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| {{Infobox_Disease | | | {{Pseudomyxoma peritonei}} |
| Name = {{PAGENAME}} |
| | {{CMG}}{{AE}}{{Nnasiri}}{{PSD}} |
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| DiseasesDB = 31498 |
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| ICD10 = {{ICD10|C|48|2|c|45}}|
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| ICD9 = {{ICD9|197.6}} |
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| ICDO = M8480 |
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| OMIM = |
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| MeshID = D011553 |
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| }} | |
| {{SI}} | |
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| {{SK}} PMP; jelly belly | | {{SK}} PMP; jelly belly; colloid carcinoma; disseminated peritoneal adenomucinosis (DPAM); malignant appendiceal tumor; malignant large bowel cystadenocarcinoma; malignant large bowel peritoneal carcinomatosis; malignant large bowel tumor; mucinous cyst adenocarcinoma; mucinous cystadenoma; peritoneal carcinomatosis; peritoneal mucinous carcinomatosis (PMCA); pseudomyxoma ovarii |
| | ==[[Pseudomyxoma peritonei overview|Overview]]== |
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| ==Overview== | | ==[[Pseudomyxoma peritonei historical perspective|Historical Perspective]]== |
| Pseudomyxoma peritonei is an uncommon [[tumor]] known for its production of [[mucus]] in the abdominal cavity.<ref name=Qu_2006>{{cite journal |author=Qu Z, Liu L |title=Management of pseudomyxoma peritonei |journal=World J Gastroenterol |volume=12 |issue=38 |pages=6124-7 |year=2006 |id=PMID 17036382}}</ref> The tumor is not harmful by itself, but the mucus has no place to go inside the abdominal cavity. If left untreated, it will eventually build up to the point where it compresses vital structures: the [[colon (anatomy)|colon]], the liver, kidneys, etc. | |
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| Unlike most [[cancer]]s, PMP does not spread through the [[lymphatic system]] or through the [[bloodstream]]. PMP is most commonly associated with [[Appendix cancer|cancer of the appendix]]; mucinous tumors of the [[ovary]] have also been implicated.
| | ==[[Pseudomyxoma peritonei pathophysiology|Pathophysiology]]== |
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| == Clinical == | | ==[[Pseudomyxoma peritonei causes|Causes]]== |
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| PMP may be diagnosed with a range of conditions. While the majority of PMP cases are associated with [[Appendix cancer|appendiceal carcinomas]],<ref name=Young_2004>{{cite journal |author=Young R |title=Pseudomyxoma peritonei and selected other aspects of the spread of appendiceal neoplasms |journal=Semin Diagn Pathol |volume=21 |issue=2 |pages=134-50 |year=2004 |pmid=15807473}}</ref> other conditions may also be found, including disseminated peritoneal adenomucinosis (DPAM), peritoneal carcinomas, several [[mucinous]] cancers (mucinous adenocarcinoma, mucinous cystadenoma, and mucinous cystadenocarcinoma), as well as other disease states.<ref name=Jacquemin_2005>{{cite journal |author=Jacquemin G, Laloux P |title=Pseudomyxoma peritonei: review on a cluster of peritoneal mucinous diseases |journal=Acta Chir Belg |volume=105 |issue=2 |pages=127-33 |year=2005 |pmid=15906901}}</ref> 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.
| | ==[[Pseudomyxoma peritonei differential diagnosis|Differentiating Pseudomyxoma peritonei from other Diseases]]== |
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| PMP is commonly discovered during surgery for other conditions, i.e., 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.
| | ==[[Pseudomyxoma peritonei epidemiology and demographics|Epidemiology and Demographics]]== |
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| == Treatment == | | ==[[Pseudomyxoma peritonei risk factors|Risk Factors]]== |
| Treatment for PMP is variable, both due to its rarity and to its frequently slow-growing nature. Treatment ranges from [[watchful waiting]] to [[debulking]] and cytoreductive surgery.<ref name=Sugarbaker_2006>{{cite journal |author=Sugarbaker P |title=New standard of care for appendiceal epithelial neoplasms and pseudomyxoma peritonei syndrome? |journal=Lancet Oncol |volume=7 |issue=1 |pages=69-76 |year=2006 |pmid=16389186}}</ref>
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| ===Surgical=== | | ==[[Pseudomyxoma peritonei natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
| Most commonly, treatment for PMP involves surgery performed by specific specialists trained in treating this disease.
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| When appropriate, surgery may include intraperitoneal hyperthermic chemotherapy, or post operative systemic chemotherapy.
| | ==Diagnosis== |
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| In situations where surgery is not required immediately, patients can be monitored via CT scans, tumor marker laboratory tests, and physical symptoms, to determined when, and if, surgery is warranted.
| | [[Pseudomyxoma peritonei history and symptoms|History and Symptoms ]] | [[ Pseudomyxoma peritonei physical examination|Physical Examination]] | [[Pseudomyxoma peritonei laboratory findings|Laboratory Findings]] | [[Pseudomyxoma peritonei CT|CT]] | [[Pseudomyxoma peritonei MRI|MRI]] | [[Pseudomyxoma peritonei ultrasound|Ultrasound]] | [[Pseudomyxoma peritonei other imaging findings|Other Imaging Findings]] | [[Pseudomyxoma peritonei other diagnostic studies|Other Diagnostic Studies]] |
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| 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.
| | ==Treatment== |
| | [[Pseudomyxoma peritonei medical therapy|Medical Therapy]] | [[Pseudomyxoma peritonei surgery |Surgery]] | [[Pseudomyxoma peritonei cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Pseudomyxoma peritonei future or investigational therapies|Future or Investigational Therapies]] |
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| In debulking, the surgeon attempts to remove as much tumor as possible, while 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. <ref name=Harmon_2005>{{cite journal |author=Harmon R, Sugarbaker P |title=Prognostic indicators in peritoneal carcinomatosis from gastrointestinal cancer |journal=Int Semin Surg Oncol |volume=2 |issue=1 |pages=3 |year=2005 |url=http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=549516 |pmid=15701175}}</ref>
| | ==Case Studies== |
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| ===Drug treatments===
| | [[Pseudomyxoma peritonei case study one|Case #1]] |
| [[Chemotherapy]] is infused directly into the abdominal cavity to kill remaining cancerous cells. The drugs may be manually applied to the 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. Cancer cells reproduce quickly on scar tissue, and there is lots of scar tissue after surgery. Even with aggressive, heated chemotherapy treatment PMP recurrence is common and further surgeries are frequently needed. Patients often require frequent [[Computed tomography|CT scans]] for a period of time to spot any tumor regrowth. | |
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| Oral and [[intravenous]] chemotherapy has become more commonly used during the past five years. In cases in patients have experienced stability in tumor growth through treatment with various systemic chemotherapies.
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| Additionally recent (2003) publications linking the MUC2 enzyme overexpression to Pseudomyxoma cell reproduction has launched research efforts into additional drug treatments.
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| == References ==
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| {{Reflist|2}}
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| == External links ==
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| <!-- Please do not add links to this section without discussing it on the talk page first. -->
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| * [http://search.dmoz.org/cgi-bin/search?search=Pseudomyxoma+peritonei Pseudomyxoma peritonei] at the [[Open Directory Project]]
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| * [http://www.christie.nhs.uk/patients/treatments/pmp/default.aspx UK NHS PMP Treatment Information]
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| * [http://www.surgicaloncology.com/pmp.htm Detailed technical article by Dr. Sugarbaker]
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| * [http://library.med.utah.edu/kw/human_reprod/mml/hr11.html PMP surgical video] from the [[University of Utah]]
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| * [http://www.peritonectomy.com The portal for Peritonectomy]
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| {{Epithelial neoplasms}} | | {{Epithelial neoplasms}} |
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| [[Category:Types of cancer]] | | [[Category:Types of cancer]] |
| | [[Category:Disease]] |
| [[Category:Surgery]] | | [[Category:Surgery]] |
| [[Category:Oncology]] | | [[Category:Oncology]] |
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