Mucinous Adenocarcinoma of the Appendix
Mucinous Adenocarcinoma of the Appendix and Pseudomyxoma Peritonei Diagnosis
Mucinous Adenocarcinoma of the Appendix is among the most common diagnoses under the "umbrella description" of Pseudomyxoma Peritonei.
The diagnosis of Peritoneal Mucinous Carcinomatosis indicates the presence of mucin accompanied by abundant malignant epithelium, high-grade atypia (Ronnett et al, 1995). Mucinous Carcinomatosis indicates a more challenging prognosis relative to the more common pattern of Disseminated Peritoneal Adenomucinosis aka DPAM.
Approximately 20% of appendix cancer cases are categorized as Mucinous Cystadenocarcinoma. This type of tumor produces mucin that eventually fills the abdominal cavity causing distension, bloating, pain, shortness of breath and interference with digestive and bowel function.
Refer to our diagnostics page for detailed information describing laboratory and imaging testing.
The PMP Pals' Network hosts the Mucinous Adenocarcinoma of the Appendix Pals' Resource Group, comprised of patients from all over the world, who share this diagnosis.
If you are a patient, review a copy of your written pathology report and check the "final paragraph" description for the term "Mucinous Adenocarcinoma" or ask your physician to review your pathology report with you. Each patient should be given a copy of his or her own written pathology report.
View "Pal Profiles" and photos of patients living successfully following the diagnosis of Mucinous Adenocarcinoma.
Subscribing members of the PMP Pals' Network programs are welcome to participate in the Mucinous Adenocarcinoma of the Appendix Resource Group.
Mucinous Adenocarcinoma of the Appendix and Pseudomyxoma Peritonei Prognosis
Many factors contribute to the prognosis of patients diagnosed with Mucinous Adenocarcinoma. 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.
If left untreated, tumor cells and mucin can increase with time, eventually resulting in bowel obstruction, when the areas of the abdominal cavity and pelvis normally utilized for nutritional function and elimination, eventually become replaced by mucinous tumors.
This Week's Q&A is between the PMP Pals' Network and pathologist, Dr Brigitte Ronnett
Q: What's the difference between Adenomucinosis and Adenocarcinoma?
A: "Adenomucinosis is pathologically and prognostically 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 surounding the behavior of PMP."
This Week's Article of Interest:
Is Appendiceal Mucinous Adenocarcinoma Hereditary?
Primary appendiceal mucinous adenocarcinoma in two first-degree relatives: case report and review.
Source: Racek AR, Rabe KG, Wick MJ, Psychogios A, Lindor NM.Department of Medical Genetics, Mayo Clinic, 200 First Street SW, Rochester, 55905, MN, USA. nlindor@mayo.edu.
ABSTRACT: Carcinomas of the appendix are exceedingly rare tumors and have an annual age-adjusted incidence of around 0.4 cases per 100,000. Appendiceal adenocarcinoma accounts for < 0.5% of all gastrointestinal neoplasms and, of these, mucinous adenocarcinomas account for the majority.
Published accounts of familial instances of primary appendiceal tumors are strikingly rare. We report two siblings who both developed primary mucinous adenocarcinomas. A genetics evaluation was conducted to determine if there was a recognizable underlying single gene disorder; no DNA mismatch repair defect was evident, and no other diagnosis was apparent.
A review of appendiceal cancers seen at Mayo Clinic from l997 to the present was conducted to search for additional familial cases. Among 316 cases of primary appendiceal cancer of any histologic type, this sib pair was the only family reporting a second affected family member. The occurrence of appendiceal cancer in siblings may represent a random occurrence.
An exceedingly rare predisposition syndrome cannot be ruled out.
Source: PMID: 21542938 [PubMed - in process] PMCID: PMC3098790
Prognosis for VEGF
Mucinous Adenocarcinoma of the Appendix: Prognosis for VEGF expression patients
Analysis of Mucinous Adencarcinoma of the Appendix prognosis following "second look" surgery.
Source: Dr Paul Sugarbaker 2000
Mucinous Adenocarcinoma of the Appendix and Pseudomyxoma Peritonei Treatment
HIPEC or Hyperthermic Intraperitoneal Chemotherapy, for the treatment of Mucinous Adenocarcinoma, may be administered if complete tumor debulking is accomplished through cytoreductive surgery.
The HIPEC treatment includes the infusion of the abdominal cavity with a specific chemotherapy, heated to approximately 40 degrees Celsius for a period of approximately 90 minutes.
The purpose of the HIPEC treatment is to attempt to kill any tumor cells which were not removed during the actual surgery process.
Refer to our extensive listing of HIPEC Treatment Centers to identify a specialist in your region.
Treatment with CRS and HIPEC
Source: Universite de Montreal, Canada, 2009
Treatment with Adjuvant Chemotherapy
Source: World Journal of Surgical Oncology, China 2008
Refer to the HIPEC page for extensive articles regarding Appendix Cancer treatment
Clinical Trials
Click here for a listing of research studies and clinical trials
Peritoneal Carcinomatosis Research, International
Review an extensive series of research articles by the world's most renowned specialists in this field:
Management of Peritoneal Carcinomatosis from Gastrointestinal Malignancies:
Research from 3rd International Symposium on Peritioneal Carcinomatosis, June 2008
Source: Prof Pompiliu Piso and Colleagues
Mucinous Adenocarcinoma of the Appendix and Pseudomyxoma Peritonei Diagnosis*
Mucinous Adenocarcinoma of the Appendix Synonyms
Mucinous Adenocarcinoma of the Appendix CT Images
Clinicopathologic Features Mucinous Adenocarcinoma of the Appendix
Diagnosed with endoscopy ultrasound
Atlas of Appendix Cancer
Source: Dr Paul H Sugarbaker
Mucinous Adenocarcinoma of the Appendix Pathlogy**
Mucinous Adenocarcinoma of the Appendix
Genetic Factors Related to Mucinous Adenocarcinoma of the Appendix
MUC2 Gene Coding
REG 4 Gene Coding
Hereditary or Familial Cancers
Hereditary Cancers including gastric and colon cancers
Source: Creighton University
Hereditary Cancers including colorectal cancers
Source: MD Anderson
Hereditary Cancers including colorectal cancers: testing, genetics and more
Source: Memorial Sloan Ketting
Mucinous Adenocarcinoma of the Appendix, Complications
Ureteric Obstruction
Complications, with renal effects
Source: Indian Journal of Pathology and Microbiology 2008
Mucinous Adenocarcinoma of the Appendix, OB/GYN
Case discovered during Cesarean section delivery
Case originating with the ovary
Source: Gynecological Oncology, Japan, 2009
Case originating with the cervix and linked with HPV
Source: Croatian Journal of Medicine, 2008
Adenocarcinoma of the Colon
HIV Associated with Adenocarcinoma of the Colon
Source: Clinical Colorectal Cancer
Mucinous Adenocarcinoma, Misc, Animal Cases
Intestinal Mucinous Adenocarcinoma with Pseudomyxoma Peritonei in a Dog
Source: Journal of the American Animal Hospital Assoc, 2003
Mucinous Adenocarinoma with Peritoneal Carcinomatosis in a Lion
Source: Springerlink, 2007
Pseudomyxoma Peritonei
Tumor Grade
Q: What does "tumor grade mean?"
“Tumor grades” distinguish tumor from what 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
See DIAGNOSTIC TESTS for more detailed information
Tumor grading and staging
Source: NIH
Caregiving
Are you a caregiver for a loved one diagnosed with Mucinous Adenocarcinoma?
We provide many helpful resources on our CAREGIVING page!
After Treatment is Completed; Now What?
Post Treatment Plan for Cancer Survivors
Source: Journey Forward
This web page has been contributed by the family and friends of Jeff Beach.
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