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{{Pseudomyxoma peritonei}}
{{Pseudomyxoma peritonei}}
{{CMG}}{{AE}}{{PSD}}
{{CMG}}{{AE}}{{Nnasiri}}
==Overview==
==Overview==
The predominant therapy for pseudomyxoma peritonei is surgical resection. Supportive therapy for pseudomyxoma peritonei includes chemotherapy. [[Chemotherapy]] is infused directly into the abdominal cavity to kill cancerous cells.  
Pseudomyxoma peritonei (PMP) is a rare condition consisting of [[intraperitoneal]] [[mucinous]] [[tumors]] and [[ascites]], most commonly arising from [[mucinous]] [[tumors]] of the [[Vermiform appendix|appendix]] and less commonly from [[ovary]] and [[colon]]. Pseudomyxoma peritonei symptoms include [[abdominal distention]], [[bloating]], [[hernia]], and [[infertility]]. It is a very uncommon [[tumor]] with an [[incidence]] of 0.1 people per 100,000 per year, slightly more common in the [[female]] gender with a median age of 50 years at the time of diagnosis. [[Prognosis]] is good in general. Pseudomyxoma peritonei is [[Histology|histologically]] classified into [[disseminated peritoneal adenomucinosis]] (DPAM), [[peritoneal mucinous carcinomatosis]] (PMCA). Mainstay of treatment is cytoreduction surgery combined with [[hyperthermic intraperitoneal chemoperfusion]] (HIPEC).


==Medical Therapy==
==Medical Therapy==
*The effect of systemic chemotherapy in pseudomyxoma peritonei seems questionable.<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><ref name="ChenHuang2008">{{cite journal|last1=Chen|first1=Chin-Fan|last2=Huang|first2=Che-Jen|last3=Kang|first3=Wan-Yi|last4=Hsieh|first4=Jan-Sing|title=Experience with adjuvant chemotherapy for pseudomyxoma peritonei secondary to mucinous adenocarcinoma of the appendix with oxaliplatin / fluorouracil /leucovorin (FOLFOX4)|journal=World Journal of Surgical Oncology|volume=6|issue=1|year=2008|pages=118|issn=1477-7819|doi=10.1186/1477-7819-6-118}}</ref>  
Mainstay of treatment for pseudomyxoma peritonei is cytoreductive surgery combined with [[intraperitoneal]] [[chemotherapy]] with [[mitomycin]] at 42°C.<ref name="ChenHuang2008">{{cite journal|last1=Chen|first1=Chin-Fan|last2=Huang|first2=Che-Jen|last3=Kang|first3=Wan-Yi|last4=Hsieh|first4=Jan-Sing|title=Experience with adjuvant chemotherapy for pseudomyxoma peritonei secondary to mucinous adenocarcinoma of the appendix with oxaliplatin / fluorouracil /leucovorin (FOLFOX4)|journal=World Journal of Surgical Oncology|volume=6|issue=1|year=2008|pages=118|issn=1477-7819|doi=10.1186/1477-7819-6-118}}</ref><ref name="Sugarbaker2006">{{cite journal|last1=Sugarbaker|first1=Paul H|title=New standard of care for appendiceal epithelial neoplasms and pseudomyxoma peritonei syndrome?|journal=The Lancet Oncology|volume=7|issue=1|year=2006|pages=69–76|issn=14702045|doi=10.1016/S1470-2045(05)70539-8}}</ref><ref name="pmid19567401">{{cite journal |vauthors=Vaira M, Cioppa T, DE Marco G, Bing C, D'Amico S, D'Alessandro M, Fiorentini G, DE Simone M |title=Management of pseudomyxoma peritonei by cytoreduction+HIPEC (hyperthermic intraperitoneal chemotherapy): results analysis of a twelve-year experience |journal=In Vivo |volume=23 |issue=4 |pages=639–44 |date=2009 |pmid=19567401 |doi= |url=}}</ref>
*Jones et al reported their experience in the treatment of pseudomyxoma peritonei of ovarian origin with cisplatinum, doxorubicin, and cyclophosphamide, with excellent responses.  
*On the other hand, Smeenk et al reported the poor response of six patients (3 patients with DPAM, another 3 patients with PMCA-I, and all 6 patients with lesions diffusely spread throughout the abdomen) after 5-FU based systemic chemotherapy, and subsequent poor prognosis was noted in the study.
*Regarding the benefit of new therapeutic agents (including Capecitabine, Oxaliplatin, Irinotecan and Bevacizumab) and modern schedules for patients with metastatic CRC, clinical experience with the use of these agents for pseudomyxoma peritonei are still absent, and it is questionable whether they will do any better in this situation, especially for patients with PMCA.
*Due to the limited experience and indeterminate effects of systemic chemotherapy in pseudomyxoma peritonei, some studies still suggest that systemic therapy should be reserved for a palliative setting in patients with recurrent or progressive disease
*[[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 pseudomyxoma peritonei 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.
*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.
*Additionally recent (2003) publications linking the MUC2 enzyme overexpression to Pseudomyxoma cell reproduction has launched research efforts into additional drug treatments.


== References ==
== References ==
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Latest revision as of 13:27, 3 April 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Nima Nasiri, M.D.[2]

Overview

Pseudomyxoma peritonei (PMP) is a rare condition consisting of intraperitoneal mucinous tumors and ascites, most commonly arising from mucinous tumors of the appendix and less commonly from ovary and colon. Pseudomyxoma peritonei symptoms include abdominal distention, bloating, hernia, and infertility. It is a very uncommon tumor with an incidence of 0.1 people per 100,000 per year, slightly more common in the female gender with a median age of 50 years at the time of diagnosis. Prognosis is good in general. Pseudomyxoma peritonei is histologically classified into disseminated peritoneal adenomucinosis (DPAM), peritoneal mucinous carcinomatosis (PMCA). Mainstay of treatment is cytoreduction surgery combined with hyperthermic intraperitoneal chemoperfusion (HIPEC).

Medical Therapy

Mainstay of treatment for pseudomyxoma peritonei is cytoreductive surgery combined with intraperitoneal chemotherapy with mitomycin at 42°C.[1][2][3]

References

  1. Chen, Chin-Fan; Huang, Che-Jen; Kang, Wan-Yi; Hsieh, Jan-Sing (2008). "Experience with adjuvant chemotherapy for pseudomyxoma peritonei secondary to mucinous adenocarcinoma of the appendix with oxaliplatin / fluorouracil /leucovorin (FOLFOX4)". World Journal of Surgical Oncology. 6 (1): 118. doi:10.1186/1477-7819-6-118. ISSN 1477-7819.
  2. Sugarbaker, Paul H (2006). "New standard of care for appendiceal epithelial neoplasms and pseudomyxoma peritonei syndrome?". The Lancet Oncology. 7 (1): 69–76. doi:10.1016/S1470-2045(05)70539-8. ISSN 1470-2045.
  3. Vaira M, Cioppa T, DE Marco G, Bing C, D'Amico S, D'Alessandro M, Fiorentini G, DE Simone M (2009). "Management of pseudomyxoma peritonei by cytoreduction+HIPEC (hyperthermic intraperitoneal chemotherapy): results analysis of a twelve-year experience". In Vivo. 23 (4): 639–44. PMID 19567401.


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