscopy, as it often presents without symptoms. It is usually discovered during surgery for an appendectomy or through imaging tests conducted for unrelated conditions.