Pseudomyxoma peritonei
| Pseudomyxoma Peritonei | |
|---|---|
| Classification and external resources | |
| ICD-10 | C48.2 |
| ICD-9 | 197.6 |
| ICD-O: | M8480 |
| DiseasesDB | 31498 |
| eMedicine | med/1795 |
| MeSH | D011553 |
Pseudomyxoma Peritonei is an uncommon tumor known for its production of mucin in the abdominal cavity.[1] If left untreated, mucin will eventually build up to the point where it compresses vital structures: the colon, the liver, kidneys,stomach, spleen, pancreas, etc.
Unlike most cancers, this disease rarely spreads through the lymphatic system or through the bloodstream. Therefore it is characterized by mucin and scattered cancer cells in the abdominal cavity. This disease is most commonly associated with cancer of the appendix; mucinous tumors of the ovary have also been implicated, although in most cases ovarian involvement is favored to be a metastasis from an appendiceal or other gastrointestinal source. There have been efforts to rename or reclassify this as a condition that begins in the appendix as low grade mucinous neoplasm of the appendix with disseminated peritoneal adenomucinosis.
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[edit] History
The first case was described by Carl F. Rokitansky in 1842. Werth in 1884 coined the term pseudomyxoma peritonei, describing it in association with a mucinous ovarian tumour. In 1901 Frankel described the first case associated with a cyst of the appendix.
[edit] Clinical
The overall incidence is ~1-2 per million per year. It is slightly more common in women than men (male:female ratio = 9:11). The median age at presentation is typically about 50 years with a range of 20-25 years. It may be diagnosed with a range of conditions. While the majority of these cases are associated with appendiceal carcinomas,[2] other conditions may also be found, including disseminated peritoneal adenomucinosis (DPAM), peritoneal carcinomas, several mucinous tumors (mucinous adenocarcinoma, mucinous cystadenoma, and mucinous cystadenocarcinoma), as well as other disease states. [3] Other primary sites that have been reported include colon, rectum, stomach, gallbladder, bile ducts, small intestine, urinary bladder, lung, breast, fallopian tubes and pancreas. Symptoms may include abdominal or pelvic pain and/or bloating, distension, digestive disorders, weight changes, increased girth and infertility. Diagnosis is confirmed through pathology. Diagnostic tests may include CT scans, and the evaluation of tumor markers.
This disease is most often discovered during surgery for other conditions, e.g., hernia repair, following which an experienced pathologist can confirm the diagnosis. Due to the rarity of this disease, it is important to obtain an accurate diagnosis so that appropriate treatment may be obtained.
The primary tumour appears to arise from the MUC2 expressing goblet cells and most commonly from these cells in the appendix. The K-Ras (p53) gene may be involved in the oncogensis.
[edit] Treatment
Treatment is variable, both due to its rarity and to its frequently slow-growing nature. Treatment ranges from watchful waiting to debulking and intraperitoneal hyperthermic chemotherapy (IPHC) with cytoreductive surgery.[4]
[edit] Surgical
Most commonly, treatment involves surgery, and, as of 2011, specifically CRS, or cytoreductive surgery, with IPHC, performed by specific specialists trained in treating this disease.
When appropriate, surgery may include intra peritoneal hyperthermic chemotherapy (IPHC), or early post-operative intraperitoneal chemotherapy (EPIC).
In situations where surgery is not required immediately, patients can be monitored via CT scans, tumor marker laboratory tests, and physical symptoms, to determine when, and if, surgery is warranted.
Although some surgical procedures may be rather extensive, patients can and do recover from surgery, and the majority of these patients can and do live productive lives.
In debulking, the surgeon attempts to remove as much tumor as possible. CRS or cytoreductive surgery involves surgical removal of the peritoneum and any adjacent organs which appear to have tumor seeding. Since the mucus tends to pool at the bottom of the abdominal cavity, it is common to remove the ovaries, fallopian tubes, uterus, and parts of the large intestine. Depending upon the spread of the tumor, other organs might be removed, including but not limited to the gallbladder, spleen, and all or portions of the small intestine and/or stomach. For organs that cannot be removed safely (like the liver), the surgeon strips off the tumor from the surface.[5]
[edit] Chemotherapy
Chemotherapy (typically the agent Mitomycin C) may be infused directly into the abdominal cavity to kill remaining microscopic cancerous cells. The heated chemotherapy (HIPEC) is perfused throughout the abdominal cavity for an hour or two as the last step in the surgery, or ports are installed to allow circulation and/or drainage of the chemicals for one to five days after surgery (EPIC). EPIC is typically given in multiple cycles for several months after surgery.[6]
Systemic chemotherapy may be administered as additional or adjuvant treatment. Due to the increased availability of new chemotherapies developed for colon and colorectal cancer patients, some patients have experienced stability in tumor growth with systemic chemotherapy. Systemic chemotherapy is reserved for patients with advanced disease, recurrent disease, or disease that has spread to the lymph nodes or distant sites.
This disease may recur following surgery and chemotherapy. Periodic post operative CT scans and tumor marker laboratory tests are used to monitor the disease for any tumor regrowth.
Additionally recent (2003) publications linking the MUC2 enzyme overexpression to the cell reproduction has launched research efforts into additional drug treatments.
[edit] Research & Related Non-Profits
Research for treatment is presently conducted at major institutions in the USA, Australia, Italy, England, France, Germany, Japan, the Netherlands, and Spain. Hundreds of published studies may be found in medical libraries and in online resources throughout the internet.
In 2004 APCAN, a charity was formed by patients to fund research for the treatment of appendiceal cancer, from which the condition of pseudomyxoma peritonei originates.
In 2008, PMP patients throughout the U.S. formed PMP Research Foundation (PMPRF). PMPRF's mission is to fund promising research to improve treatment options and ultimately to find a cure for the disease.
PMPRF has retained NORD (National Organization for Rare Disorders) to administer its grants. To date, PMPRF has awarded 6 grants totaling $300,000 to prestigious organizations such as the Mayo Clinic, University of Pittsburgh Medical Center and other research centers in the UK and in Italy.
Prospective donors who wish to further PMP research can donate at www.pmpcure.org
An extensive listing of where donations can be sent directly to individual appendiceal cancer and pseudomyxoma peritonei research study programs is listed at www.pmppals.org
Future research into the treatment of PMP will necessitate an improved understanding of the molecular structure of the disease and targeted drug therapies that may interfere with the growth of mucinous tumor.
[edit] PMP Pals' Network
In 1998, patients, and family caregivers in the US, UK and Holland united to share resources including regarding medical options, research, clinical trials, preparation and recuperation from/for surgery and/or chemotherapy, assistance with health insurance and disability claims, and assisting patients with obtaining transportation and family lodging near cancer centers.
These patients, and family caregivers, found that their need for personal interaction was not being met via message boards, therefore they created the PMP Pals' Network, providing opportunities for patients and families to communicate directly, meet personally, and communicate via email,telephone or via Skype.
During the past thirteen years, the PMP Pals' Network of volunteers has expanded to include 48 countries and assists patients and their families in 12 languages.
The PMP Pals' Network hosts an annual international three day conference in which patients, their families and cancer treatment specialists from around the world gather to exchange resources. Information on opportunities for participation in this conference and other resource programs is available at the PMP Pals' Network website.
In August 2010 Dawn Green also set up the Pseudomyxoma Survivor website to offer a UK-based support network for PMP survivors, their care givers, and families. This site was set up in honour of a fellow PMP patient, Julie Stanton, who died after a three-year fight with the disease. The aims of the site are also to raise the profile of this rare disease and to raise funds to support the two UK PMP treatment centres at the Basingstoke and North Hampshire Hospital and The Christie Hospital Manchester. Many responses have already been received from around the world and the site now also has a Facebook page.
[edit] References
- ^ Qu Z, Liu L (2006). "Management of pseudomyxoma peritonei". World J Gastroenterol 12 (38): 6124–7. PMID 17036382.
- ^ Young R (2004). "Pseudomyxoma peritonei and selected other aspects of the spread of appendiceal neoplasms". Semin Diagn Pathol 21 (2): 134–50. doi:10.1053/j.semdp.2004.12.002. PMID 15807473.
- ^ Jacquemin G, Laloux P (2005). "Pseudomyxoma peritonei: review on a cluster of peritoneal mucinous diseases". Acta Chir Belg 105 (2): 127–33. PMID 15906901.
- ^ Sugarbaker P (2006). "New standard of care for appendiceal epithelial neoplasms and pseudomyxoma peritonei syndrome?". Lancet Oncol 7 (1): 69–76. doi:10.1016/S1470-2045(05)70539-8. PMID 16389186.
- ^ Harmon R, Sugarbaker P (2005). "Prognostic indicators in peritoneal carcinomatosis from gastrointestinal cancer". Int Semin Surg Oncol 2 (1): 3. doi:10.1186/1477-7800-2-3. PMC 549516. PMID 15701175. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=549516.
- ^ Culliford AT, Paty PB (2001). "Surgical debulking and intrapertioneal chemotherapy for established peritoneal metastases from colon and appendix cancer". Ann Surg Oncol 8 (10): 787–95. doi:10.1007/s10434-001-0787-9. PMID 11776492.
[edit] External links
- PMP Pals Network: Volunteers, Research and Support for Patients and Their Families
- UK based support network and fundraising for PMP
- PMP Pals Network: Volunteers and Resources for Patients & Caregivers
- Funding for Appendix Cancer Research
- PMP Research Foundation: Funding for promising PMP research
- PMP Awareness Group
- Pseudomyxoma peritonei at the Open Directory Project
- UK NHS PMP Treatment Information, The Christie Hospital
- Detailed technical article by Dr. Sugarbaker
- PMP surgical video from the University of Utah
- The portal for Peritonectomy
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