Pseudomyxoma peritonei: Difference between revisions
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== Clinical == |
== Clinical == |
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PMP may diagnosed with a range of conditions |
PMP may 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. |
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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. |
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. |
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== Treatment == |
== Treatment == |
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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]]. 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 can not be removed safely (like the [[liver]]), the surgeon strips off the tumor from the surface. Cytoreductive surgery usually takes between ten and thirteen hours, and is sometimes referred to by patients as MOAS (Mother Of All Surgeries) or as the ''Sugarbaker procedure'' after the doctor who pioneered this form of treatment. |
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> 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 can not be removed safely (like the [[liver]]), the surgeon strips off the tumor from the surface. Cytoreductive surgery usually takes between ten and thirteen hours, and is sometimes referred to by patients as MOAS (Mother Of All Surgeries) or as the ''Sugarbaker procedure'' after the doctor who pioneered this form of treatment.<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> |
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[[Chemotherapy]] drugs are put directly into the abdominal cavity to kill any 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. It is very important to remove or kill every last cancer cell because the 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. |
[[Chemotherapy]] drugs are put directly into the abdominal cavity to kill any 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. It is very important to remove or kill every last cancer cell because the 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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== External links == |
== External links == |
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===Treatment information=== |
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* [http://search.dmoz.org/cgi-bin/search?search=Pseudomyxoma+peritonei Pseudomyxoma peritonei] at the [[Open Directory Project]] |
* [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/profinfo/departments/Pseudomyxoma/detailedinfo.htm UK NHS PMP Treatment Information] |
* [http://www.christie.nhs.uk/profinfo/departments/Pseudomyxoma/detailedinfo.htm UK NHS PMP Treatment Information] |
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Revision as of 04:32, 24 February 2007
| Pseudomyxoma peritonei | |
|---|---|
| Specialty | Oncology  |
Pseudomyxoma peritonei (PMP) is a rare form of tumor, commonly known as jelly belly for its production of mucus in the abdominal cavity.[1] 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, the liver, kidneys, etc.
Unlike most cancers, PMP does not spread through the lymphatic system or through the bloodstream. It is believed that most PMP starts as cancer of the appendix; the Helicobacter pylori bacterium also seems to be implicated.[citation needed] Mucinous tumors of the ovary have also been implicated.
Clinical
PMP may diagnosed with a range of conditions. While the majority of PMP cases are associated with appendiceal carcinomas,[2] 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.[3] 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.
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.
Treatment
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.[4] 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 can not be removed safely (like the liver), the surgeon strips off the tumor from the surface. Cytoreductive surgery usually takes between ten and thirteen hours, and is sometimes referred to by patients as MOAS (Mother Of All Surgeries) or as the Sugarbaker procedure after the doctor who pioneered this form of treatment.[5]
Chemotherapy drugs are put directly into the abdominal cavity to kill any 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. It is very important to remove or kill every last cancer cell because the 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 CT scans for a period of time to spot any tumor regrowth.
Oral and intravenous chemotherapy is not routinely used, as it not very effective; the tumor does not have a good blood supply and so does not absorb the drugs easily. However, there are some cases in some patients where the tumor growth has been held in check for several years with a combination of orally administered drugs. Additionally recent (2003) publications linking the MUC2 enzyme overexpression to Pseudomyxoma cell reproduction has launched research efforts into additional drug treatments.
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. 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. PMID 16389186.
- ^ Harmon R, Sugarbaker P (2005). "Prognostic indicators in peritoneal carcinomatosis from gastrointestinal cancer". Int Semin Surg Oncol. 2 (1): 3. PMID 15701175.