Appendix cancer is a rare malignancy that begins in the appendix, a small pouch-like organ located in the lower right abdomen.
What is appendix cancer?
Appendix cancer begins when cells in the appendix start to change and grow out of control, forming a mass or tumor. The appendix is a small, finger-shaped pouch attached to the large intestine. While its exact function is not completely understood, it's thought to play a role in housing beneficial bacteria for the digestive system. Cancer can affect any part of the appendix.
According to the National Cancer Institute, appendix cancer is rare, occurring at a rate of 1 to 2 per million people in the U.S. However, studies show an increase in appendix cancer among Gen X and Millennials. The Annals of Internal Medicine released findings that rates of appendix cancer diagnosed in people younger than age 50 is at 1 in every 3. Historically, doctors were seeing 1 in every 8 people over the age of 50 being diagnosed with appendix cancer. Researchers have not pinpointed why there is an increase in diagnosis in younger populations, but being aware of the risk factors and causes can help spot an issue earlier.
AHN Molecular, Metastatic, and Rare Cancer Center of Excellence
Our Molecular, Metastatic, and Rare Cancers Center of Excellence was designed to address the unique challenges posed by complex cancer types, including appendix cancer. This includes rare cancers, such as peritoneal and pleural metastases, and hereditary cancer syndromes. We use cutting-edge genomic sequencing and molecular profiling to identify specific genetic mutations, alterations, and biomarkers that drive these metastatic processes. Our treatment options are focused on your specific cancer and needs.
Why choose AHN for your appendix cancer care?
Our center stands at the forefront of innovation, offering a combination of expertise, resources, and compassion that few institutions can match. Here are some key differentiators:
- Precision oncology: We employ next-generation sequencing and molecular profiling to analyze the genetic and molecular characteristics of each tumor. This enables us to customize treatment strategies that target the specific mutations driving the cancer, maximizing efficacy while minimizing side effects.
- Comprehensive multidisciplinary care: Our team includes oncologists, molecular pathologists, geneticists, radiologists, surgeons, and specialized nurses who collaborate to create and implement individualized care plans. Each patient’s case is reviewed in tumor boards, ensuring a holistic and tailored approach.
- Access to innovative clinical trials: We provide patients with access to a robust portfolio of clinical trials, including those investigating novel therapies such as bispecific antibodies. Our close partnerships with pharmaceutical companies and research institutions allow us to bring promising new treatments to our patients before they are widely available.
- Focus on rare and metastatic cancers: Our expertise extends to cancers that are often underrepresented in clinical studies, such as sarcomas, neuroendocrine tumors, and metastatic cancers of unknown primary origin. This focus ensures that patients with rare and complex conditions receive the same level of specialized care as those with more common malignancies.
- Patient-centered care: From advanced pain management to psychological support, our center is committed to treating the whole patient. Dedicated patient navigators guide individuals and their families through every stage of the journey, providing education, emotional support, and assistance with logistics.
Appendix cancer symptoms and signs
While appendix cancer is rare and many symptoms and signs of it are shared with other conditions, it’s important to note any changes in your health and talk with your doctor about different symptoms you’re experiencing. Regular communication with your doctor can help spot potential issues before they become worse. Some symptoms and signs of appendix cancer to be on the lookout for include:
- Abdominal pain: This is the most common symptom. It may be vague, or a general discomfort in the abdomen.
- Bloating: A feeling of fullness or swelling in the abdomen.
- Changes in bowel habits: This could include diarrhea, constipation, or other changes in your normal bowel patterns.
- Nausea and vomiting: Feeling sick to your stomach and/or throwing up.
- Appendicitis-like symptoms: Because appendix cancer can sometimes be discovered during or after surgery for what appears to be appendicitis, some symptoms may mimic appendicitis. These include sharp pain in the lower right abdomen, fever, and loss of appetite.
- Ascites: Accumulation of fluid in the abdominal cavity, leading to swelling.
- Hormone-related symptoms: Certain types of appendix cancer (carcinoid tumors) can produce hormones that cause specific symptoms, such as flushing of the skin, diarrhea, wheezing, and rapid heartbeat.
- Weight loss: Unexplained weight loss can sometimes be a sign of cancer.
- Palpable mass: In rare cases, a lump or mass may be felt in the abdomen.
Causes and risk factors
The exact cause of appendix cancer isn’t fully understood, but research has identified certain risk factors that may increase the likelihood of developing this rare disease. The most well-known cause for appendix cancer is a genetic mutation that causes cells to grow and multiply uncontrollably. The risk factors that increase the possibility of genetic mutation often include:
- Family history of MEN1 syndrome: Multiple endocrine neoplasia type 1 (MEN1) is a rare, inherited disorder that causes tumors in the endocrine glands and other parts of the body. People with MEN1 have an increased risk of developing carcinoid tumors, including those in the appendix.
- Preexisting conditions: Certain preexisting conditions may slightly increase the risk, such as:
- Atrophic gastritis: Chronic inflammation of the stomach lining.
- Pernicious anemia: A decrease in red blood cells that occurs when the intestines cannot properly absorb vitamin B12.
- Age: Appendix cancer is most commonly diagnosed in adults between the ages of 50 and 59.
- Sex: Some studies suggest that appendix cancer may be slightly more common in women than in men.
- Race: Some studies suggest that appendix cancer may be more common in white people than in Black people.
- Smoking: A history of smoking or a current smoker will have increased risk of appendix cancer.
Appendix cancer screening and diagnosis
Due to its rarity and lack of specific symptoms, there are no standard screening guidelines for appendix cancer. Diagnosis typically occurs when the appendix is removed during surgery for suspected appendicitis, or during imaging tests for other conditions.
Imaging tests
Imaging tests create pictures of the inside of your body, allowing doctors to look for abnormalities. These tests are helpful in identifying tumors, including those affecting the appendix, assessing their size and location, and determining if the cancer has spread.
- Computed tomography (CT) scan: This is often the primary imaging test used. CT scans use X-rays to create detailed cross-sectional images of the abdomen and pelvis. They can help detect tumors in the appendix, as well as any spread to nearby tissues or organs.
- Magnetic resonance imaging (MRI): MRI uses strong magnets and radio waves to create detailed images. It can be helpful in evaluating the extent of the tumor and assessing if it has spread.
- Ultrasound: An ultrasound uses sound waves to create images of the organs in the abdomen and pelvis. It may be used as an initial test, particularly in children or pregnant women, but it's generally not as detailed as CT or MRI.
- Angiography: If the tumor is suspected to be affecting blood vessels, an angiography might be performed. This involves injecting a dye into the blood vessels and taking X-rays to visualize them.
Biopsy
A biopsy is the removal of a tissue sample for examination under a microscope. It's the only way to definitively diagnose appendix cancer. At AHN, we work to create a thorough review of your tissue sample to identify the health issues affecting our patients. The type of biopsy performed depends on the location and size of the suspected tumor. Your biopsy procedure may include:
- Surgical biopsy: If the tumor is easily accessible, a surgeon may remove a sample of tissue during an exploratory laparotomy (open surgery) or laparoscopy (minimally invasive surgery). In some cases, the entire appendix is removed.
- Image-guided biopsy: If the tumor is deep within the abdomen, for example spreading to the peritoneum (the lining of the abdomen), a radiologist may use imaging techniques like CT or ultrasound to guide a needle to the tumor and extract a tissue sample.
- What to expect: Before the biopsy, your doctor will explain the procedure and any associated risks. You may receive local anesthesia to numb the area or general anesthesia to put you to sleep. After the biopsy, the tissue sample is sent to a pathologist, who examines it under a microscope to look for cancer cells.
- Pathology report: The pathologist will create a report detailing the type of cancer, its grade (how aggressive it is), and other important characteristics. Additional tests like next gen sequencing (NGS) or molecular profiles can also be done to further characterize the tumor and may open treatment options. This information is crucial for determining the best course of treatment.
Blood tests
Blood tests alone cannot definitively diagnose appendix cancer, but they can provide valuable information about your overall health and help identify certain markers that may indicate cancer. Some of the blood tests that may be used include:
- Complete blood count (CBC): This test measures the levels of different types of blood cells. Abnormalities in blood cell counts can sometimes indicate the presence of cancer or other conditions.
- Comprehensive metabolic panel (CMP): This test measures various substances in your blood, such as electrolytes, liver enzymes, and kidney function. It helps assess the overall health of your organs and can detect abnormalities that may be associated with cancer.
- Tumor markers: Some appendix cancers produce specific substances called tumor markers, which can be detected in the blood. We look for the following tumor markers:
- Carcinoembryonic antigen (CEA): This marker is sometimes elevated in appendix cancer, particularly in adenocarcinomas.
- CA 19-9: A carbohydrate antigen that may indicate the presence of cancer.
- CA 125: This is a cell surface glycoprotein that, when elevated, may indicate a presence of cancer.
- Hormone levels: In the case of carcinoid tumors, blood tests can also be used to measure hormone levels (such as serotonin) that may be produced by the tumor. We look for:
- Chromogranin A (CgA): This marker is often elevated in carcinoid tumors of the appendix.
- Serotonin: An excessive amount of serotonin can be a tumor marker.
- 5-Hydroxyindoleacetic acid (5-HIAA): If a carcinoid tumor is suspected, a urine test may be done to measure 5-HIAA, a breakdown product of serotonin.
Types and stages of appendix cancer
Appendix cancers are classified based on the type of cells where the cancer originates. The main types include:
- Neuroendocrine tumors: These are the most common type. They develop from specialized cells called neuroendocrine cells. They often grow slowly and may not cause symptoms until they are advanced.
- Epithelial tumors: These tumors start from the lining of the appendix. They can spread and cause a condition called pseudomyxoma peritonei (PMP) where mucus builds up in the abdomen. There are several subtypes of epithelial tumors including:
- Adenocarcinomas: The most common type of epithelial tumor. These are gland-forming cancers.
- Mucinous adenocarcinomas: A type of adenocarcinoma that produces large amounts of mucus. They can spread and cause a condition called pseudomyxoma peritonei (PMP), where mucus builds up in the abdomen.
- Signet ring cell adenocarcinomas: A rare and aggressive type of adenocarcinoma where the cells have a characteristic appearance under the microscope.
- Goblet cell carcinoids (also called goblet cell adenocarcinomas): These have features of both carcinoid and adenocarcinoma tumors.
- Low-grade mucinous neoplasm of the appendix (LAMN): This is a non-nvasive or minimally invasive tumor of the appendix that produces mucus and generally has a low potential for aggressive spread.
- High-grade mucinous neoplasm of the appendix (HAMN): Considered an intermediate lesion, this may have a more aggressive course of spread.
Once your doctor has determined the type of appendix cancer, they can do further testing to determine the stage of the cancer. Staging helps your AHN care team understand if and how the cancer has spread to other areas of the body. Staging is based on tumor size, lymph node involvement, and metastasis (spread to other organs). Appendix cancer staging uses the TNM system. T describes the size and extent of the primary tumor, N describes the involvement of lymph nodes, and M describes metastasis. Your doctor will most likely stage your cancer as the following:
- Stage 0 (Carcinoma in situ): Abnormal cells are found only in the lining of the appendix.
- Stage 1: The cancer is confined to the appendix.
- Stage 2: The cancer has grown through the wall of the appendix and may have spread to nearby tissues.
- Stage 3: The cancer has spread to nearby lymph nodes.
- Stage 4: The cancer has spread to distant sites, such as the peritoneum.
Appendix cancer treatment
In appendix cancer, the primary focus is on treating the appendix tumor. Treatment options depend on the type and stage of the cancer, as well as the patient's overall health. Your AHN care team will tailor your treatment plan to your specific needs and health condition.
Surgery
Appendix cancer surgery usually involves an appendectomy (removal of the appendix) for early-stage disease. More advanced cases may require right hemicolectomy (removal of part of the colon) and lymph node dissection.
Cytoreductive surgery (CRS) and Hyperthermic Intraperitoneal Chemotherapy (HIPEC)
CRS involves surgically removing all visible tumors and cancerous tissue from the abdominal cavity. The goal is to remove as much of the cancer as possible, and can include removing multiple organs. This can be a lengthy and complex surgery, often lasting many hours. Your AHN surgeon and care team are highly skilled and experienced in this procedure and understand the risks, complications, and emotions involved with this treatment option. They will be with you throughout your surgery, treatment, and recovery to help you achieve your best outcome.
HIPEC is a highly concentrated, heated chemotherapy treatment delivered directly into the abdominal cavity after surgery to remove visible tumors. After surgeons remove as much of the visible tumor as possible (a procedure called cytoreduction), the heated chemotherapy solution is circulated within the abdomen for a period, typically 60 to 90 minutes. The heat enhances the chemotherapy's effectiveness in killing remaining cancer cells. HIPEC is used to treat cancers that have spread within the abdominal cavity, particularly mucinous adenocarcinomas that can cause pseudomyxoma peritonei (PMP). It aims to eliminate cancer cells that may not be visible during surgery. AHN’s HIPEC capabilities work to attack cancer cells causing fewer side effects than traditional chemotherapy.
Systemic chemotherapy
Systemic chemotherapy, which is traditional chemotherapy, involves using drugs that travel through the bloodstream to reach cancer cells throughout the body. Chemotherapy drugs interfere with cancer cells' ability to grow and divide. The specific drugs and regimen depend on the type and stage of appendix cancer. Patients can expect to receive chemotherapy in cycles, with rest periods in between to allow the body to recover. Side effects can vary but may include nausea, fatigue, hair loss, and increased risk of infection. Medications can help manage many of these side effects. Regular monitoring of blood counts and overall health is essential. Not all appendix cancer needs systemic chemotherapy or responds to it. Your AHN care team will determine which course of treatment will be best for you.
Targeted therapy
Targeted therapy uses drugs that target specific molecules (genes or proteins) involved in cancer cell growth and survival. By targeting these specific molecules, targeted therapies can block cancer cell growth or spread with fewer side effects than traditional chemotherapy. However, this requires biomarker testing. Targeted therapies are usually taken as pills or given intravenously. Side effects depend on the specific drug and target. Common side effects may include skin rashes, diarrhea, or high blood pressure. Regular monitoring is needed to assess the treatment's effectiveness and manage side effects.
Immunotherapy
AHN’s immunotherapy capabilities harnesses the body's own immune system to fight cancer. These drugs help the immune system recognize and attack cancer cells. Immunotherapy is usually administered intravenously. Side effects can vary widely; some common ones include fatigue, skin reactions, and gastrointestinal issues. In some cases, immunotherapy can cause the immune system to attack healthy organs, leading to more serious side effects. Testing is critical prior to starting immunotherapy because not everyone is a candidate for it and biomarker testing is required to ensure you will respond. Regular monitoring is required.
Palliative care
Palliative care focuses on providing relief from the symptoms and stress of a serious illness like cancer. AHN palliative care aims to improve quality of life for both you and your family by supporting your physical and emotional health. Palliative care can include pain management, symptom control, emotional support, and help with decision-making. Palliative care is helpful and available, no matter your cancer stage or diagnosis point, and it can be given alongside other treatments. The goal is to help patients live as comfortably and fully as possible.
Appendix cancer FAQs
Given the rarity of appendix cancer, being diagnosed or being at higher risk can create a lot of questions. AHN is here to help. Your specific and dedicated care team will be your resource to answer any and all of your questions, but to help get you started, we’ve included answers to some frequently asked questions our patients often have. These can help you organize your thoughts prior to meeting with your care team.
How rare is appendix cancer?
Appendix cancer is very rare. It's estimated that about 1 in 500,000 people will be diagnosed with it each year. This makes it one of the least common types of cancer. Cases have been on the rise in younger populations, and AHN, along with researchers, will continue to monitor this change in diagnosis rates.
What is the survival rate for appendix cancer?
Survival rates vary widely depending on the type and stage of the cancer at diagnosis. Generally:
- Early-stage: When the cancer is found early and hasn't spread, the five-year survival rate can be quite high (80–90%).
- Later-stage: If the cancer has spread to nearby lymph nodes or distant sites, the survival rate is lower, ranging from 20% to 70%.
These are general estimates, and individual outcomes can vary significantly based on various factors.
Can appendix cancer be cured?
Yes, in many cases, especially when detected at an early stage. Treatment with surgery, and sometimes chemotherapy, can be effective in curing appendix cancer. Even in more advanced stages, treatment can help control the disease and improve quality of life.
What is stage 1 cancer of the appendix?
Stage 1 appendix cancer means that the cancer is confined to the appendix itself. It hasn't spread to nearby tissues, lymph nodes, or distant sites.
Does appendix cancer spread quickly?
The rate of spread depends on the type of appendix cancer:
- Carcinoid tumors: These often grow slowly.
- Adenocarcinomas: These can be more aggressive and spread more quickly, especially signet ring cell adenocarcinomas.
- Mucinous adenocarcinomas: These can spread and cause a condition called pseudomyxoma peritonei (PMP).
What are the signs and symptoms of appendix cancer?
Symptoms can be vague and are often similar to other more common conditions, which can make diagnosis challenging. They may include:
- Abdominal pain (often mimicking appendicitis)
- Bloating
- Changes in bowel habits
- A mass in the abdomen
- In some cases, no noticeable symptoms
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