Pseudomyxoma Peritonei Diagnosis and Pathology
PMP Diagnosis, Pathology and Staging
Commonly asked questions...
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 actual 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 several hundred cases are diagnosed in the United States annually, with hundreds more in Europe, Asia, Australia,and Canada. It is possible that, due to a lack of appropriate diagnostics and medical care, fewer cases are diagnosed in South America, Africa and Mexico.
Diagnostic Tests for Pseudomyxoma Peritonei and Appendix Cancer
CT Scans
Several diagnostic tests are utilized to detect and monitor cancer. Among the tools most commonly used are those that utilize imaging techniques.Different scans used for different cancers.
Physicians utilize 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 and Appendix Cancer patients.
Consult with your physician about which type of scan is most appropriate for your particular medical care.
The CT (Computerized Tomography) scan is the preferred method (preferred over PET or MRI) of scanning to detect and monitor Appendix cancer, including Pseudomyxoma Peritonei.CT scans may include the administration of enteric IV ionic or non ionic contrast, oral contrast and rectal contrast. On average, after the contrasts have been administered, the patient spends approximately ten minutes being scanned. Scanned images may be transferred to CD format so that the patient may retain a personal copy of the test(s.)
Prior to the scheduled day of your scan, ask your oncologist, surgeon or radiologist the following questions:
Why is this scan being ordered?
Are any risks associated with this scan?
Where will the scan be performed?
Can I have the scan at my local scanning facility or hospital or must I travel to your clinic/hospital for the scan?
Will I be medicated for the scan?
Can I drive myself home following the scan?
How much will the scan cost?
Will my insurance pay for the scan?
When will the scan results be available to me?
Will you review the results with me, or will someone else?
May I have a digital copy (CD) of the scan?
Will I be charged for a digital copy?
Do I need to fast prior to the scan?
Will I need to drink anything special for the scan?
Will I need a bowel prep prior to the scan?
How long with the scan take?
What will I feel during the scan?
Is the test uncomfortable in any way?
Will I feel claustrophobic during the scan?
Will I experience any side effects following the scan?
Will the scan be noisy? If so, may I wear ear plugs?
Do I need to wear special clothing for the scan?
Can I take this scan if I have a pacemaker, or implants of any kind?
Do I need to remove hearing aids, jewelry or contact lenses prior to taking this scan?
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
CT Scan Radiation Risk Articles
Radiation Risks Nearly Double for Younger Patients
Source: Health Day News, May 2010
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
PET Scans
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.
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
For more information about scans and other tests, see our Diagnostics page
Cancer Staging
Definition of Cancer Staging
Source: American Joint Committee on Cancer
International Codes for Diseases
International Codes for Diseases Including Pseudomyxoma Peritonei
Tumors and CystsTumors 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.
Blood tests for early detection of GI Cancers
Source: Medscape Oncology Jan 2010
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
Biomarkers Predict Outcome of EFGR Targeted Therapy Colorectal Cancers
Source: Journal of the National Cancer Inst, 2009
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
PC's Limit Colon Cancer /Colorectal Cancer Screening Methods
Source: Reuters, July 2009
Colonoscopy is the "gold standard" test for Colorectal Cancer
Deep sedation during Colonoscopy improves cancer detection
Source: Digestive Distress Week, June 2009
CT Colonography vs Colonoscopy
Source: Reuters April 2009
"Virtual" Colonoscopy may not be covered by Medicare
Source: Medscape, May 2009
Improved 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
Click here to reference additional information about EFGR on our CHEMOTHERAPY page
Biomarkers Predict Outcome of EFGR Targeted Therapy Colorectal Cancers
Source: Journal of the National Cancer Inst, 2009
Enteroscopy
Small bowel enterscopy
Source: Medscape, June 2009
ERCP
ERCP procedure described
Source: Medline
Genomes
Genomics of Metastasis
Source: MedScape 2009
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 Oncogene Predictor of Response to Cetuximab
Source: Medscape July 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
Pathology
Armed Forces Institute of Pathology
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
Peritoneal Carcinomatosis of Unknown Primary Origin (CUP)
Peritoneal Carcinomatosis of Unknown Primary Origin (CUP)
Source: MD Anderson 2008
Lab Tests, Misc.
New cancer biomarkers for diagnostic testing
Source: UCSF News, June 2009
HER2 Overexpression in Gastric Cancer Patients
Source: ASCO 2009
Nano signals: healthy vs cancerous cells
Source: ACOR June 2009
Interpreting Lab Test Results
Estrogen and Progestrogen receptor markers
K167 MIB cell marker
DNA Ploidy Cell Cycle Analysis
Kidney function testing
Genetic Counseling
Genetic Counseling
Environmental/Pollution Scorecard
Cancer Staging
"What is appendix cancer staging?
“How is appendix cancer staged?”
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.
“What does TNM mean?”
TNM is an abbreviation for:
Tumor
Node (lymph nodes)
Metastasis (where the cancer has spread)
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: “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.”
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
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.
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
May God bless you.” JN, USA, mother of a patient of Dr Paul Mansfield
This page is sponsored in memory of Jan Norris
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