What is the treatment of appendix cancer?
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.
Surgery For Appendix Cancer
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 spreading to surrounding lymph nodes and the liver. Removal of the right colon and regional lymph nodes is recommended. (Right Hemicolectomy)
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.
Appendix cancer has often spread to the peritoneal surfaces of the abdomen by the time it is discovered. The surgery to remove as much of the cancer in the abdomen as possible, reducing the “bulk” of the cancer is called “debulking” or “cytoreduction” surgery. “Cyto” is a word root meaning cells, so cytoreduction means to surgically “reduce” number of cancer cells. Both terms mean the same thing and are usually used interchangeably.
Cytoreduction (debulking) surgery is often long , complicated and 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). “Complete cytoreduction” or “complete debulking” means that all of the visible tumors are successfully removed during surgery. “Incomplete cytoreduction” or “incomplete debulking” means that all of the tumors could not be removed during the surgery and that visible tumors were left behind.
The factor most associated with long term survival is completeness of cytoreduction, or the removal of all visible tumors.
To destroy very small tumors and cancer cells left behind that are too small to be seen, and to prevent them from later growing into new cancerous tumors in the abdomen, the surgery can be followed by treatment with intraoperative Hyperthermic Intraperitoneal Chemotherapy or by treatment with early post-operative intraperitoneal chemotherapy (EPIC) at facilities that have the capability to provide these treatments.
These chemotherapy treatments may be effective on very tiny tumors that cannot be removed, but are not able to completely destroy larger tumors left behind.
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.
When intravenous chemotherapy (IV chemo) is indicated for the treatment of appendix cancer, the specific drugs used are usually the same as those used to treat colon cancer. Appendix cancer is so uncommon that clinical trials comparing the effectiveness of the various available chemotherapy agents have not been done. Since colon cancer and appendix cancer pathologies are similar, the effectiveness of these chemotherapy medications is assumed to be beneficial in the treatment of appendix cancer, and in many cases it has been demonstrated to be effective. Most often a combination of several drugs is used.