APPENDIX CANCER AND PSEUDOMYXOMA PERITONEI
When you are living with a rare disease like appendix cancer/PMP, you can feel isolated and lonely. No one around you really knows what you are going through. That’s why PMP Pals offers several programs to connect patients and caregivers to support each other and build the emotional resilience you need to meet the challenges of this disease.
Click on the links below to learn more about our great programs and to join in. And, caregivers, these programs are not just for patients, they are for you, too. A boost of peer support may be just what you need to get through a tough day.
CYTOREDUCTIVE SURGERY (CRS)
CRS is surgery to remove cancer cells. We focus here on CRS for peritoneal surface malignancies – cancers that have spread to the peritoneal cavity and to organs in that part of the body.
Peritonectomy. For appendix cancer with abdominal spread, the surgical procedure normally used to remove the diseased tissue is called a “peritonectomy.” This word refers to the removal of the peritoneum – the lining of the abdominal cavity that surrounds organs like your intestines, spleen, and ovaries and uterus. The word “peritonectomy” also can refer to the overall surgical procedure to remove one or more organs, or parts of organs, within the peritoneal cavity where appendix cancer cells and mucin are found. Because of the large surface area of the peritoneum and the organs it surrounds, peritonectomy procedures can include appendectomy (removal of appendix), hysterectomy (removal of uterus and ovaries), splenectomy (removal of spleen), “resectioning” of the small and large intestines, and other procedures involving the liver, diaphragm, gall bladder, and other abdominal parts.
Each procedure within the peritonectomy operation can be complicated and time-consuming. Performed together as part of CRS, the procedures can last more than ten hours and involve many areas of the peritoneum.
CC Score. The goal of CRS is to remove all visible tumors to eliminate all disease present and stop the progression of cancer through the abdominal cavity. The surgeon will assign a “Completeness of Cytoreduction Score” (CCS) following the surgery to indicate whether the residual tumor remains. A score of C0 indicates no residual tumor nodules; C1 indicates residual tumor nodules less than 2.5 millimeters (mm). A score of C0 or C1 indicates satisfactory cytoreduction has been achieved. A score of C2 means residual tumor nodules greater than 2.5mm but less than 2.5 centimeters (cm). And, C3 means residual tumor nodules greater than 2.5 cm remain. The score can help predict the likelihood of recurrence and the expected length of survival.
If all visible tumors cannot be removed due to risk or other factors, then the surgeon may remove as much tumor material as possible, as well as mucin that has accumulated in the abdomen. This “debulking” can have a palliative effect for the patient, relieving symptoms such as bloating, blockages, constipation, vomiting, loss of appetite, and pain. It also can reduce damage that can be done by tumor pressure on organs or blood vessels.
This is the surgical removal of the appendix. For some very small types of tumors of the appendix (less than 1.5cm), or if the tumor has not breached the wall of the organ, an appendectomy may be the only treatment that needs to be performed. However, if the appendix wall has been breached, additional procedures may be necessary, depending on the spread and growth of cancer cells and the development of mucin.
This is the removal of a part of the colon close to the appendix. A surgeon may remove nearby blood vessels and lymph nodes at the same time. A right hemicolectomy is a surgery performed on the right side of the colon. Before proceeding with a hemicolectomy, a patient should discuss whether cancer has spread to lymph nodes or the surgery is preventative, the impact on bowel function, and the possibility of tumor entrapment that could affect the success of future treatments such as HIPEC.
HIPEC - HEATED INTRAPERITONEAL CHEMOPERFUSION
HIPEC often immediately follows cytoreduction surgery before the incision is closed, either when all visible tumor has been removed or when any remaining tumor is small enough to be penetrated and destroyed by the HIPEC chemotherapy agent. HIPEC is considered a “regional” chemotherapy – that is, the chemo drug is administered directly into the body “region” affected by a metastases, rather than “systemic” – in which the chemo drug is administered intravenously or orally to circulate throughout the entire body.
EPIC, PIPAC, HITHOC – OTHER REGIONAL CHEMOTHERAPIES
EPIC is early postoperative intraperitoneal chemotherapy. It is done after cytoreduction surgery, usually starting on the first day after surgery and administered for three days, as the patient is recovering. PIPAC is Pressurized Intraperitoneal Aerosol Chemotherapy administered during a laparoscopy. HITOC is hyperthermic intraperitoneal chemoperfusion, which is used to treat PMP in the thoracic (chest) cavity. The decision to use one of these therapies instead of HIPEC may be determined based on the patient’s individual condition as well as the surgeon’s or hospital’s preferred choice of regional chemotherapy.
This word normally refers to “systemic” chemo (administered orally or intravenously to reach all parts of your body), not to “regional” chemo that is delivered in a manner to target a region of the body.
Chemotherapy uses one or more anti-cancer drugs as part of a standardized regimen.
Chemotherapy may be given with curative intent, or the goal may be to prolong life or to reduce symptoms (palliative chemotherapy). A medical oncologist provides the treatment.
Systemic chemotherapy may be used with other treatment modalities that are local — that is, the effect is confined to the anatomic area where they are applied, such as surgery, HIPEC, or radiation.
Neoadjuvant therapy is the administration of chemotherapy before the main treatment, such as before CRS. Neoadjuvant therapy is used to reduce the size or extent of cancer before surgery so that the surgical procedure will be easier and more likely to succeed, with reduction of surgical risk.
Adjuvant therapy, also known as adjunct therapy, is chemotherapy given after surgery. An example is the additional treatment usually given after surgery where all detectable disease has been removed, but where there remains a statistical risk of relapse due to the presence of undetected disease. If known disease is left behind following surgery, then further treatment is not technically adjuvant but is often referred to as such.
Radiation therapy using x-rays is commonly used for many forms of cancer but is not widely used for appendix cancer. New research is ongoing into the use of proton therapy, a type of radiation therapy using energy from positively charged particles (protons) to shrink a tumor. One benefit is a more-focused x-ray and thus less damage to surrounding tissue. However, proton therapy is not widely available in the U.S.
This is the use of drugs to stimulate the immune system to fight cancer. Cancer immunotherapy is a treatment that activates the body’s immune system to fight cancer. Immunotherapy drugs do not directly target tumors – instead, your immune system is awakened to do battle with the cancer cells. Much of the immunotherapy treatment for appendix cancer currently is done as part of clinical trials only.
Researchers are designing advanced technological tools to match each patient with the drug best suited to treat a particular tumor. Researchers are able to generate a profile of the abnormalities in a tumor’s genetic code and use it to select treatment. The goal is to tailor treatment to patients most likely to benefit from the treatments. These techniques are developed at large research institutions and are often associated with clinical trials.
Larger cancer centers, such as the NCI Comprehensive Cancer Centers, often are the venues participating in clinical trials. These trials, also referred to as “translational medicine,” bring new scientific discoveries into the realm of patient care through a rigorous, structured program of investigation. Participating patients can have early access to treatments, investigational drugs, and medical devices that could become standard care in the future. For example, appendix cancer patients hopefully watch for results from the BromAc clinical trials in Australia, investigating the use of pineapple and other compounds (Bromelain & Acetylcysteine) to thin abdominal mucin to make it easier to extract. For more information on clinical trials in the U.S., see Home – ClinicalTrials.gov
The focus of palliative care is to improve the quality of life by preventing or treating symptoms and side effects of cancer or from the treatment for cancer. This approach can also address spiritual and emotional health issues. Palliative care can help with pain management, treatment plans, counseling, caregiver support, and other quality of life measures. Unlike hospice care, a patient is not required to forego further treatment for the disease.
Hospice care is available to patients in the final stages of a terminal illness. This care offers help with pain management, symptom management, and spiritual and emotional health. Various services are provided. Normally, a patient is expected to forego further treatment that is intended to be curative.
NEUROENDOCRINE TUMORS - TREATMENT
If you have been diagnosed with a type of tumor known as neuroendocrine tumors (also referred to as “carcinoid” tumors), you should look at resources specifically focused on that form because of differences in symptoms and treatment. See, for example, Neuroendocrine Tumor of the Gastrointestinal Tract: Types of Treatment | Cancer.Net, a detailed webpage maintained by the American Society of Clinical Oncology. More information can be found at Carcinoid Tumor | Johns Hopkins Medicine.