Appendix Cancer

DESCRIPTION AND TYPES

Tumors that occur in the appendix comprise a large group of both benign and malignant diseases. Appendix cancer is extremely rare. Most patients are diagnosed after undergoing surgery for acute appendicitis or when an abdominal mass is seen during a CT scan for an unrelated condition.

Tumor size is directly related to prognosis (outlook). Appendix tumors less than two centimeters have a low tendency to spread and patients generally have an excellent five-year survival rate, while tumors larger than 2.5 centimeters may require more aggressive treatment and patients have a lower survival rate.

Tumors of the appendix can be divided into two major groups:

Carcinoid Tumors

Carcinoid tumors are the most common form, representing about half of all appendix tumor cases. These tumors are more common in women, and generally occur in the fourth decade of life. About two-thirds of carcinoid tumors are found in the tip of the appendix, where they do not obstruct the opening between the appendix and colon.

In the majority of cases, carcinoid tumors are small (less than two centimeters) and have a very low risk of spread to nearby lymph nodes. Appendectomy (removal of the appendix) is adequate treatment in these cases. Larger tumors have a slightly higher risk of metastasizing to surrounding lymph nodes and the liver. Removal of the right colon and regional lymph nodes is recommended.

More than 85% of patients with carcinoid tumors of the appendix (even with regional metastases) have a five-year survival rate.

Most patients have no symptoms. However, some signs may include:

  • Acute appendicitis: most cases are discovered during surgery for appendicitis
  • Carcinoid syndrome (if spread to liver): flushing, shortness of breath, diarrhea, heart valve disease on the right side

Non-Carcinoid Tumors

Non-carcinoid tumors of the appendix originate from the epithelial cells lining the inside of the appendix. Most of the cells creating these tumors produce a gelatinous material known as mucin. Due to the anatomic structure of the appendix and the mucin production of these tumors, patients with non-carcinoid tumors of the appendix are at risk for spread of tumor cells and mucin within the abdominal cavity. If left untreated, both tumor cells and mucin can accumulate over time and lead to life-threatening bowel obstruction and cachexia (weight loss, muscle atrophy, significant loss of appetite, fatigue). Prognosis and treatment for these tumors depend on a number of patient and tumor-related factors.

Pseudomyxoma peritonei (PMP) is defined by the presence of acellular (i.e., few, if any, tumor cells) mucin within the abdominal cavity. The most common presentation of PMP is a mucin-producing tumor of the appendix whose cells have spread outside the appendix and into the abdominal cavity. The resulting tumor, which looks like orange jelly, can get as large as 20-30 pounds.

There are no PMP-specific symptoms, but some of the more frequent presenting signs include:  

  • Increase in abdomen size/girth
  • Vague abdominal discomfort; persistent discomfort in the lower right abdomen
  • Pelvic discomfort
  • New hernias
  • Bowel obstruction
  • Ovarian masses

Adenocarcinoid tumors, also known as goblet cell carcinomas, have characteristics similar to both carcinoid and adenocarcinoma tumors of the appendix. Most patients are diagnosed in their 50s. The prognosis for these tumors is slightly poorer than that of carcinoid tumors, with an overall five year survival rate of 78%. However, only about 14% of patients with Stage IV disease survive longer than five years. The main presenting symptom is acute or chronic abdominal pain.

Symptoms

Appendix cancer may not cause any specific symptoms until it has progress into advanced stages. However, abdominal discomfort and bloating is sometimes experienced with advanced disease.

...AND MD ANDERSON

PREVENTION

Risk Factors

Appendix cancer has no known cause. There are no known lifestyle changes that one can make to lower the risk of developing appendix cancer.

DIAGNOSIS

Often, appendix cancer remains undiagnosed until it is unexpectedly found during or after abdominal surgery or when an abdominal mass is seen during a CT scan for an unrelated condition. If appendix cancer is suspected during abdominal surgery, the surgeon would take a tissue sample (biopsy) for a pathologist to review. Otherwise, the cancer may be diagnosed after your abdominal surgery as part of the routine pathology reading. In that case, another surgery would most likely be recommended to make sure all the cancerous tissue is removed.

TREAT

Treating appendix cancer depends on the type of tumor, its location within the appendix and whether or not it has spread. Because these tumors are relatively rare, particularly the non-carcinoid tumors, most treatment strategies have not been tested in standard clinical trials. In general, patients will undergo surgery to remove the appendix and/or right colon.

For patients with non-carcinoid tumors that have spread within the abdominal cavity, the most effective therapy appears to be a combined approach of cytoreductive (tumor debulking) surgery and hyperthermic intraperitoneal (i.e., in the abdominal cavity) chemotherapy.

Cytoreductive surgery is a major operation to remove all the tumor and mucin present within the abdominal cavity. This can often require removing involved organs such as parts of the intestine, gallbladder, spleen, ovaries, uterus and even the lining of the abdominal cavity (peritoneum). In patients whose tumors can be completely or nearly completely removed by surgery, administration of hyperthermic intraperitoneal chemotherapy has been shown to significantly improve survival.

Hyperthermic intraperitoneal chemotherapy (HIPEC) is performed only if a complete tumor debulking is achieved. During the surgery, the abdominal cavity is filled with a chemotherapy drug, which is heated to over 40 degrees Celsius. The patient's abdomen is then gently rocked back and forth for 90 minutes to ensure that the chemotherapy bathes all areas of the abdominal cavity, killing any remaining tumor cells.

Currently there is no published data supporting the use of systemic (intravenous) chemotherapy for patients with non-carcinoid appendical tumors. However, there may be an evolving role for the systemic therapies in the management of metastatic colorectal cancer. There is no known role for radiation therapy in the treatment of cancers of the appendix.

SUPPORT

Cancer is a journey that no one needs to take alone. There are many forms of support to help you through every stage: diagnosis, treatment and survivorship. Whether you meet with other cancer survivors like yourself, use complementary therapies or individual coping mechanisms, support is available. Listed below are just some of the ways to find help and hope.

Support Groups

Getting together with other cancer patients in a support group is a valuable coping tool. Support groups are usually focused on a single disease or topic, such as breast cancer survivors or people coping with life-changing side effects from their cancer or cancer therapy. These groups allow participants to meet others like themselves and seek strength from each other. Most major cities and cancer hospitals offer support groups that meet weekly or monthly. There are also dozens of online support Web sites or message boards for those who may not have access to a traditional meeting. 

Complementary Therapies

Complementary therapies are used in conjunction with cancer treatment, in an effort to reduce treatment side effects, ease depression and anxiety and help cancer patients take their mind off the negative aspects of their situation. Complementary therapies may include mind-body exercises like yoga, Tai Chi and Qi gong; visualization or guided imagery; using art or music as therapy and self-expression and traditional Eastern medicine such as acupuncture. 

Physical Activity

Staying physically active as much as possible during cancer treatment has many positive benefits. Physical activity stimulates the release of endorphins, a hormone that helps elevate mood, as well as decreasing feelings of fatigue.

Exercises for cancer patients can range from simple stretches done in the bed or chair, to more active pursuits such as walking or light gardening work. However, it’s important not to push yourself too hard. Check with your doctor before attempting any physical activity to make sure you are up to it.

Journaling/Blogging

Many people find it helpful to keep a journal of their cancer treatment experience. It may be as simple as recording symptoms and side effects into a notebook, or may include personal emotions and opinions about what they may be going through. Journals can be private, like a diary, or shared with loved ones and even strangers. 

Increasingly, people are turning to the Internet to share their cancer journey with the world at large and to seek out others with similar experiences. Many cancer patients have begun their own Web log, or “blog” to publicize their battle with cancer. Twitter, a mini-blogging technology that limits posts to 140 characters, has also proven to be a helpful tool for cancer patients to keep friends updated and reach out to others.