Appendix Cancer | Stages, Symptoms, & Treatments | MedStar Health

Experienced doctors, personalized treatment, and patient-centered care

If you seek care for appendix cancer at MedStar Health, you're in good hands. Here, you'll find a team of experts who specialize in using the latest research and technology to diagnose and treat cancers anywhere in the gastrointestinal tract. In fact, we're one of few cancer programs in the country to have a dedicated center for gastrointestinal cancers. Our experts are world-renowned in their efforts to find new, innovative ways to treat GI cancers, from complex surgeries to novel therapies studied and approved in clinical trials.

Appendix (or appendiceal) cancer is rare and estimated to affect one or two people out of every million in the United States each year. In those cases, abnormal cells grow wildly in the appendix (a small pouch connected to your colon with no known purpose) and form a tumor. Tumors can be either benign (non-cancerous) or malignant (cancerous).

If an appendix tumor breaks through the appendix, it can cause a very thick jelly-like substance to flow through the abdomen. It surrounds the digestive organs, like the colon, small bowel, and liver, and it eventually stops them from functioning properly. This condition is called pseudomyxoma peritonei, and it requires prompt but different treatment than cancer inside the appendix.

Symptoms and risk factors

Abdominal pain or bloating could be symptoms of appendix cancer or another illness.

Appendix tumors may not cause any symptoms, or they may cause vague symptoms that are often easily ignored until a serious problem develops. That’s why it’s important to alert your doctor to any new or unusual symptoms.

Symptoms of this cancer include:

  • Discomfort in the lower right section of your abdominal area
  • Bloating
  • An inflated abdomen, caused by fluid buildup
  • Reflux
  • Lack of appetite
  • Shortness of breath
  • Problems digesting your food
  • Constipation and/or diarrhea


Symptoms of pseudomyxoma peritonei include:

  • "Jelly belly," a distended abdomen caused by a mucin buildup
  • Inguinal hernia in men, when a mucinous tumor and/or part of the small intestine bulge through a weak area in the lower abdominal area
  • Ovarian mass or tumor in women

We're still learning what causes appendix cancer.

We continue to learn more about factors that contribute to cancer growth. The following risk factors may increase your risk of developing cancer in the appendix:

  • Smoking
  • A family history of appendix cancer
  • A family history of multiple endocrine neoplasia type 1 (MEN1) syndrome
  • Having certain conditions that affect how the stomach makes acid (atrophic gastritis, pernicious anemia, or Zollinger-Ellison syndrome)

If you have several risk factors, it's important to discuss them with your doctor.

Screening and prevention

This type of cancer is hard to detect in early stages.

People with this cancer don't usually know it in early stages, and there aren't any screening tests widely available to catch it early. The best way to prevent this type of cancer is to adopt a healthy lifestyle which can lower your risk of developing many types of cancer. You can decrease your cancer risk by:

  • Limiting your use of tobacco and alcohol
  • Eating a diet rich in fruit, vegetables, and other power foods
  • Maintaining a healthy weight

Diagnosis

Appendix tumors are most commonly found during routine exams for other problems.

Appendix tumors frequently go undetected because symptoms are easily explained away. Often, they are found during a routine examination or an abdominal procedure for a different problem, which can include appendicitis, infertility, hernia, ovarian tumors, or infertility.

To diagnose cancer of the appendix or pseudomyxoma peritonei, your doctor will need to examine results from one or more tests:

Stages of appendix cancer

Once the tumor is diagnosed, your doctor will stage the cancer, determining how extensive the disease is and whether it has spread to other organs. Staging helps your doctor decide on the most effective treatment and ranges from stage zero to four with several sub-categories. It can be generalized as:

  • Localized: Cancer is isolated to the appendix

  • Regional: Cancer has spread to nearby tissues, lymph nodes, or organs

  • Metastatic: Cancer has spread to other organs in the body

Types of cancer

The most common types of this cancer are carcinomas and carcinoid tumors of the appendix.

The primary types of appendix tumors include:

  • Carcinoid tumors: These slow-growing neuroendocrine tumors (NET) begin in the appendix and account for about one-half to two-thirds of cases. They do not usually cause symptoms unless they spread to other organs.

  • Carcinomas, including:

    • Mucinous adenocarcinomas: This tumor begins in the appendix wall and produces a thick, sticky substance called mucin.

    • Adenocarcinoma tumors (Goblet cell carcinomas): These are more aggressive than carcinoid tumors and are similar to mucinous adenocarcinomas.

    • Colonic-type adenocarcinomas: This type of tumor represents about ten percent of appendix cancer cases. It develops at the bottom of the organ, near the colon.

    • Signet-ring cell adenocarcinomas: This is the rarest and most aggressive form of appendix cancer, and it can also cause appendicitis.

Treatments

A multidisciplinary approach provides personalized, comprehensive treatment options

Because we have a specialized center for treating GI cancers, we have more experience treating appendix cancer than many other centers in the region. If you have cancer, your care team will involve experts from surgery, radiation oncology, medical oncology, and other disciplines. Together, we'll determine the best course of action for your unique case based on:

  • The size of your tumor
  • How far it has spread
  • The type of appendix tumor
  • Your general health


Surgery

In many cases, surgery is the most effective treatment option, although the procedure we recommend will vary based on the type and stage of your cancer. Our surgeons are skilled at performing a comprehensive range of surgical procedures, including the following:

  • Appendectomy: If cancer is contained to the appendix, your surgeon may remove your appendix.

  • Hemicolectomy: During this procedure, a surgeon removes the appendix, right, and nearby lymph nodes to treat carcinoid tumors larger than two centimeters. It may also be used for other types of appendix tumors.

  • Cytoreductive (debulking) surgery: When a non-carcinoid appendix cancer has spread, your surgeon may remove your tumor and surrounding fluid. Parts of other organs may also be removed if they cannot be separated from the tumor, including parts of the intestine, gallbladder, lining of the abdominal cavity, ovaries, and uterus. This approach may be combined with Hyperthermic intraperitoneal chemotherapy (HIPEC).


Hyperthermic intraperitoneal chemotherapy (HIPEC)

One of the treatment options available for this type of cancer is Hyperthermic Intraperitoneal Chemotherapy (HIPEC), a technique co-developed by one of the world’s leading experts in surgically treating advanced cancers, Dr. Paul Sugarbaker. It involves filling the abdominal cavity with a chemotherapy drug that is heated to increase effectiveness. Then, doctors rock the abdomen back and forth to evenly distribute the chemotherapy.

Chemotherapy

Chemotherapy relies on drugs or drugs in combination with other treatments to attack cancer cells. The role of chemotherapy varies depending on each patient. Your medical oncologist will help you understand your options depending on your type of cancer and whether it has spread (metastasized). In some cases, patients undergo chemotherapy before or after debulking surgery. Other patients may benefit from a one-time dose of chemotherapy using HIPEC during surgery.


Immunotherapy and targeted therapy

Targeted and immunotherapies offer advanced treatment options for patients who have tumors with certain biomarkers. We may conduct genetic profiling to determine if you would benefit from drugs that target specific gene alterations in tumors. This may include both FDA-approved drugs and those currently being tested in clinical trials.


Radiation therapy

Radiation therapy is a common approach to treating many other cancers. However, most patients with this form of cancer don't receive radiation. This decision is made on a case-by-case basis depending on what your care team thinks will be most effective in treating your cancer.

Looking for expert cancer care?

With multiple locations throughout the region, patients have access to many of the nation’s renowned cancer specialists offering high quality care, second opinions and a chance for better outcomes close to where they live and work. Georgetown Lombardi Comprehensive Cancer Center, one of the nation’s comprehensive cancer centers designated by the National Cancer Institute (NCI), serves as the research engine allowing patients access to clinical trials that often lead to breakthroughs in cancer care.

Our locations

Distance from Change locationEnter your location

MedStar Franklin Square Cancer Center at Loch Raven Campus

5601 Loch Raven Blvd.
Russell Morgan Building
First Floor
Baltimore, MD 21239

MedStar Georgetown Cancer Institute at MedStar Franklin Square Medical Center

9103 Franklin Square Dr.
The Harry and Jeanette Weinberg Cancer Institute
Suite 220
Baltimore, MD 21237

MedStar Georgetown Cancer Institute at MedStar St. Mary’s Hospital

25500 Point Lookout Rd.
First Fl.
Leonardtown, MD 20650

Why choose us

We take a multi-specialty approach to caring for each patient. Studies show that patients who receive cancer care from a team of specialists with expertise in different disciplines experience better outcomes, faster treatment after diagnosis, and more satisfying experiences. At MedStar Health, your care team involves gastrointestinal cancer experts who are leaders in research and clinical care. They meet weekly to discuss every case and design a personalized treatment plan that best meets each patient's individual needs. Your treatment team may include:

As part of the National Cancer Institute-designated comprehensive cancer center, patients benefit from groundbreaking research and access to novel clinical trials. When you think you've run out of treatment options, it's worth getting a second opinion at MedStar Health. The Georgetown Lombardi Comprehensive Cancer Center is the research engine behind our robust clinical trials program and the only NCI-designated cancer center in Washington, D.C. This distinction highlights our track record of pioneering the latest therapies and surgical techniques years before they're widely available.

Awards and recognition

Recipient of an Accreditation with Commendation, the highest level of approval, from the American College of Surgeons’ Commission on Cancer (CoC)

Numerous surgeons, medical oncologists, and surgical oncologists recognized as "Top Doctors" by both Baltimore Magazine and the Washingtonian

Magnet® designation from the American Nurses Credentialing Center (ANCC)

Clinical trials and research

Clinical trials

Clinical trials help find new and better ways to treat cancer. Your care team may recommend a clinical trial as part of your treatment if they feel it would benefit you. Eligibility may vary based on your tumor's stage, location, and genetic profile.

Research

Through The Otto J. Ruesch Center for the Cure of Gastrointestinal Cancers at Georgetown Lombardi Comprehensive Cancer Center, we're dedicated to finding curative therapies for patients with GI cancers.

Support services

Beyond treating your cancer, we're invested in your physical, spiritual, and emotional health. We provide comprehensive support services to help meet your needs and those of your family, so you can focus on healing.


Genetic counseling

While these types of cancers aren't hereditary, our genetic counselors can help you assess and manage your risk for other GI cancers that may be. Equipped with this information, you can take steps to lower your cancer risk and inform relatives who may also be affected by your results.


Personalized rehabilitation

If cancer and its treatment interferes with your ability to return to a full life, you may benefit from rehabilitation. Our comprehensive rehabilitation services are individualized to help you overcome any cancer-related challenges, from pain or weakness to difficulty sleeping, swallowing, or concentrating.


Survivorship

Returning to life as you knew it before cancer can be overwhelming. We're here to help ease the transition through education, resources, and support you may need. Our specialists will help you manage your physical and emotional wellness, address any late-effects of treatment, and successfully navigate survivorship.