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What Is Appendix Cancer?
Appendiceal cancer originates from the cells that form the appendix, a small pouch of tissue located in the abdomen. The appendix is considered a part of the intestines and colon, which function to absorb nutrients and eliminate waste from the body. Although the exact role of the appendix is not fully understood, it is believed to play a role in supporting the immune system.Appendix Cancer Prognosis and Survival Rates
Appendiceal cancer is an uncommon type of cancer that originates in the appendix. In the United States, it is estimated to affect approximately 1 to 2 individuals per 1 million people each year. However, recent studies indicate a rising incidence of appendiceal cancer in the population. This type of cancer is more frequently diagnosed in individuals between the ages of 50 and 55, though it can occur at any age. Appendiceal cancer can metastasize to various areas of the abdomen, typically occurring when the appendix ruptures. In rare cases, the cancer may spread through lymph nodes to locations outside the abdominal cavity.
Survival rates for appendiceal cancer are determined by analyzing data from groups of patients diagnosed with the disease in the past. Due to the relatively small number of individuals diagnosed with appendiceal cancer, these survival rates may not always reflect individual outcomes accurately.
For neuroendocrine tumors of the appendix and other low-grade tumors, the 5-year survival rate ranges from 67% to 97%. However, for advanced appendiceal cancer or cases where the disease has spread to other parts of the body, the 5-year survival rate may be significantly lower.
Why Come to CU Cancer Center for Appendix Cancer
As the only National Cancer Institute-designated Comprehensive Cancer Center in Colorado and one of only four in the Rocky Mountain region, the University of Colorado Cancer Center has doctors who provide cutting-edge, patient-centered bile duct care, and researchers focused on diagnostic and treatment innovations.
→ Actor's Death Sheds Light on Appendix Cancer
The CU Cancer Center offers multidisciplinary clinics offering patients access to a varied team of specialists all in one place, simplifying the process of getting the best treatment plan.
Types of Appendix Cancer
Appendix cancer is a rare form of cancer that originates in the appendix, a small pouch attached to the large intestine. The most common types of appendix cancer include:
Neuroendocrine tumor of the appendix
Neuroendocrine tumors of the appendix develop from enterochromaffin cells (ECs), which produce chemicals essential for digestion and intestinal movement. These tumors are the most prevalent form of appendiceal cancer (50% of the appendiceal tumors) and are commonly referred to as carcinoid tumors.
Epithelial Appendiceal Neoplasm
Epithelial appendiceal cancer arises from the cells lining the appendix and is often referred to as adenocarcinoma. These epithelial cells produce mucin, a jelly-like substance that protects the lining of the stomach, intestines, and appendix. A significant number of epithelial appendiceal cancers can lead to mucin accumulation, potentially causing the appendix to rupture. When mucin from the appendix accumulates in the abdomen, it results in a condition known as pseudomyxoma peritonei (PMP).
Mucinous neoplasms represent a rare and complex subset of epithelial appendiceal cancer. Based on cellular appearance under a microscope, these neoplasms can be classified as either low-grade (LAMN) or high-grade (HAMN) and may exhibit the potential to spread to other body parts. Signet Ring Cell Carcinoma is a rare and aggressive form of mucinous adenocarcinoma carcinoma that tends to spread quickly and is associated with a poor prognosis.
Goblet Cell Carcinoids
These tumors exhibit characteristics of both carcinoid tumors and adenocarcinomas. They are usually more aggressive than typical carcinoid tumors and can metastasize to lymph nodes and other organs.
Causes of Appendix Cancer
The exact causes of appendix cancer remain largely unknown. However, genetic mutations and environmental factors may play a role in its development. Certain pre-existing conditions, such as appendiceal cysts or appendicitis, may also contribute to the risk of developing cancer in the appendix.
Risk Factors
Several risk factors may increase the likelihood of developing appendix cancer:
- Age: Appendix cancer can occur at any age, but it is more commonly diagnosed in individuals over the age of 50.
- Gender: There is a slight male predominance, with men being more likely to develop appendix cancer than women.
- Genetic Predisposition: Individuals with specific genetic syndromes, such as familial adenomatous polyposis (FAP) or Lynch syndrome, have a higher risk of developing appendiceal cancer.
- History of Appendicitis: A history of appendicitis may increase the risk of developing appendiceal tumors, although the exact relationship remains unclear.
Understanding the different types of appendiceal cancer can help inform the right treatment and improve outcomes. The CU Cancer Center is dedicated to providing comprehensive care and support for patients diagnosed with this rare cancer.
Genetic Factors: Does Appendiceal Cancer Run in Families?
Currently, appendiceal cancer is not recognized as a hereditary condition and does not typically run in families.
Understanding Appendiceal Cancer Formation
The exact cause of appendiceal cancer remains unknown. Ongoing research continues to explore the mechanisms of cancer development, although establishing definitive causes can be challenging.
Symptoms
Appendix cancer often presents with subtle symptoms that may be mistaken for other conditions. Early detection is crucial for effective treatment. Common symptoms associated with appendix cancer may include:
- Abdominal Pain: Persistent pain in the lower right abdomen is a common early symptom. This pain may be sharp or dull and can vary in intensity.
- Nausea and Vomiting: Patients may experience nausea and vomiting, which can occur intermittently and may be related to bowel obstruction.
- Changes in Bowel Habits: Some individuals may notice changes in their bowel habits, such as diarrhea or constipation, often accompanied by discomfort.
- Loss of Appetite: A decrease in appetite or unexplained weight loss can be significant indicators of underlying health issues, including appendix cancer.
- Fatigue: Unexplained fatigue or a general feeling of malaise may accompany other symptoms, affecting daily activities.
- Abdominal Swelling: In advanced cases, swelling or distension of the abdomen may occur, sometimes due to fluid accumulation.
Due to the nonspecific nature of these symptoms, it is essential for individuals experiencing them to consult a health care professional for thorough evaluation and diagnosis. Early intervention can significantly improve outcomes for those diagnosed with appendiceal cancer.
Appendiceal Cancer: Overview and Diagnosis
Appendiceal cancer often presents without symptoms in its early stages.
Many cases of appendiceal cancer are diagnosed incidentally during an appendectomy, a surgical procedure typically performed to treat suspected appendicitis. In asymptomatic individuals, tumors may also be detected during imaging tests conducted for unrelated reasons.
- Diagnostic Imaging: If symptoms indicative of appendiceal cancer arise, health care providers may utilize imaging techniques such as CT (computed tomography) scans and MRI (magnetic resonance imaging) to assess the appendix.
- Biopsy: To confirm the presence of appendiceal cancer, a biopsy may be performed. This involves obtaining a small tissue sample from the tumor using a needle. A pathologist will then analyze the cells from this sample under a microscope to determine the tumor type. Due to the challenging nature of obtaining biopsies from the appendix, they are typically conducted on tissues in areas where the cancer may have metastasized.
- Diagnostic Laparoscopy: This minimally invasive procedure involves inserting an instrument into the abdomen to directly visualize the internal organs. If tumors are identified, a biopsy of the peritoneum, the membrane lining the abdominal organs, may be conducted to evaluate for cancer spread.
- Laboratory Tests: Should a biopsy confirm appendiceal cancer, the physician will order blood tests to assess protein levels. These results can help determine the cancer's stage, indicating its extent and progression. Understanding the stage of cancer is crucial for devising an appropriate treatment plan.
Accurate diagnosis is essential to ensure effective treatment for appendiceal cancer.
Treatments
Treatment for appendiceal cancer is tailored to the individual patient. It is essential to consult an expert in appendiceal cancer to determine the most effective approach.
Treatment Options:
- Surgery: Surgical intervention may involve the removal of the appendix. This may be sufficient for early-stage tumors. In more advanced cases, part of the colon may also need to be removed. If the disease has spread beyond the appendix, surgery may be an option to remove the affected organs or tissues. This can include sections of the liver and the peritoneum.
- Chemotherapy: In cases where appendiceal cancer has metastasized to other areas of the abdomen, chemotherapy may be utilized to target and eliminate cancer cells. A specialized method known as hyperthermic intraperitoneal chemotherapy (HIPEC) may also be employed to treat cancer that has spread into the peritoneum. HIPEC involves administering heated chemotherapy directly into the abdominal cavity during surgery, often after the affected tissue has been removed (also known as debulking) to destroy cancer cells in the peritoneum that are too small to be seen.
The University of Colorado (CU) Cancer Center partners with UCHealth, Children’s Hospital Colorado, and Rocky Mountain Regional VA to provide clinical care. Please make an appointment with one of our clinical partners to be seen by a CU Cancer Center doctor.
UCHealth Cancer Care - Anschutz Medical Campus
1665 Aurora Court Anschutz Cancer Pavilion
Aurora, CO 80045
720-848-0300
UCHealth Cherry Creek Medical Center
100 Cook Street
Denver, CO 80206
720-848-0000
UCHealth Cancer Center - Highlands Ranch
1500 Park Central Drive
Highlands Ranch, CO 80129
720-516-1100
UCHealth Lone Tree Medical Center
9548 Park Meadows Drive
Lone Tree, CO 80124
720-848-2200
Children's Hospital Colorado:
13123 East 16th Avenue
Aurora, CO 80045
720-777-6740
Rocky Mountain Regional VA Medical Center:
1700 North Wheeling Street
Aurora, CO 80045-7211
303-399-8020
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Information reviewed by Alexis Leal, MD, in March 2024.