The Appendix Cancer / Pseudomyxoma Peritonei Research Foundation (“ACPMP”) was proud to be the gold sponsor of the 10th International Congress on Peritoneal Surface Malignancies from November 17th through 19th (“PSOGI 2016”) in Washington, DC – building upon ACPMP’s participation in the 2010 Congress in Uppsala, the 2012 Congress in Berlin, and the 2014 Congress in Amsterdam.
We provided scholarships to 19 participants from 13 countries, enabling them to attend the conference from around the world - including attendees from the United States, Lithuania, Pakistan, India, Brazil, Korea and Montreal. ACPMP worked with Dr. Sugarbaker and the planning PSOGI 2016 planning committee to make these opportunities available, and reviewed scholarship applications to award these dedicated individuals who would otherwise not have been able to attend the conference. We spent a good amount of time talking to the scholarship recipients, many of whom may be the next generation of clinicians and researchers.
We have been involved in several of these meetings over the years and were once again overwhelmed by the passion the assembled medical and scientific professionals bring to the challenge of improving the treatment and finding a cure for peritoneal surface malignancies (PSMs). The level of enthusiasm that was on display in the morning (before 7am some days) was equaled by the camaraderie and sharing of views in the evenings.
One highlight was the main banquet for the event which included the first screening of a special video highlighting the history of HIPEC and the progress that has been made in the past 30 years in treating PSMs. Special lifetime achievement awards were also presented to pioneers Frans Zoetmulder of the Netherlands and Francois Gilly of France (pictured) for their groundbreaking work in the treatment of PSMs, including being early adopters and supporters of the use of HIPEC in the face of much disagreement and scrutiny. Another highlight of the dinner was recognition of the first graduates of the European School of Peritoneal Surface Oncology, a joint venture between PSOGI and the European Society of Surgical Oncology (ESSO) that was created to provide structured training in the management of peritoneal cancers and certification that graduates of the program are well schooled in the science and clinical practices associated with treating PSMs. The school and these first certificates (awarded to ten individuals who completed the two-year program) were the culmination of years of work and persistence by Dr. Marcello Deraco of Italy and Dr. Santiago Gonzalez Moreno of Spain to create such a structured program.
For patient advocates, ACPMP hosted a dinner that brought together representatives from ACPMP, PMP Pals, and Pseudomyxoma Survivor as well as Laurie Todd, the Insurance Warrior. It was great fun getting this group together in person as so many of us have corresponded online over the years.
**A special thank you to ACPMP Vice President and board member Therese Surges and ACPMP volunteer Lynda Meador for attending the PSOGI event sessions and reporting the following highlights from the program!
- The congress included 680 participants from 54 countries, with over 270 abstract posters on display.
- Presentations included confirmation that cytoreductive surgery + HIPEC is currently the Standard of Care for appendix cancer and peritoneal mesothelioma.
- A number of clinical trials are ongoing throughout the world to determine best treatments for peritoneal surface malignancies from colorectal, ovarian, and gastric cancers with or without HIPEC. For example:
- Wake Forest will soon publish results of their trial for best HIPEC efficacy between mitomycin C and oxaliplatin.
- Memorial Sloan Kettering is continuing its Intraperitoneal Chemotherapy After cytoReductive Surgery (ICARUS) trial, which compares outcomes for patients who receive treatment with HIPEC (hyperthermic intraperitoneal chemotherapy) versus EPIC (early post-operative intraperitoneal chemotherapy). It was noted that MSK overestimated the number of patients eligible for the trial and has now added other institutions to collaborate and attempt to increase the number of trial participants.
- Although differences among the experts regarding the proper definition of PMP persist, generally it is considered a low-grade tumor that produces mucin resulting in “jelly belly” effect.
- Using CT and tumor markers in order to watch and wait for low-grade PMP is often considered to be a reasonable approach because the low-grade form of the disease is typically slow-growing.
- Maximum debulking without complete tumor removal can still result in long term survival for patients for whom complete tumor removal is not possible.
- Typically, specialists will only remove organs impacted by disease when treating PMP.
- Even with a high Peritoneal Cancer Index (PCI) [i.e., significant disease spread throughout the abdominal cavity], complete cytoreductive surgery (CRS) may be possible for PMP patients with good chance for full cure.
- Studies are in progress to determine ways to dissolve and thin the mucus associated with PMP, which may improve treatment options, including palliative options for non-surgical candidates. Myrtol is being studied along with Dr. Morris’s research on the mucin reducing potential of bromelain. [See details on this research and more at ACPMP’s extensive research library.]
- In low-grade PMP, there is little cellularity in the mucin and it’s hard to find cells. In PMP, there are a low number of mutations per tumor, but there are mutations and expressions in PMP that influence how aggressive it can be.
- The Completeness of Cytoreduction (“CC”) Score measures the degree to which visible disease is removed in cytoreductive surgery. Per Drs. Sugarbaker and Moran, it is impossible to achieve a score of CC-0 (no peritoneal seeding visualized) in cytoreductive surgery for PMP. The best score truly achievable is CC-1 (only nodules of less than 2.5 cm persist after surgery).
- Approximately one-third of PMP patients treated surgically will need a second operation. There is still some debate among experts as to the role of so-called “second look” surgery, even in the absence of evidence of disease through imaging or increased tumor markers.
- The existence of a mucinous tumor of the appendix does not necessarily mean a patient has PMP. In addition, adenocarcinoma of the appendix does not necessarily mean a patient has PMP.
- Appendix cancer pathology exists on a tumor behavioral continuum from low to high grade. The grade can be determined by sending tumor samples to a pathologist with expertise in appendix cancer.
Peritoneal Surface Malignancy Registries:
- Cancer registries are systematic collections of data about tumors, including information about patients, diagnosis, treatment and status. Registries make it possible for researchers and surgeons to examine what has happened before in order to assist with decision making now.
- In the U.S., laws such the Healthcare Insurance Portability and Accountability Act (HIPAA) make collection and sharing of data more difficult, but these registries can be very helpful sources of consolidated information!
- The Dutch registry is the largest on PSMs, but they are usually captured as metastases from some other primary tumor site. If PSMs were counted as a separate cancer from the primary tumor site, it would rank as #5 in the top 10 cancers of males and females.
- Contrast enhanced CT scan is the principal method used for identification and surveillance of PSMs. However, some experts (e.g., the French and Christies in the UK) prefer T2-MRI imaging. In order for MRI to be used effectively to screen for PSMs, specific preparation methods must be used and a qualified radiologist must be trained to interpret the scans. The cost of MRI scans in the United States is also higher than CT, which likely has affected the adoption of MRI in the U.S.
- With CT, knowing how to interpret the scan is critical. Some expert PSM specialists review the scans themselves or have local radiologists trained in PSMs who are able to look critically at the diseases’ favorite spots.