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                                                                                                                                                                  Pseudomyxoma Peritonei Prognosis

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                                                                                                                                                                  What it the prognosis for Pseudomyxoma Peritonei patients?

                                                                                                                                                                  What are the long term survival rates for PMP patients?

                                                                                                                                                                  What is the prognosis for patients diagnosed with PMP?

                                                                                                                                                                  Is there a cure for PMP?


                                                                                                                                                                  The doctor says I have "PMP." What is my prognosis?

                                                                                                                                                                  What is the prognosis for "PMP?"

                                                                                                                                                                  What are the  long term survival statistics for Pseudomyxoma Peritonei?

                                                                                                                                                                  What are my chances for long term survival?



                                                                                                                                                                  Pseudomyxoma Peritonei syndrome generally originates with the appendix, including appendix carcinoid tumors. The prognosis for patients diagnosed with appendix carcinoid tumors is generally very good with early detection and appropriate medical treatment.

                                                                                                                                                                  Appendix carcinoid tumors, located at the tip of the appendix, of less than two centimeters, generally have a low risk of spreading to the lymph nodes.

                                                                                                                                                                  Many factors contribute to the prognosis and long term survival rates. These factors may include pathology, staging at the time of diagnosis, overall health of the patient at the time of the diagnosis, age at the time of diagnosis,  access to specialized care, etc.

                                                                                                                                                                  In general, prognosis and long term survival have improved during the past decade, with earlier detection,
                                                                                                                                                                  enhanced education of healthcare providers, including radiologists and pathologists,
                                                                                                                                                                  followed by referrals to specialists
                                                                                                                                                                  and improved methods of treatment.

                                                                                                                                                                  Long term survival may increase with early and accurate diagnosis, overall health of the patient at the time of diagnosis, the actual pathology/diagnosis itself, and appropriate treatment provided by a specialist.

                                                                                                                                                                  Patients are reminded not to accept the original dismal prognosis that may initially be offered by a non specialist.

                                                                                                                                                                  Each case is unique and each patient is an individual. Published statistics do not reflect the unique case of a newly diagnosed patient, whose options for long term survival are continually improving through modern medical treatment.



                                                                                                                                                                  Prognosis and Long Term Survival Articles

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                                                                                                                                                                  Here are a selection of medical journal excerpts and reports providing specific details about the prognosis and long term survival statistics for Pseudomyxoma Peritonei.


                                                                                                                                                                  CRS and HIPEC offer longterm survival rates for Peritoneal Carcinomatosis of disseminated Appendiceal tumor origin
                                                                                                                                                                  Source: Dr Armando Sardi, Mercy Med Center, Journal of American College of Surgeons, Sept 2009

                                                                                                                                                                  Epithelial appendiceal neoplasms: report reviews 900 cases treated at the Washington Hospital Center
                                                                                                                                                                  Source: Dr Paul H Sugarbaker, Cancer Journal, May 2009

                                                                                                                                                                  Diverticulosis patients with Appendix Cancer prognosis
                                                                                                                                                                  Source: Journal of Surgical Pathology, 2009

                                                                                                                                                                  Pseudomyxoma Peritonei and Appendix Cancer Prognosis following serial debulking
                                                                                                                                                                  Source: University Central Hospital Helsinki, Finland, June 2009

                                                                                                                                                                  Prognosis following Chemo Hyperthermic Profusion
                                                                                                                                                                  Source: Fukai School of Medicine, Japan, April 2009


                                                                                                                                                                  Appendix Cancer Prognosis for VEGF expression patients with Mucinous Adenocarcinoma
                                                                                                                                                                   

                                                                                                                                                                  Appendix Cancer Prognosis and Survival Rates
                                                                                                                                                                  Source: Dr Paul Sugarbaker, Washington Hospital Center

                                                                                                                                                                  Prognostic indicators for Peritoneal Carcinomatosis originating with gastrointestinal adenocarcinoma
                                                                                                                                                                  Source: Dr Paul Sugarbaker, 2005

                                                                                                                                                                  Appendix cancer prognosis and survival rates following surgery and IP
                                                                                                                                                                  Source: Mayo Clinic

                                                                                                                                                                  Pseudomyxoma Peritonei Long Term Survival (10 year study) 
                                                                                                                                                                  Source: Annals of Surgical Oncology, 2009, Prof David L Morris, St George Hospital,Univ of New South Wales, Sydney, AU
                                                                                                                                                                  This report describes the long-term survival in patients with pseudomyxoma peritonei treated with cytoreductive surgery and perioperative intraperitoneal chemotherapy: 10 years of experience.  This report demonstrates long-term survival outcomes, acceptable perioperative outcomes, and a learning curve associated with the treatment of patients with pseudomyxoma peritonei.

                                                                                                                                                                  Pseudomyxoma Peritonei
                                                                                                                                                                  Biological Features Are the Dominant Prognostic Determinants After Complete Cytoreduction and Hyperthermic Intraperitoneal Chemotherapy
                                                                                                                                                                  Source: Annals of Surgery, Dr Marcello Deraco, Dr Baratti, 2009

                                                                                                                                                                  Morbidity and Mortality: Study of HIPEC and CRS for Treatment of Peritoneal Carcinomatosis
                                                                                                                                                                  Source: Annals of Surgery, Feb 2009, Dr David Morris, Dr Tristan Yan

                                                                                                                                                                  Conclusions: The morbidity and mortality outcomes of CRS and HIPEC are similar to a major gastrointestinal surgery, such as a Whipple's procedure. To derive the maximal benefit of this treatment, careful patient selection with an optimal level of postoperative care must be advocated to avoid undesirable complications of this treatment.

                                                                                                                                                                  DPAM: Peritonectomy Improves Survival 
                                                                                                                                                                  Source Prof David L Morris, 2009

                                                                                                                                                                  12 Year Study of CRS and HIPEC for Pseudomyxoma Peritonei Treatment
                                                                                                                                                                  Source: In Vivo, 2009


                                                                                                                                                                  DPAM Long Term Survival
                                                                                                                                                                  Source: Dr BM Ronnett, Dr Paul H Sugarbaker, 2009

                                                                                                                                                                  Pseudomyxoma Peritonei Long Term Survival (10 year study) 
                                                                                                                                                                  Source: Annals of Surgical Oncology, 2009, Prof David L Morris, St George Hospital

                                                                                                                                                                  Appendiceal Mucinous Neoplasms: Study Analysis Predicting Recurrance
                                                                                                                                                                  Source: American Journal of Surgical Pathology, 2009

                                                                                                                                                                  CRS with HIPEC have improved quality and quantity of life for patients
                                                                                                                                                                  Source:Dr Brian Loggie, 2007


                                                                                                                                                                  Survival Study for Pseudomyxoma Peritonei: DPAM, PMCA, PMCA 1
                                                                                                                                                                  Source: Dr Edward Levine, American Journal of Surgical Pathology, May 2006

                                                                                                                                                                  Appendix Cancer Prognosis
                                                                                                                                                                  Source: Dr Paul Sugarbaker, Washington Hospital Center

                                                                                                                                                                  Appendiceal Adenocarcinoma: Long Term Outcome After Surgery
                                                                                                                                                                  Source: Harvard Medical School, 2004

                                                                                                                                                                  Morbidity and Mortality Study for Pseudomyxoma Peritonei following CRS and HIPEC
                                                                                                                                                                  Source: Pseudomyxoma Peritonei Center, Hampshire, June 2009
                                                                                                                                                                   
                                                                                                                                                                  Pseudomyxoma Peritonei Prognosis following serial debulking (long term study)
                                                                                                                                                                  Source: Helskinki Univ Central Hospital, Finland, June 2009


                                                                                                                                                                  Pseudomyxoma Peritonei Prognosis for VEGH  expression patients with Mucinous Adenocarcinoma of the Appendix
                                                                                                                                                                  Source: Annals of Surgical Oncology 2008


                                                                                                                                                                  Pseudomyxoma Peritonei Prognosis following Chemo Hyperthermic Profusion
                                                                                                                                                                  Source: Fukai School of Medicine, Japan, April 2009


                                                                                                                                                                  Pseudomyxoma Peritonei prognosis: biologic features affecting prognosis
                                                                                                                                                                  Source: Annals of Surgical Oncology 2009


                                                                                                                                                                  Pseudomyxoma Peritonei prognosis and survival  following surgery and HIPEC
                                                                                                                                                                  Source: Pub Med, Feb 2009


                                                                                                                                                                  Pseudomyxoma Peritonei prognosis and survival evaluating tumor markers following surgery and HIPEC
                                                                                                                                                                  Source: Annals of Surgical Oncology, 2008


                                                                                                                                                                  Pseudomyxoma Peritonei prognosis  following HIPEC treatment
                                                                                                                                                                  Source: ASCO 2007


                                                                                                                                                                  Pseudomyxoma Peritonei and Peritonei Carcinomatosis Prognosis Indicators 
                                                                                                                                                                  Source: Pub Med 2005


                                                                                                                                                                  Pseudomyxoma Peritonei Treatment: Library of Medical Publications
                                                                                                                                                                  Source: Pub Med 2009


                                                                                                                                                                  Appendix Cancer Library including Pseudomyxoma Peritonei Treatment and Prognosis: Multiple Medical Publications
                                                                                                                                                                  Source: Pub Med 2009


                                                                                                                                                                  Analysis of 46 Pseudomyxoma Peritonei patients' prognosis following "second look" surgery
                                                                                                                                                                  Source: Dr Paul Sugarbaker 2000



                                                                                                                                                                  Prognostic Factors and Quality of Life

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                                                                                                                                                                  Toward curative treatment of peritoneal carcinomatosis from nonovarian origin by cytoreductive surgery combined with perioperative intraperitoneal chemotherapy: a multi-institutional study of 1290 patients.

                                                                                                                                                                  Glehen O, Gilly FN, Boutitie F, Bereder JM, Quenet F, Sideris L, Mansvelt B, Lorimier G, Msika S, Elias D; the French Surgical Association.Cancer. 2010 Aug 24. [Epub ahead of print]PMID: 20737573
                                                                                                                                                                  [PubMed - as supplied by publisher]

                                                                                                                                                                  Related citationsSecondary Cytoreduction and Perioperative Intraperitoneal Chemotherapy after Initial Debulking of Pseudomyxoma Peritonei: A Study of Timing and the Impact of Malignant Dedifferentiation.

                                                                                                                                                                  Chua TC, Al-Zahrani A, Saxena A, Liauw W, Zhao J, Morris DL.J Am Coll Surg. 2010 Aug 20. [Epub ahead of print]PMID: 20729102
                                                                                                                                                                  [PubMed - as supplied by publisher]Related citations

                                                                                                                                                                  [Prospective study of quality of life after cytoreductive surgery and hyperthermic intraperitoneal chemotherapy using oxaliplatin for peritoneal carcinomatosis.]

                                                                                                                                                                  Lim C, Tordjmann D, Gornet JM, Nemeth J, Valleur P, Pocard M.Bull Cancer. 2010 Aug 6.
                                                                                                                                                                  [Epub ahead of print] French. PMID: 20693116
                                                                                                                                                                  [PubMed - as supplied by publisher]Related citations

                                                                                                                                                                  Primary peritonectomy/HIPEC for disseminated peritoneal adenomucinosis achieves much lower recurrence rates and better survival than secondary procedures.

                                                                                                                                                                  Zhu KJ, Morris DL.Surgeon. 2009 Dec;7(6):345-50.PMID: 20681377
                                                                                                                                                                  [PubMed - indexed for MEDLINE]Related citations

                                                                                                                                                                  Mucinous adenocarcinomas with intra-abdominal dissemination: a review of current therapy.

                                                                                                                                                                  Winder T, Lenz HJ.
                                                                                                                                                                  Oncologist. 2010;15(8):836-44. Epub 2010 Jul 23.PMID: 20656916

                                                                                                                                                                  [PubMed - in process]Related citations

                                                                                                                                                                  [Prognostic factors and reproductive outcomes of borderline ovarian tumors: a review of 186 patients]

                                                                                                                                                                  Tao T, Cao DY, Yang JX, Huang HF, Wu M, Pan LY, Lang JH, Guo LN, Shen K.Zhonghua Yi Xue Za Zhi. 2010 May 18;90(19):1304-8. Chinese. PMID: 20646576
                                                                                                                                                                  [PubMed - indexed for MEDLINE]

                                                                                                                                                                  Related citationsCase records of the Massachusetts General Hospital. Case 19-2010. A 35-year-old man with adenocarcinoma of the cecum.

                                                                                                                                                                  Ryan DP, Engelman JA, Ferrone CR, Sahani DV, Lisovsky M.N Engl J Med. 2010 Jun 24;362(25):2411-9. No abstract available. PMID: 20573930 [PubMed - indexed for MEDLINE]Related citations

                                                                                                                                                                  Mesenteric cyst: report of a case-resulting in pseudomyxoma peritonei.

                                                                                                                                                                  Zappa L, Sugarbaker PH.Tumori. 2010 Mar-Apr;96(2):332-5.PMID: 20572595
                                                                                                                                                                  [PubMed - indexed for MEDLINE]Related citations

                                                                                                                                                                  [Peritoneal carcinosis can have as good a prognosis as primary colonic cancer, and should be managed according to evidence-based practice]

                                                                                                                                                                  Graf W, Mahteme H.Lakartidningen. 2010 May 12-25;107(19-20):1320. Swedish. No abstract available. PMID: 20556987 [PubMed - indexed for MEDLINE]
                                                                                                                                                                  Related citations

                                                                                                                                                                   


                                                                                                                                                                  Meet Pseudomyxoma Peritonei Long Term Survivors!

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                                                                                                                                                                  Communicate with any of our Pal Mentors who have been disease free from five to twenty years!

                                                                                                                                                                  Read articles about our cancer free "Pals!"

                                                                                                                                                                  Read "Cancer Patients Can Outlive Statistics" by PMP Pals
                                                                                                                                                                  Read survival stories from our fellow "Pals!"

                                                                                                                                                                  Q&A: How are patients selected for surgery?

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                                                                                                                                                                  This question is frequently asked by newly diagnosed patients and “veteran” patients experiencing a “recurrence of disease.”

                                                                                                                                                                  Why are some patients accepted for CRS or CRS/HIPEC while others are not?

                                                                                                                                                                  Is there any truth to the rumors that surgeons “cherry pick” their patients?

                                                                                                                                                                  Why isn’t every patient a candidate for surgery?



                                                                                                                                                                  Surgeons refer to the process of evaluating a surgical candidate as “patient selection.”

                                                                                                                                                                  Many factors are taken into consideration for patient selection.

                                                                                                                                                                  The success of CRS and HIPEC may be dependent on the:

                                                                                                                                                                  patient’s overall general health,

                                                                                                                                                                  complete removal of all tumor tissue,

                                                                                                                                                                  location of tumor site(s) and

                                                                                                                                                                  type of tumor (histology and differentiation.)

                                                                                                                                                                  The following is a very general explanation of how patients are selected as surgical candidates:

                                                                                                                                                                  1. Preliminary disease criteria

                                                                                                                                                                  Patients with metastasis to the peritoneum, aka Peritoneal Carcinomatosis.

                                                                                                                                                                  Patients with disease contained within the abdomen (without metastasis outside the abdomen)

                                                                                                                                                                  2. Preliminary general health criteria for patient selection

                                                                                                                                                                  Good overall heath lacking any major co-morbid conditions*.

                                                                                                                                                                  Age (some surgeons limit the ages of patients they will accept into surgery)

                                                                                                                                                                  Mental health (coherence, ability to understand instructions, evidence of chemical dependencies, etc.)

                                                                                                                                                                  Ability to pay for surgery (adequate health insurance coverage or ability to pay out-of-pocket for medical care)

                                                                                                                                                                  3. Preliminary review of patient medical history

                                                                                                                                                                  Medical history including surgical history, if applicable, co-morbid conditions* (ie diabetes, lung or heart disease) current medications (prescribed and OTC) allergies and family history

                                                                                                                                                                  History of present illness including summary of symptoms

                                                                                                                                                                  Operative reports of previous surgeries

                                                                                                                                                                  Pathology reports

                                                                                                                                                                  Record of previous chemotherapy and radiotherapy treatments, if applicable, including dates and protocols

                                                                                                                                                                  4. Extent of disease

                                                                                                                                                                  Evaluation of CT scans to determine PCI (Peritoneal Cancer Index.)

                                                                                                                                                                  The PCI helps the surgeon to determine the extent, volume and locations of the disease.

                                                                                                                                                                  Evaluation of tumor block samples from original surgery(ies) if applicable.

                                                                                                                                                                  Laparoscopy optional

                                                                                                                                                                  Biopsy optional

                                                                                                                                                                  Evaluation of tumor markers and associated lab tests

                                                                                                                                                                  Physical examination of the patient

                                                                                                                                                                   


                                                                                                                                                                   What's the difference between Adenomucinosis and Adenocarcinoma?

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                                                                                                                                                                  A: Dr. Brigitte Ronnett: "Adenomucinosis is pathologically and prognostic ally very different from adenocarcinoma.  Adenomucinosis has very low-grade pathologic features and a better prognosis, although some patients can have more trouble with it than others.




                                                                                                                                                                  Many patients get diagnosed with "adenocarcinoma" but we would classify these cases as adenomucinosis because they have a much better prognosis (and different pathology) than what we call adenocarcinoma.  I hope more pathologists will adopt this classification system so that we can remove some of the mystery surrounding the behavior of PMP."

                                                                                                                                                                  Q: GG states: "As you know, I write the PMP Pals newsletter from the perspective of patients. For the majority of us, our understanding of this topic (appendiceal cancer and/or Pseudomyxoma Peritonei) is rather limited. However, a clear understanding appears to be limited among local physicians as well.

                                                                                                                                                                  Some patients become fearful to pursue treatment when they are given a dim prognosis from their local physicians. My goal is to encourage patients to educate themselves about their treatment options, with experienced specialists.

                                                                                                                                                                  To my understanding, the first step in battling this disease is to confirm the diagnosis, then proceed in locating a qualified and experienced physician for specific treatment."

                                                                                                                                                                  A: Dr. Brigitte Ronnett: “These are the definitions pertaining to appendiceal cancer and Pseudomyxoma Peritonei:

                                                                                                                                                                  Adenocarcinoma - malignant tumor that forms glands; this is what we refer to as the aggressive appendiceal tumors in the category PMCA in our papers.

                                                                                                                                                                  Mucinous adenocarcinoma - a subtype of adenocarcinoma in which the glands have mucinous cytoplasm, sometimes producing abundant extracellular mucin (causing confusion with PMP/DPAM)

                                                                                                                                                                  Adenocarcinoid - relatively unusual tumor, typically arising in the appendix, named for its histologic similarity to carcinoid tumor of the appendix (and other organs); combines features of carcinoid (usually goblet cell carcinoid) and adenocarcinoma; thought to possibly have behavior between benign and adenocarcinoma, but in our study we found that appendiceal adenocarcinomas with adenocarcinoid appearance usually are infiltrative and aggressive, so we consider them a variant of adenocarcinoma (they have areas that resemble goblet cell carcinoid of the appendix but also can have signet ring cells and others patterns of adenocarcinoma that are aggressive types of mucinous adenocarcinoma)

                                                                                                                                                                  Disseminated peritoneal adenomucinosis (DPAM) - term created to describe the relatively bland peritoneal mucinous tumor associated with ruptured appendiceal adenomas and PMP; we use this term to distinguish these cases from the more aggressive cases of mucinous adenocarcinoma (PMCA.)

                                                                                                                                                                  Some pathologists believe what we call DPAM should be called well differentiated mucinous carcinoma in the appendix and peritoneum in PMP cases but this makes the process sound like the other categories of mucinous carcinoma (PMCA and PMCA with intermediate features) and causes confusion; what we call well differentiated mucinous carcinoma (PMCA with intermediate features) is different from DPAM, although these two types can be more difficult to distinguish, and we have shown that DPAM has a better prognosis than the intermediate form of PMCA, warranting separated designation.

                                                                                                                                                                  I think DPAM is the "true" PMP.”


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                                                                                                                                                                  Copyright 2011 by PMP Pals' Network. All rights reserved.Updated 09.20.11

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                                                                                                                                                                  Copyright (c) 2012 PMP Pals' Network. All rights reserved. Website design by PMP Pals' Publishing. Information on this website is not intended as a substitute for licensed, professional medical advice. Each case is unique. Patients should seek the counsel of their own licensed, healthcare professional(s.)