I have appendix cancer and chemotherapy won’t work. Do I have other options?

A few months ago I began having abdominal pain and losing weight. It was finally determined that I had cancer of the appendix that has spread along the lining of the abdomen. I saw a surgeon who was curt and did not look at me. He said that there is nothing that can be surgically done because of the way the cancer spread along the lining. He sent me to an oncologist who was little bit better but also told me straight out that chemotherapy does not work well in this disease. I’m young, physically active, and I and my wife went to do everything possible. Do we have other options?

First of all, I’m sorry that you had this experience. I am sure that the physicians with whom you dealt with were not mean, they simply did not know what to do. Some doctors become cold and distant when they feel impotent and unable to help. Unfortunately, medical schools do not prepare doctors adequately on how to break bad news and how to interact with people often are in a very vulnerable situation.

There are definitely other options. One of them that is worth looking into is Heated intraperitoneal Chemotherapy. It remains controversial despite a consensus statement but many oncologists feel strongly positive about it. I would put it this way: in a situation of far advanced cancer that spread along the abdominal lining, this technique provides hope for a cure or significant relegation of life. At one point in my career I practiced institution that offered this option and had seen some very good results, as well as, unfortunately, cases where it did not work.

Gastrointestinal and gynecologic malignancies frequently disseminate to the peritoneal surfaces, the lining of the abdomen. Because in many such cases there are no metastases outside of the belly, adequate treatment of disease would improve the survival. It would only do so if dissemination did not occur elsewhere in the body; therefore, the first step is to ensure that the diseases localized. Even for patients with distant metastases, eradication of cancer on abdominal and pelvic surfaces would result in a quality of life advantage because intestinal obstructions would occur much less frequently. However in such a case one has to consider whether the aggressiveness of the approach is warranted.

The method pioneered by Dr. Sugarbaker is to treat the peritoneal surface component of these malignancies with heated intraoperative intraperitoneal chemotherapy. Heat synergizes the cytotoxic effects of chemotherapy. In addition, heat increases the penetration of chemotherapy solution into cancer nodules. To improve penetration of tumor nodules by chemotherapy, the size of tumor nodules is maximally cytoreduced before the heated chemotherapy treatments.  Dr. Sugarbaker has been pioneering more and more aggressive surgical approaches over the past two decades. There is a great deal of information on these approaches. The literature contains nine international studies, an international registry of 506 patients from 28 institutions, and one single-institution Phase III randomized study with 105 patients. The nine studies showed a three-year survival rate of 25% to 58%, and a five-year survival rate of 11% to 32%.

The registry showed an overall median survival rate(how many were alive) of 19.2 months, with a three-year survival rate of 39% and a five-year survival rate of 19%. Of course these are the patients that were very motivated and many of them sought this therapy out but these are still very impressive results in this disease. In the Phase III study, the median survival rate for the chemotherapy-arm was 12.6 months vs 22.3 months for the patients in the HIPEC arm.

A consensus statement signed by the Peritoneal Surface Malignancies Group (PSMG), a collection of 72 national and international surgical oncologists from 14 countries who perform this procedure, concluded that delivering heated chemotherapy into the peritoneal cavity following cytoreductive surgery may significantly increase the life expectancy of some Stage IV colorectal cancer patients. This is a carefully phrased statement, as professionals should write, but significant and important. However this recommendation was made by a selected group of the proponents of this therapy and it has not been widely accepted among those who did not perform this procedures. These are complicated and difficult techniques and they require special equipment and training. Heated intraperitoneal Chemotherapy is becoming more and more available, however.

There are a number of trials with different drugs that are ongoing or completed and awaiting publication. One such trial is:

Sponsor:

Institut Gustave Roussy

Information provided by:

National Cancer Institute (NCI)

ClinicalTrials.gov Identifier:

NCT00006112

In summary, the technique is considered by some to be experimental, but some experts disagree. If this very reasonable for those that want aggressive treatment to seek it out.

Intravenous chemotherapy is much easier and also reasonable but does not offer you the kind of results that you are looking for. Appendiceal cancer is usually treated with colon cancer chemotherapy as the apppendix is a part of colon.  The most common chemotherapy regimens are Folfox or Xelox with or without Avastin, which is how colon cancer is treated. There are very few studies of chemotherapy specifically for this rare cancer subtype of appendiceal cancer. To give you a sense how rare it is, from 1973 to 1998, 2117 primary malignant tumors of the appendix were reported to the SEER program. We do know that the prognosis depends on histology.  I have not been able to find trials specifically for systemic chemotherapy in this rare disease.

Watson, Paul Advanced Colorectal Cancer: Consensus Group Recommends Heated Chemotherapy after Surgery, but Other Experts Disagree, Citing Need for Phase III Data. Oncology Times. 29(2):24,27-29, January 25, 2007.

nccn.org, colorectal cancer

O. Anthony Stuart, BS, Arvil D. Stephens, BS, Laura Welch, MD and Paul H. Sugarbaker, MD Safety Monitoring of the Coliseum Technique for Heated Intraoperative Intraperitoneal Chemotherapy With Mitomycin C Annals of Surgical Oncology 9:186-191 (2002)

NHS Guidelines – http://www.nice.org.uk/pdf/ip/IPG116guidance.pdf

Esquivel, Jesus (2010). “Technology of Hyperthermic Intraperitoneal Chemotherapy in the United States, Europe, China, Japan and Korea”. In Vincent T. DeVita Jr., Theodore S. Lawrence, Steven A. Rosenberg. Cancer: Principles & Practice of Oncology—Annual Advances in Oncology, Volume 1. Lippincott Williams & Wilkins. pp. 188–193.

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