Pseudomyxoma Peritonei Diagnosis, Pathology and Monitoring
Peritoneal Surface Malignancies: Detection and Monitoring
Frequently Asked Questions
Dr Laura Lambert Prefers CT Scans for Monitoring PSMs
Dr Laura Lambert
The PMP Pals' Network asks the following question of PSM treatment specialists:
“Do you prefer the MRI or CT scan for detecting and monitoring PSMs?
Dr Laura Lambert, provides the following response:
“This is a very important question. As surgical oncologists, we want to take only those patients who will benefit from cytoreduction into the operating room. The area in the abdomen that most often prevents us from achieving a complete cytoreduction is the mesentery of the small bowel. Unfortunately, the most challenging area to assess radiographically is the mesentery of the small bowel.
Furthermore, the most challenging tumors to treat with CRS and HIPEC are the poorly differentiated and signet ring cell types. These tumors are notoriously hard to see on either MRI or CT.
Drs. Barone and Low have shown that peritoneal surface tumors can be seen on MRI scans, and demonstrate a good degree of accuracy even with low volume tumors. However, to my knowledge, no one has clearly demonstrated that either MRI or CT is significantly better at telling us who we should not take to the operating room.
Additionally, it has not been shown that either CT or MRI is significantly better than the other at detecting a lower volume of disease at an earlier stage such that it impacts the overall outcome.
The MRI scan is more expensive and requires greater expertise at interpretation. It is more susceptible to motion artifact (blurring of the images by movement of the bowel or person). The MRI scan itself takes longer; many people are not comfortable in the MRI scanner.
CT scans are less expensive, quicker and most surgeons are very comfortable reading them – often times even better than the radiologist since we know what things look like in real life as well. The downside to CT scans is clearly the radiation, especially for young people who are concerned about the number of CT scans that they will need over their lifetime.
I am not opposed to using MRIs, but for right now I still prefer CT scans. I try to minimize the number that I order, taking into account an individual person's age, tumor type, clinical situation, logistical situation and preferences.”
Copyright 2013 © by PMP Pals’ Publishing and Dr. Laura Lambert. All rights reserved. Todos derechos reservados.
Dr Vadim Gushchin Considers MRI vs CT Scans
Dr Vadim Gushchin
Dr Vadim Gushchin, Mercy Healthcare, Baltimore MD responds to the PMP Pals’ Network:
“It is difficult to make a “one fits all” recommendation. It is much more common to see a good quality CT than a good MRI without motion and other artifacts.
Typically, a radiation exposure concern is valid but overstated. While ordering a follow-up study we tailor it to the individual patient, which in real life translates into ordering CT scans more frequently than MRIs.”
Articles posted in PMP Pals and on www.pmppals.org are written from the perspective of patients and their family caregivers and are not intended to substitute for licensed, professional legal or medical advice. Individual should seek counsel from licensed professionals regarding their specific needs.
Copyright © 2013 by PMP Pals' Network/All rights reserved. Visit us on the web at www.pmppals.org
Dr Kiran Turaga Considers MRI vs CT Scans
Dr Kiran Turaga
Dr Kiran Turaga is a surgical oncologist and an assistant professor at the Froedtert, Medical College of Wisconsin Cancer Center.
Dr Turaga specializes in the treatment of PSMs and is an experienced HIPEC treatment provider.
Dr Turaga tells the PMP Pals' Network:
"We use both technologies extensively and have developed some special protocols using MRI since we are a research facility. However, I would tell patients that sometimes the use is defined by the nature of disease/histology etc. Both have advantages and both miss detection of peritoneal disease, so a lot of it depends on the clinical picture."
Dr Brian Loggie Prefers MRI Scans to Assess PMP
Dr Brian Loggie
About Dr Brian W Loggie:
Dr Loggie is a surgical oncologist specializing in the research and treatment of Peritoneal Carcinomatosis and Peritoneal Surface Malignancies (PSMs) including malignant ascites, peritoneal mesothelioma, and, Pseudomyxoma Peritonei, as well as colorectal cancers.
During a recent interview with the PMP Pals’ Network, Dr Loggie responded to the article titled "MRI vs. Tumor Markers for Monitoring Appendiceal Cancer “ posted in the April 2 issue of “PMP Pals.”
Dr Loggie shares his opinion follows:
“Some of the advantages that I perceive for utilizing MRI scans to assess and monitor Pseudomyxoma Peritonei are:
MRI scans are clearly more sensitive in detecting for PMP than are CT scans. The mucin associated with PMP has high water content and also restricts movement of water molecules which is very good for MRI.
I used CT scans for years and am comfortable reading in reading them. I do depend on a very good radiologist for the most accurate MRI interpretation. At Alegent Creighton, have developed our own scanning algorithms to make the processing more time efficient.
There is no radiation exposure for MRI. Depending on the use of contrast and scanning algorithms, patient can get the equivalent of 8 to 30 Chest X-rays with 1 CT! Many of our patients are young and will be scanned for many years. Radiation exposure is cumulative.
Generally, no oral contrast is needed for an MRI. Our patients like this!
The disadvantages for MRI are:
Sometimes MRI scans are too sensitive. However, I would rather have this as a problem, than overlooking a possible problem. I do think it is important for the radiologist and surgeon to collaborate. This enhances the quality of interpretation, in my opinion. Additionally for patients with a lot of bulky disease, MRI sensitivity may not be an advantage [nor is it a disadvantage].
In some institutions MRI scans costs more. I can’t address the costs at other institutions but I can work from within my own institution to attempt to keep the costs reasonable here. MRI scans take more time than CT, which can contribute to cost. Costs will decrease over time as technology evolves and use increases.
MRI scans cannot be used for patients with certain pacemakers or implants because it uses a very strong magnet.
It is very different technology than CT and different machines have different electronic modes making this a much more complex area, hence the dependence on a radiologist. CT scans technology is simpler and more standardized. Not all radiologists or surgeons have the expertise to interpret an MRI. I do think there is a steep learning curve for surgeon and radiologist compared with CT, in the specifically regarding the assessment of PSMs.
MRI is continuing to evolve. We have begun a translational research project at Creighton that we think will lead to improved sensitivity and specificity for MRI scans for PMP. This is very exciting. Currently we are recommending MRI as our preferred modality for monitoring Pseudomyxoma Peritonei cases.
Lastly, in our practice, we see many patients referred to us with a PET or PET/CT scan. For low or intermediate grade PMP, PET is just not sensitive and is generally not useful. We occasionally use PET for high grade [signet cell] variants, but this is another topic!”
Copyright 2013 © by PMP Pals’ Publishing and Dr. Brian Loggie. All rights reserved. Todos derechos reservados.
MRI May Accurately Predict PCI for CRS
Dr Robert M Barone
Combined Diffusion-Weighted and Gadolinium-Enhanced MRI Can Accurately Predict the Peritoneal Cancer Index Preoperatively in Patients Being Considered for Cytoreductive Surgical Procedures
Russell N. Low MD, Robert M. Barone MD
Regional Cancer Therapies
Volume 19, Issue 5 / May , 2012
Abstract
Purpose
To determine whether abdominal and pelvic magnetic resonance imaging (MRI) with diffusion-weighted and dynamic gadolinium-enhanced imaging can be used to accurately calculate the peritoneal cancer index (PCI) before surgery compared to the PCI tabulated at surgery.
Methods
Thirty-three patients underwent preoperative MRI followed by cytoreductive surgery for primary tumors of the appendix (n = 25), ovary (n = 5), colon (n = 2), and mesothelioma (n = 1). MRIs were retrospectively reviewed to determine the MRI PCI. These scores were then compared to PCI tabulated at surgery. Patients were categorized as having small-volume tumors (PCI 0–9), moderate-volume tumors (PCI 10–20), and large-volume tumors (PCI > 20). The respective anatomic site scores for both MRI and surgery were compared.
Results
There was no significant difference between the MRI PCI and surgical PCI for the 33 patients (P = 0.12). MRI correctly predicted the PCI category in 29 (0.88) of 33 patients. Compared to surgical findings, MRI correctly predicted small-volume tumor in 6 of 7 patients, moderate-volume tumor in 3 of 4 patients, and large-volume tumor in 20 of 22 patients. MRI and surgical PCI scores were identical in 8 patients (24%). A difference of <5 was noted in 16 patients (49%) and of 5–10 in 9 patients (27%). Compared to surgical-site findings, MRI depicted 258 truly positive sites of peritoneal tumor, 35 falsely negative sites, 35 falsely positive sites, and 101 truly negative sites, with a corresponding sensitivity of 0.88, specificity of 0.74, and accuracy of 0.84.
Conclusions
Combined diffusion-weighted and gadolinium-enhanced peritoneal MRI accurately predicts the PCI before surgery in patients undergoing evaluation for cytoreductive surgery.
MRI vs Tumor Markers for Detecting Appendiceal Cancer Recurrence
Dr Russell N Low
Surveillance MR Imaging is Superior to Serum Tumor Markers for Detecting Early Tumor Recurrence in Patients with Appendiceal Cancer Treated with Surgical Cytoreduction and HIPEC
Source:
· Russell N. Low MD,
· Robert M. Barone MD,
· Melissa J. Lee MS
Copyright Annals of Surgical Oncology· April 2013, Volume 20, Issue 4, pp 1074-1081
Presented at the Seventh International Symposium on Regional Cancer Therapies, Captiva, FL, February 2012.
Abstract
Background
The purpose of this study was to determine if MRI surveillance is better than serum tumor makers in detecting early recurrence in patients with mucinous appendiceal neoplasm.
Materials and Methods
A total of 50 patients with appendiceal neoplasm (DPAM 11, PMCA 39) underwent abdominal and pelvic MRI prior to surgical cytoreduction and hyperthermic intraperitoneal chemotherapy (HIPEC). Patients then entered follow-up surveillance with serial MRI every 6 months and serial laboratory studies including CA 125, CEA, and CA19-9. Written reports for surveillance MRI exams were reviewed for tumor recurrence and compared with results of serial laboratory tests. Proof of tumor recurrence was by a consensus of surgery and histopathology, as well as clinical and imaging findings on serial examinations.
Results
During surveillance tumor recurrence was documented in 30 patients (60 %) with median time to recurrence of 13 months (range 3–56 months). MRI detected recurrent tumor in 28 patients, including 11 patients with normal laboratory values (sensitivity 0.93, specificity 0.95, accuracy 0.94, PPV 0.97, and NPV 0.90). Serial laboratory values showed tumor recurrence in 14 patients (sensitivity 0.48, specificity 1.00, accuracy 0.69, PPV 1.0, and NPV 0.57). Median survival was 50 months for 11 patients with earlier MRI detection of recurrence vs 33 months for the other 19 patients with recurrence.
Conclusions
Following cytoreductive surgery and HIPEC MRI detects tumor recurrence earlier and with greater accuracy than serial tumor markers alone.
PET Scans Question for Appendix Cancer
Dr Edward A Levine
Dr Levine answers the question:
"Are PET scans helpful for monitoring Appendix Cancer?"
Use of FDG-PET imaging for patients with disseminated cancer of the appendix.
Rohani P, Scotti SD, Shen P, Stewart JH, Russell GB, Cromer M, Levine EA.
SOURCE:
Surgical Oncology Service, Department of General Surgery, Wake Forest University Baptist Medical Center, Medical Center Boulevard, Winston-Salem, NC 27157, USA.
Abstract
The goal of this study is to evaluate the use of positron emission tomography (PET) in evaluation of patients with peritoneal dissemination of carcinoma of appendiceal origin (PDA).
Thirty-three patients with PDA, who had preoperative PET or PET/CT imaging, were analyzed. Using operative, pathology, and PET +/- CT data, presence or absence of disease in each abdominal quadrant was noted and the use of 18fluoro-deoxy-glucose (FDG) PET for each quadrant was evaluated. The mean age was 52, and there were 17 males; 58 per cent had low-grade lesions.
PET was positive in only 35 per cent of cases overall (30 and 41% sensitivity for low-grade and high-grade, respectively). PET without CT sensitivity for low-grade and high-grade lesions was 21 and 8 per cent, respectively.
PET imaging has limited use for patients with PDA. We do not recommend the use of FDG-PET for patients with PDA from cancer of the appendix.
Source: Am Surg, Dec 2010
For more information about Dr Drs Levine, Perry and Shen see our HIPEC Treatment Centers page
Frequently Asked Questions for PMP Cancer Diagnosis, Pathology and Staging
How do specialists diagnose Pseudomyxoma Peritonei?
Which tests are used to diagnose Pseudomyxoma Peritonei?
How can I understand the diagnosis of Pseudomyxoma Peritonei?
How is Appendix Cancer diagnosed?
Pseudomyxoma Peritonei Pathology
How is PMP diagnosed?
Appendix cancer and Pseudomyxoma Peritonei are diagnosed via a variety of tests that may include a combination of physical symptoms CT scans, tumor markers, and pathology/histology.
The diagnosis is confirmed by pathologists after examination of actual tissue and/or mucin or fluid samples.
Accurate diagnosis of Appendix cancer and Pseudomyxoma Peritonei requires thorough sampling and investigation by experienced surgeons and pathologists.(Ludeman & Shepherd, 2005.)
Due to the rarity of diagnosis, Appendix Cancer and Pseudomyxoma Peritonei have been designated as an "orphan diseases."The average age of diagnosis for the Pseudomyxoma Peritonei syndrome is approximately 44 years of age.
As few as less than 1,000 cases are diagnosed in the United States annually, with hundreds more in Europe, Asia, Australia, Canada and Mexico. It is possible that, due to a lack of appropriate diagnostics and/or limited medical care, fewer cases are diagnosed in South America, and Africa.
CT Scans Raise Radiation Exposure Concerns
CT Scan Orders Decrease Due to Radiation Exposure Concerns
Source: American Medical News.com 06.28.12
Diagnostic Imaging Studies 1996-2010, USA
Use of Diagnostic Imaging Studies and Associated Radiation Exposure for Patients Enrolled in Large Integrated Health Care Systems, 1996-2010
Source: JAMA 06.13.12
Rebecca Smith-Bindman, MD; Diana L. Miglioretti, PhD; Eric Johnson, MS; Choonsik Lee, PhD; Heather Spencer Feigelson, PhD, MPH ; Michael Flynn, PhD; Robert T. Greenlee, PhD, MPH; Randell L. Kruger, PhD; Mark C. Hornbrook, PhD; Douglas Roblin, PhD; Leif I. Solberg, MD; Nicholas Vanneman, MA; Sheila Weinmann, PhD; Andrew E. Williams, PhD
JAMA. 2012;307(22):2400-2409. doi:10.1001/jama.2012.5960
Conclusion Within integrated health care systems, there was a large increase in the rate of advanced diagnostic imaging and associated radiation exposure between 1996 and 2010.
The use of diagnostic imaging in the Medicare population has increased significantly over the last 2 decades, particularly using expensive new technologies such as computed tomography (CT), magnetic resonance imaging (MRI), and nuclear medicine positron emission tomography (PET).
Questions to Ask Your Doctor Regarding Scans
Diagnostic Scans for Pseudomyxoma Peritonei
Several diagnostic tools, including scans and laboratory tests, are utilized to detect and monitor cancer. Among the tools most commonly used are those that utilize imaging techniques. Physicians use X rays, CT Scans, MRIs, Nuclear Scanning, PET Scans and Ultrasonography as tools for diagnosing cancer and metastasis.
In general, CT scans are most commonly ordered for Pseudomyxoma Peritonei patients, however, MRI scans are now being considered for pre and post treatment monitoring. Consult with your physician about which type of scan is most appropriate for your particular medical care.
Prior to the scheduled day of the scan, ask your physician or radiologist the following questions:
Why is this scan being ordered?
Are any risks associated with this test?
Will the test be taken on an outpatient basis?
What “preparations” if any, are required prior or following this test? Fasting? Bowel cleansing?
Can I drive myself home following the test?
How much will the test cost?
Is the cost covered by my healthcare insurance?
Will the test be performed locally?
Who will administer the test?
When will the results be available to me?
What will I feel during the test?
Is the test uncomfortable in any way?
Will I feel claustrophobic in taking this test?
Can I take this test if I have a pacemaker, implants or hearing aids?
How long does the test take?
Does the test have any side effects?
How often should a patient be scanned?
General guidelines differ for American patients, who are generally monitored (including scans) quarterly during their first year, post treatment, as compared with patients in Australia, Canada and Europe. These differences in schedules may possibly be based on individual healthcare (insurance) plans.
Due to recent concerns about radiation exposure from CT scans, some patients have opted to reduce the number of scans during their first and subsequent years post treatment. These concerns are best discussed with your radiologist and/or surgical oncologist.
Regardless of your testing schedule, be cognizant of any changes in physical symptoms, i.e., bloating, unexplained pain, indigestion, elimination, etc.
Generally, if you remain disease free, your monitoring schedule will be reduced to annual tests, years five through ten, post treatment. After the tenth year, annual monitoring is often discontinued.
Once again, each patient’s case differs; therefore, it is necessary to address questions about your needs for scans to your surgical oncologist specialist.
Articles posted in PMP Pals and on www.pmppals.org are written from the perspectives of patients and their families and are not intended to substitute for licensed, professional legal or medical advice. Each patient is unique and should seek specific counsel from their own licensed healthcare professional. Copyright © 2012 by PMP Pals’ Publishing. All rights reserved.
Several diagnostic tools, including scans and laboratory tests, are utilized to detect and monitor cancer. Among the tools most commonly used are those that utilize imaging techniques. Physicians use X rays, CT Scans, MRIs, Nuclear Scanning, PET Scans and Ultrasonography as tools for diagnosing cancer and metastasis.
In general, CT scans are most commonly ordered for Pseudomyxoma Peritonei patients, however, MRI scans are now being considered for pre and post treatment monitoring. Consult with your physician about which type of scan is most appropriate for your particular medical care.
Prior to the scheduled day of the scan, ask your physician or radiologist the following questions:
Why is this scan being ordered?
Are any risks associated with this test?
Will the test be taken on an outpatient basis?
What “preparations” if any, are required prior or following this test? Fasting? Bowel cleansing?
Can I drive myself home following the test?
How much will the test cost?
Is the cost covered by my healthcare insurance?
Will the test be performed locally?
Who will administer the test?
When will the results be available to me?
What will I feel during the test?
Is the test uncomfortable in any way?
Will I feel claustrophobic in taking this test?
Can I take this test if I have a pacemaker, implants or hearing aids?
How long does the test take?
Does the test have any side effects?
How often should a patient be scanned?
General guidelines differ for American patients, who are generally monitored (including scans) quarterly during their first year, post treatment, as compared with patients in Australia, Canada and Europe. These differences in schedules may possibly be based on individual healthcare (insurance) plans.
Due to recent concerns about radiation exposure from CT scans, some patients have opted to reduce the number of scans during their first and subsequent years post treatment. These concerns are best discussed with your radiologist and/or surgical oncologist.
Regardless of your testing schedule, be cognizant of any changes in physical symptoms, i.e., bloating, unexplained pain, indigestion, elimination, etc.
Generally, if you remain disease free, your monitoring schedule will be reduced to annual tests, years five through ten, post treatment. After the tenth year, annual monitoring is often discontinued.
Once again, each patient’s case differs; therefore, it is necessary to address questions about your needs for scans to your surgical oncologist specialist.
Articles posted in PMP Pals and on www.pmppals.org are written from the perspectives of patients and their families and are not intended to substitute for licensed, professional legal or medical advice. Each patient is unique and should seek specific counsel from their own licensed healthcare professional. Copyright © 2012 by PMP Pals’ Publishing. All rights reserved.
Radiation Risk Articles of Interest
Australians examine possible links between cancer and exposure from CT Scans
Source: The World Today, March, 2010
Radiation Risks from Multiple Imaging
Source: Web MD, March 2010
What are the radiation exposure risks from CT scans?
Source: ABC TV News, January 2010
Multiple CT Scans Pose Risks
Source: Web MD, March 2009
Markers May Predict Survival
Inflammatory markers in blood and serum tumor markers predict survival in patients with epithelial Appendiceal Neoplasms undergoing surgical cytoreduction (CRS) and intraperitonealchemotherapy.
Chua TC, Chong CH, Liauw W, Zhao J, Morris DL.
Source
Ann Surg. 2012 Aug;256(2):342-9.
*Hepatobiliary and Surgical Oncology Unit, UNSW Department of Surgery †Cancer Care Centre, Department of Medical Oncology, St George Hospital, Sydney, Australia ‡St George Clinical School, University of New South Wales, Sydney, New South Wales, Australia.
Abstract
BACKGROUND:
The study examines the role inflammatory and tumor markers as biomarkers to preoperatively predict outcome in patients with epithelial appendiceal neoplasm undergoing cytoreduction and intraperitoneal chemotherapy.
METHODS:
Associations between baseline variables, tumor markers [CEA (carcinoembyronic antigen], CA125, CA199), inflammatory markers including neutrophils-lymphocyte ratio (NLR), platelet-lymphocyte ratio (PLR), and C-reactive protein (CRP) with progression-free survival (PFS) and overall survival (OS) were examined in patients undergoing surgical cytoreduction and intraperitoneal chemotherapy for epithelial appendicealneoplasm.
RESULTS:
A total of 174 patients with epithelial appendiceal neoplasm (low-grade pseudomyxoma, n = 117; appendiceal cancer, n = 57) underwent cytoreduction (CRS.) On univariate analysis, all 3 inflammatory and tumor markers predicted for both PFS and OS, respectively; NLR ≤ 2.6 (P = 0.01, P = 0.002), PLR ≤ 166 (P = 0.006, P = 0.016), CRP ≤ 12.5 (P = 0.001, P = 0.008), CEA (P < 0.001, P = 0.001), CA125 (P = 0.004, P < 0.001), CA199 (P < 0.001, P < 0.001). On multivariate analysis, there were no independent predictors of OS. PFS was independently associated with the presence of lymph node metastasis (P = 0.02), CA199 > 37 (P = 0.003), and a CRP > 12.5 (P = 0.013). A higher peritoneal cancer index (PCI > 24) was associated with elevation in CEA > 12, CA125 > 39, CA199 > 37, PLR > 166 and CRP > 12. The tumor histologic subtype was associated with CA 199 levels.
CONCLUSIONS:
The results from this investigation suggest that preoperative inflammatory markers in blood and serologic tumor markers may predict outcomes and are associated with tumor biology in patients with epithelial appendiceal neoplasm undergoing cytoreduction and intraperitonealchemotherapy treatment.
PMID:22750758[PubMed - in process]
Copyright © August 2012 by Pub Med
Stay updated about Pseudomyxoma Peritonei Diagnosis
Question of the Week from the PMP Pals' Emailbox!
Is there a test to determine whether my children will have PMP?
Q: TZ from the USA asks:
“I have been diagnosed with Appendix Cancer and PMP. My adult children want to be tested to determine whether they might have appendix cancer or Pseudomyxoma Peritonei in the future too.
Do tumor markers indicate a propensity for this diagnosis?
Or, are tumor markers effective for detection only after the disease as developed?”
GG responds:
“We don't have a marker that indicates a predisposition to Pseudomyxoma Peritonei.
The CEA and CA 19 9 are common markers to help detect the presences of abdominal cancer. Tumor markers are not foolproof in detecting cancers, but they are a helpful "tool", among other "tools" in diagnosing cancer.
For more information, see Appendix Cancer Staging.
TNM
“What does TNM mean?”
TNM is an abbreviation for:
Tumor
Node (lymph nodes)
Metastasis (where the cancer has spread)
Appendix Cancer Staging
"What is appendix cancer staging?
“How is appendix cancer staged?”
Appendix cancer staging describes:
Appendix cancer staging describes:
Where the cancer is located, where it has metastasized and whether it affects other organs.
Staging is determined from a series of diagnostic tests.
Staging helps physicians determine the most appropriate course of treatment.
Staging may also be used to assess the patient’s prognosis.
Definition of Cancer Staging
Source: American Joint Committee on Cancer
Q&A: What's the difference between Adenomucinosis and Adenocarcinoma
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 responds:
“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.”
Diagnostic Tests for Pseudomyxoma Peritonei and Appendix Cancer
CT Scans: What to Expect When you have a CT Scan
Source: Radiology.org
How CT scans are performed
Source: Medline
Contrasts used for CT Scans
Prevent Kidney Damage during CT Scans
Source: MedNews, July 2009
Prevention of Risks to Kidneys in Preparation for CT Scan
Source: University of Michigan, Study funded by NIH
Diagnostic Imaging in the Detection of Pseudomyxoma Peritonei originating from the Appendix
CT Images of Pseudomyxoma Peritonei
Source: MedPix
CT Diagnostics for Pseudomyxoma Peritonei
Source: Bejing 2008
Diagnostic Imaging of Pancreatic Cancer
Source: Dr Haydee Ojeda-Fournier, UCSD
For more information about scans and other tests, see our Diagnostics page
International Codes for Diseases
International Codes for Diseases Including Pseudomyxoma Peritonei
Tumors and Cysts
Tumors and cysts are generally measured and referred to using the metric system.
A cyst or tumor described as measuring "1 cm" equals approximately 3/8 of an inch.
Tumor Marker Tests
The most common tumor marker tests used to monitor Pseudomyxoma Peritonei are the CEA and the CA 19 9.Your oncologist may order additional tumor marker tests.
Tumor markers are usually not used to diagnose cancer but they may used a diagnostic tool or as a method of monitoring the success of whether or not a cancer treatment, (such as chemotherapy) is effective for the patient. Although often less expensive and less invasive than other diagnostic tests, tumor marker testing is not a substitute for other tests (ie biopsies, scans, etc.)
Tumor markers may be found in blood, tumors and other tissue, and in urine. Tumor markers (proteins) may be produced by cancer cells, or may be made by the body in response to cancer or other conditions including inflammation.
Some cancer patients do not exhibit or produce elevated levels of particular tumor markers.
Whenever possible, tumor marker tests should be prepared at the same lab, every time, using results of the same value, ie ng/mL (nanograms per milliliter) or U/mL (units/milliliter) to avoid confusion or misinterpretation of any fluctations.
The presence of tumor markers does not necessarily indicate the diagnosis of cancer. Some tumor markers are created by normal cells. Non-cancerous conditions (ie inflammation)may also cause levels of particular tumor markers to be higher than normal.
Generally, the CEA or Carcinoembryonic Antigen, is used to monitor appendix, colon,colorectal, gastric, liver, stomach and pancreatic cancer. The normal range for the CEA is 0 to 5.
The CA 19-9* is used to monitor appendix, colorectal and pancreatic cancer.
Normal blood levels of CA 19-9 are below 37 U/mL (units/milliliter)
The CA 72-4 is use to monitor gastric, pancreatic and stomach cancer.
The Epidermal Growth Factor Receptor (EGFR) is also known as HER1. It may be used to monitor colon and pancreatic cancers. An increased amount of EFGR may indicate that the cancer may grow more quickly and metastasize. Patients with elevated EGFR may require more aggressive treatment, including with drugs that block (or inhibit) the EGFR receptors.
The Alpha fetoprotein (AFP) used for liver cancer.
The CA 125 is used for epithelial ovarian cancer.
Normal blood levels are usually less than 35 U/mL (units/milliliter.)
The BTA may be used to monitor patients with bladder cancer, but may also be an indication of kidney stones or urinary tract infections.
What are Tumor Markers?
Source: Lab Tests Online
Tumor Marker Descriptions
Source: ACS
CA 19-9 Tumor Marker Test
CEA Tumor Marker Test**
Tumor Grade
“Tumor grade” describes how much the tumor appears like normal tissue when examined under a microscope. The tumor grade helps physicians predict how quickly the cancer may grow.
G1: well-differentiated tumor cells
G2: moderately differentiated tumor cells
G3: poorly differentiated tumor cells
G4: undifferentiated tumor cells
Tumor Profiling: Cell Based Oncology Assays
Exigon Diagnostics (formerly Oncotech)
Source: Exigon
Parkland OncoDiagnostic Lab personal tumor profiling
Rational Therapeutics: personal tumor profiling
(The PMP Pals' Network does not specifically endorse this or any other company. We simply provide this link for your information and for discussion with your personal healthcare provider.)
Precision Therapeutics:personal tumor profiling and analysis
The PMP Pals' Network does not specifically endorse this or any other company. We simply provide this link for your information and for discussion with your personal healthcare provider.)
Red Path: personal tumor case analysis
The PMP Pals' Network does not specifically endorse this or any other company. We simply provide this link for your information and for discussion with your personal healthcare provider.)
Additional Diagnostic Tests
Your physician may order additional or different tests based on your specific needs. Other tests may include a barium enema, colonoscopy,(see below) upper GI series or ultrasonography. However, generally these particular tests are not as effective, or may not be effective at all, in diagnosing Appendix Cancer including Pseudomyxoma Peritonei syndrome.
Ask your physician how the Peritoneal Cancer Index (scroll below) relates to your specific case.
For more detailed information regarding diagnostic tests, order the PMP Pals' Network Handbook.
Biomarkers
Personalized Biomarkers
Source: NCI Bulletin, 2010
Biomarkers, Defined
Source: Massachusetts General Hospital, Center for Biomarker Imaging
Biopsies
The Biopsy Report: Explains biopsies, pathology terminology, etc
Source: The Cancer Guide
Optical Biopsy
Optical Biopsy: Endoscopic Detection and Diagnosis
Source: Thomas D Wang, Stanford University 2004
WavStat Optical Biopsy
Source: SpectraScience, Inc, 2008
Bowel Prep for Diagnostic Testing
MoviePrep Bowel Prep
Colonoscopy
Are colonoscopies helpful in the diagnosis of Appendix Cancer and Pseudomyxoma Peritonei?
Why didn't my "clear" colonoscopy detect Pseudomyxoma Peritonei?
Colorectal Cancer Screening
Source: MD Anderson March 2010
Colonoscopy is the "gold standard" test for Colorectal CancerImproved screening techniques may be needed for Appendix Cancer
Source: Cancer Journal of Gastroenterology, Canada, 2009
Colonoscopies may not detect obstructions
Source: The Oncologist June 2009
Virtual Colonscopy
Source: Dept of Radiology State University of New York
Atlas of Colonscopy (graphic photos, excellent suggestions for patients prepping for post op endoscopy or colonoscopy)
Source: Helmet Messman and Jurgen Barnet, 2005
Atlas of Colonscopy (lumen and stoma details)
Video of tapeworm disovery during colonoscopy
Source: Symposier 2010
Researchers find new ways to detect flat polyps: VA hospital Palo Alto CA
Source: ABC News San Francisco, March 16, 2010
Patients who have colonoscopies performed by gastroenterologists may be less likely to develop colon cancer
Source: Science Daily, Feb 2010
EFGR
EFGR definition
Source: NCI
Biomarkers Predict Outcome of EFGR Targeted Therapy Colorectal Cancers
Source: Journal of the National Cancer Inst, 2009
ERCP
ERCP procedure described
Source: Medline
Genomes
International Cancer Genome Consortium
Source:ICGC November 2008
Red Path: personal tumor case analysis
The PMP Pals' Network does not specifically endorse this or any other company. We simply provide this link for your information and for discussion with your personal healthcare provider.)
Genetic Coding, Appendix Cancer
MUC2 Gene Coding for Appendix Cancer
REG4 Gene Coding
Health Insurance for Diagnostic Tests
Histoendoscopy
Histoendoscopy: Endoscopic Imaging for Colorectal Adenomas
Source: Medscape June 2009
KRAS Mutation Testing and Therapy
KRAS Mutatation Predictors and Colorectal Cancers
Source: Exiqon
Genzyme Diagnostics
Source: Genzyme
Exigon Diagnostics (formerly Oncotech)
Source: Exigon
Kras mutation testing
KRAS Mutant
Source: New England Journal of Medicine, 2009
KRAS mutant status helps predict survival of Colorectal Cancer pts treated w/Cetuximab, Folifiri, Folfox 6
Source: ESMO GI, June 2009
KRAS mutant targeted therapy
Source: Biochemical and Biophysical Research, 2009
Kras mutation testing
Parkland OncoDiagnostic Lab
Ras and Carcinogenesis
Source: Pub Med 1988
Pathogenesis
Insights into Pathogenesis of CRC: Colorectal Cancer
Source: Current Opinion in Gastroenterology,2010
Peritoneal Cancer Index
Peritoneal Cancer Index
Source: Annual of Surgical Oncology, Feb, 2009, Dr David Morris, Dr Tristan Yan
Peritoneal Cancer Index
Source: Dr Paul H Sugarbaker
Peritoneal Cancer Index
Source: ASCO 2007, Dr Jesus Esquivel
Peritoneal Cancer Index (Ovarian)
Source: Dr A Tentes, European Journal of Surgical Oncology 2003
PET Scans for Diagnosis of Pseudomyxoma Peritonei, Appendix Cancer, DPAM
PET Scans for Healthy Adults
Source: ASCO 2004
Lab Tests, Misc.
New cancer biomarkers for diagnostic testing
Source: UCSF News, June 2009
Nano signals: healthy vs cancerous cells
Source: ACOR June 2009
Interpreting Lab Test Results
Estrogen and Progestrogen receptor markers
DNA Ploidy Cell Cycle Analysis
Kidney function testing
Genetic Counseling
Genetic Counseling
Environmental/Pollution Scorecard
Q&A: How are Pseudomyxoma Peritonei Patients Selected for Surgery?
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
Here's what families say about the PMP Pals' Network!
“You are a special angel to all of these people who so desperately need help and hope. I am sure I speak for all PMP Pals in saying a special thank you for all you have done to spread the word when we are given such shocking news.
My daughter was given a death sentence when she was first diagnosed, but because of your information is still doing well. You were our salvation. We received hope and were able to fight on. I love you and your hard work that you continue to do for so many PMP Pals. I can never forget how much you gave to our family. May God bless you.” JN, USA, mother of a patient of Dr Paul Mansfield
This page is sponsored in memory of Jan Norris by her husband, Steve
Jan Norris1948-2003
Visitors to www.pmppals.org are encouraged to discuss publications and information contained herein with their licensed, professional healthcare providers.
The information provided on www.pmppals.org is not intended as a replacement for licensed, professional medical or legal advice.
Please respect your fellow patients and caregivers by not copying or cutting and pasting any pages from this website onto yours.
The PMP Pals Network is a volunteer patient advocacy program. We support the services that we provide, including this web page, as volunteers and through subscriptions to our publications.
We neither solicit nor receive funds from pharmaceutical companies or healthcare providers, thus maintaining our dedication to serving as patient advocates.
Updated 05.08.13
The information provided on www.pmppals.org is not intended as a replacement for licensed, professional medical or legal advice.
Please respect your fellow patients and caregivers by not copying or cutting and pasting any pages from this website onto yours.
The PMP Pals Network is a volunteer patient advocacy program. We support the services that we provide, including this web page, as volunteers and through subscriptions to our publications.
We neither solicit nor receive funds from pharmaceutical companies or healthcare providers, thus maintaining our dedication to serving as patient advocates.
Updated 05.08.13
