Pseudomyxoma peritonei overview: Difference between revisions
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==Overview== | ==Overview== | ||
Pseudomyxoma peritonei is a very rare [[tumor]] characterized by the presence of abundant [[mucus]] in the abdominal cavity, it's consisting of intraperitoneal mucinous tumors and ascites, most commonly arises from mucinous tumors of the appendix but it can have the ovary or colon as the primary site of origin. The tumor is not harmful by itself, but the mucus accumulation in abdominal cavity compresses vital organs such as spleen, kidney, colon. On 1842, Carl F. Rokitansky described the first case of pseudomyxoma peritonei. Pseudomyxoma peritonei may be divided into two pathological subtypes with different etiology and prognosis, these two subtypes are Peritoneal adenomucinosis and Peritoneal mucinous carcinoma. The ''K-Ras'' and ''p53'' genes are involved in the oncogenesis. Pseudomyxoma peritonei must be differentiated from peritoneal carcinomatosis without mucinous ascites, peritoneal sarcomatosis, and | Pseudomyxoma peritonei is a very rare [[tumor]] characterized by the presence of abundant [[mucus]] in the abdominal cavity, it's consisting of intraperitoneal mucinous tumors and ascites, most commonly arises from mucinous tumors of the [[appendix]] but it can have the ovary or colon as the primary site of origin. The tumor is not harmful by itself, but the mucus accumulation in abdominal cavity compresses vital organs such as spleen, kidney, colon. On 1842, Carl F. Rokitansky described the first case of pseudomyxoma peritonei. Pseudomyxoma peritonei may be divided into two [[pathological]] subtypes with different [[etiology]] and prognosis, these two subtypes are Peritoneal adenomucinosis and Peritoneal mucinous carcinoma. The ''K-Ras'' and [[P53 gene|''p53'' genes]] are involved in the [[oncogenesis]]. Pseudomyxoma peritonei must be differentiated from [[peritoneal carcinomatosis without mucinous ascites]], [[peritoneal sarcomatosis]], and [[tuberculosis]] peritonitis. The incidence of pseudomyxoma peritonei is approximately 0.1 person per 100,000 individuals worldwide. Females are more commonly affected with pseudomyxoma peritonei than males. The median age at diagnosis is 50 years. There are no established risk factors for pseudomyxoma peritonei. Patients with pseudomyxoma peritonei may develop abdominal or pelvic pain, bloating, [[abdominal distension]], weight change, and [[infertility]] symptoms if left untreated. Common [[physical examination]] findings of pseudomyxoma peritonei include [[abdominal tenderness]], abdominal distention. On abdominal [[CT scan]], pseudomyxoma peritonei is characterized by low attenuation, loculated [[ascitic]] mucinous fluid in the [[peritoneum]], [[omentum]] and [[mesentery]] with scalloping of visceral surfaces of the liver. On abdominal MRI, pseudomyxoma peritonei is characterized by a mass which is hypointense on T1-weighted [[MRI]] and hyperintense on T2-weighted MRI. Finding on ultrasound scan suggestive of pseudomyxoma peritonei are echogenic peritoneal masses or [[ascites]] which is not mobile. Cytoreductive surgery combined with [[hyperthermic intraperitoneal chemoperfusion]] is the optimal way of treatment for pseudomyxoma peritonei. | ||
==Historical Perspective== | ==Historical Perspective== | ||
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==Pathophysiology== | ==Pathophysiology== | ||
Pseudomyxoma peritonei is a rare poorly understood neoplasm which may be divided into two pathological subtypes:<ref name="pmid26475379">{{cite journal |vauthors=Noguchi R, Yano H, Gohda Y, Suda R, Igari T, Ohta Y, Yamashita N, Yamaguchi K, Terakado Y, Ikenoue T, Furukawa Y |title=Molecular profiles of high-grade and low-grade pseudomyxoma peritonei |journal=Cancer Med |volume=4 |issue=12 |pages=1809–16 |date=December 2015 |pmid=26475379 |pmc=5123786 |doi=10.1002/cam4.542 |url=}}</ref> | Pseudomyxoma peritonei is a rare poorly understood neoplasm which may be divided into two pathological subtypes:<ref name="pmid26475379">{{cite journal |vauthors=Noguchi R, Yano H, Gohda Y, Suda R, Igari T, Ohta Y, Yamashita N, Yamaguchi K, Terakado Y, Ikenoue T, Furukawa Y |title=Molecular profiles of high-grade and low-grade pseudomyxoma peritonei |journal=Cancer Med |volume=4 |issue=12 |pages=1809–16 |date=December 2015 |pmid=26475379 |pmc=5123786 |doi=10.1002/cam4.542 |url=}}</ref> | ||
*Disseminated peritoneal adenomucinosis (DPAM) | *[[Disseminated peritoneal adenomucinosis]] (DPAM) | ||
*Peritoneal mucinous carcinomatosis (PMCA) | *[[Peritoneal mucinous carcinomatosis]] (PMCA) | ||
==Causes== | ==Causes== | ||
Pseudomyxoma peritonei is caused by a pre-existing intraperitoneal mucinous neoplasm. | Pseudomyxoma peritonei is caused by a pre-existing intraperitoneal mucinous neoplasm. | ||
*The ''K-Ras'' and ''p53'' genes may be involved in the oncogenesis.<ref name="pmid28280620">{{cite journal |vauthors=Borazanci E, Millis SZ, Kimbrough J, Doll N, Von Hoff D, Ramanathan RK |title=Potential actionable targets in appendiceal cancer detected by immunohistochemistry, fluorescent in situ hybridization, and mutational analysis |journal=J Gastrointest Oncol |volume=8 |issue=1 |pages=164–172 |date=February 2017 |pmid=28280620 |pmc=5334061 |doi=10.21037/jgo.2017.01.14 |url=}}</ref> | *The ''K-Ras'' and [[P53 gene|''p53'' genes]] may be involved in the oncogenesis.<ref name="pmid28280620">{{cite journal |vauthors=Borazanci E, Millis SZ, Kimbrough J, Doll N, Von Hoff D, Ramanathan RK |title=Potential actionable targets in appendiceal cancer detected by immunohistochemistry, fluorescent in situ hybridization, and mutational analysis |journal=J Gastrointest Oncol |volume=8 |issue=1 |pages=164–172 |date=February 2017 |pmid=28280620 |pmc=5334061 |doi=10.21037/jgo.2017.01.14 |url=}}</ref> | ||
==Differentiating Pseudomyxoma peritonei from other Diseases== | ==Differentiating Pseudomyxoma peritonei from other Diseases== | ||
Differential diagnosis of pseudomyxoma peritonei include:<ref name="LevyShaw2009">{{cite journal|last1=Levy|first1=Angela D.|last2=Shaw|first2=Janet C.|last3=Sobin|first3=Leslie H.|title=Secondary Tumors and Tumorlike Lesions of the Peritoneal Cavity: Imaging Features with Pathologic Correlation|journal=RadioGraphics|volume=29|issue=2|year=2009|pages=347–373|issn=0271-5333|doi=10.1148/rg.292085189}}</ref> | Differential diagnosis of pseudomyxoma peritonei include:<ref name="LevyShaw2009">{{cite journal|last1=Levy|first1=Angela D.|last2=Shaw|first2=Janet C.|last3=Sobin|first3=Leslie H.|title=Secondary Tumors and Tumorlike Lesions of the Peritoneal Cavity: Imaging Features with Pathologic Correlation|journal=RadioGraphics|volume=29|issue=2|year=2009|pages=347–373|issn=0271-5333|doi=10.1148/rg.292085189}}</ref> | ||
*Peritoneal carcinomatosis without mucinous ascites | *[[Peritoneal carcinomatosis without mucinous ascites]] | ||
*Peritoneal sarcomatosis | *Peritoneal sarcomatosis | ||
*[[Peritonitis]] | *[[Peritonitis]] | ||
*Peritoneal mesothelioma | *Peritoneal [[mesothelioma]] | ||
==Epidemiology and Demographics== | ==Epidemiology and Demographics== | ||
*The incidence of pseudomyxoma peritonei is approximately 0.1 per 100,000 individuals worldwide. | *The [[incidence]] of pseudomyxoma peritonei is approximately 0.1 per 100,000 individuals worldwide. | ||
*Females are more commonly affected with pseudomyxoma peritonei than males. | *Females are more commonly affected with pseudomyxoma peritonei than males. | ||
*The median age at diagnosis is 50 years.<ref name="pmid25343082">{{cite journal |vauthors=Zhong Y, Deng M, Xu R, Kokudo N, Tang W |title=Pseudomyxoma peritonei as an intractable disease and its preoperative assessment to help improve prognosis after surgery: A review of the literature |journal=Intractable Rare Dis Res |volume=1 |issue=3 |pages=115–21 |date=August 2012 |pmid=25343082 |pmc=4204597 |doi=10.5582/irdr.2012.v1.3.115 |url=}}</ref> | *The median age at diagnosis is 50 years.<ref name="pmid25343082">{{cite journal |vauthors=Zhong Y, Deng M, Xu R, Kokudo N, Tang W |title=Pseudomyxoma peritonei as an intractable disease and its preoperative assessment to help improve prognosis after surgery: A review of the literature |journal=Intractable Rare Dis Res |volume=1 |issue=3 |pages=115–21 |date=August 2012 |pmid=25343082 |pmc=4204597 |doi=10.5582/irdr.2012.v1.3.115 |url=}}</ref> | ||
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==Natural History, Complications and Prognosis== | ==Natural History, Complications and Prognosis== | ||
*Patients with pseudomyxoma peritonei may develop abdominal or pelvic pain, [[bloating]], [[abdominal distension]], [[weight change]], and [[infertility]].<ref name="pmid21304299">{{cite journal |vauthors=Youssef H, Newman C, Chandrakumaran K, Mohamed F, Cecil TD, Moran BJ |title=Operative findings, early complications, and long-term survival in 456 patients with pseudomyxoma peritonei syndrome of appendiceal origin |journal=Dis. Colon Rectum |volume=54 |issue=3 |pages=293–9 |date=March 2011 |pmid=21304299 |doi=10.1007/DCR.0b013e318202f026 |url=}}</ref> | *Patients with pseudomyxoma peritonei may develop abdominal or pelvic pain, [[bloating]], [[abdominal distension]], [[weight change]], and [[infertility]].<ref name="pmid21304299">{{cite journal |vauthors=Youssef H, Newman C, Chandrakumaran K, Mohamed F, Cecil TD, Moran BJ |title=Operative findings, early complications, and long-term survival in 456 patients with pseudomyxoma peritonei syndrome of appendiceal origin |journal=Dis. Colon Rectum |volume=54 |issue=3 |pages=293–9 |date=March 2011 |pmid=21304299 |doi=10.1007/DCR.0b013e318202f026 |url=}}</ref> | ||
*Prognosis is generally good and the 10-year survival rate of patients with pseudomyxoma peritonei is approximately 63%.<ref name="pmid22614976">{{cite journal |vauthors=Chua TC, Moran BJ, Sugarbaker PH, Levine EA, Glehen O, Gilly FN, Baratti D, Deraco M, Elias D, Sardi A, Liauw W, Yan TD, Barrios P, Gómez Portilla A, de Hingh IH, Ceelen WP, Pelz JO, Piso P, González-Moreno S, Van Der Speeten K, Morris DL |title=Early- and long-term outcome data of patients with pseudomyxoma peritonei from appendiceal origin treated by a strategy of cytoreductive surgery and hyperthermic intraperitoneal chemotherapy |journal=J. Clin. Oncol. |volume=30 |issue=20 |pages=2449–56 |date=July 2012 |pmid=22614976 |doi=10.1200/JCO.2011.39.7166 |url=}}</ref> | *[[Prognosis]] is generally good and the 10-year [[Survival rates|survival rate]] of patients with pseudomyxoma peritonei is approximately 63%.<ref name="pmid22614976">{{cite journal |vauthors=Chua TC, Moran BJ, Sugarbaker PH, Levine EA, Glehen O, Gilly FN, Baratti D, Deraco M, Elias D, Sardi A, Liauw W, Yan TD, Barrios P, Gómez Portilla A, de Hingh IH, Ceelen WP, Pelz JO, Piso P, González-Moreno S, Van Der Speeten K, Morris DL |title=Early- and long-term outcome data of patients with pseudomyxoma peritonei from appendiceal origin treated by a strategy of cytoreductive surgery and hyperthermic intraperitoneal chemotherapy |journal=J. Clin. Oncol. |volume=30 |issue=20 |pages=2449–56 |date=July 2012 |pmid=22614976 |doi=10.1200/JCO.2011.39.7166 |url=}}</ref> | ||
*Overall risk of complications after surgery such as urinary tract involvement due to tumor seeding in pelvis is very low. <ref name="pmid16353186">{{cite journal |vauthors=Smeenk RM, Bex A, Verwaal VJ, Horenblas S, Zoetmulder FA |title=Pseudomyxoma peritonei and the urinary tract: involvement and treatment related complications |journal=J Surg Oncol |volume=93 |issue=1 |pages=20–3 |date=January 2006 |pmid=16353186 |doi=10.1002/jso.20427 |url=}}</ref> | *Overall risk of complications after [[surgery]] such as urinary tract involvement due to [[tumor]] seeding in pelvis is very low. <ref name="pmid16353186">{{cite journal |vauthors=Smeenk RM, Bex A, Verwaal VJ, Horenblas S, Zoetmulder FA |title=Pseudomyxoma peritonei and the urinary tract: involvement and treatment related complications |journal=J Surg Oncol |volume=93 |issue=1 |pages=20–3 |date=January 2006 |pmid=16353186 |doi=10.1002/jso.20427 |url=}}</ref> | ||
==History and Symptoms== | ==History and Symptoms== | ||
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*[[bloating]] | *[[bloating]] | ||
*[[abdominal distention]] | *[[abdominal distention]] | ||
*Shortness of breath | *[[Shortness of breath]] | ||
* | *[[Weight change|Weight changes]] | ||
*Menstrual irregularity in women | *[[Menstrual irregularities|Menstrual irregularity]] in women | ||
==Physical Examination== | ==Physical Examination== | ||
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==Laboratory Findings== | ==Laboratory Findings== | ||
The laboratory findings associated with pseudomyxoma peritonei include: | The laboratory findings associated with pseudomyxoma peritonei include: | ||
*Increased levels of tumor markers such as [[carcinoembryonic antigen]] (CEA), carbohydrate antigen 19.9 (CA19.9), carbohydrate antigen 125 ([[CA125]]).<ref name="AminiMasoumi-Moghaddam2014">{{cite journal|last1=Amini|first1=Afshin|last2=Masoumi-Moghaddam|first2=Samar|last3=Ehteda|first3=Anahid|last4=Morris|first4=David|title=Secreted mucins in pseudomyxoma peritonei: pathophysiological significance and potential therapeutic prospects|journal=Orphanet Journal of Rare Diseases|volume=9|issue=1|year=2014|pages=71|issn=1750-1172|doi=10.1186/1750-1172-9-71}}</ref> | *Increased levels of [[tumor markers]] such as [[carcinoembryonic antigen]] (CEA), carbohydrate antigen 19.9 (CA19.9), carbohydrate antigen 125 ([[CA125]]).<ref name="AminiMasoumi-Moghaddam2014">{{cite journal|last1=Amini|first1=Afshin|last2=Masoumi-Moghaddam|first2=Samar|last3=Ehteda|first3=Anahid|last4=Morris|first4=David|title=Secreted mucins in pseudomyxoma peritonei: pathophysiological significance and potential therapeutic prospects|journal=Orphanet Journal of Rare Diseases|volume=9|issue=1|year=2014|pages=71|issn=1750-1172|doi=10.1186/1750-1172-9-71}}</ref> | ||
==CT== | ==CT== | ||
Findings on abdominal CT scan of patients with pseudomyxoma peritonei include:<ref name="pmid22147976">{{cite journal |vauthors=Lee NK, Kim S, Kim HS, Jeon TY, Kim GH, Kim DU, Park DY, Kim TU, Kang DH |title=Spectrum of mucin-producing neoplastic conditions of the abdomen and pelvis: cross-sectional imaging evaluation |journal=World J. Gastroenterol. |volume=17 |issue=43 |pages=4757–71 |date=November 2011 |pmid=22147976 |pmc=3229624 |doi=10.3748/wjg.v17.i43.4757 |url=}}</ref> | Findings on abdominal [[CT scan]] of patients with pseudomyxoma peritonei include:<ref name="pmid22147976">{{cite journal |vauthors=Lee NK, Kim S, Kim HS, Jeon TY, Kim GH, Kim DU, Park DY, Kim TU, Kang DH |title=Spectrum of mucin-producing neoplastic conditions of the abdomen and pelvis: cross-sectional imaging evaluation |journal=World J. Gastroenterol. |volume=17 |issue=43 |pages=4757–71 |date=November 2011 |pmid=22147976 |pmc=3229624 |doi=10.3748/wjg.v17.i43.4757 |url=}}</ref> | ||
*Accumulation of fluid throughout the peritoneum, omentum and mesentery seen as low attenuation mucin depositis on CT scan. | *Accumulation of fluid throughout the [[peritoneum]], [[omentum]] and [[mesentery]] seen as low attenuation mucin depositis on [[CT scan]]. | ||
*Scalloping of visceral organs. | *Scalloping of [[visceral]] organs. | ||
==MRI== | ==MRI== | ||
*On abdominal MRI, pseudomyxoma peritonei is characterized by a mass which is hypointense on T1-weighted MRI and hyperintense on T2-weighted MRI, it can further clarify the cystic structure of mass.<ref name="pmid26494720">{{cite journal |vauthors=Wrafter PF, Connelly T, Khan JS, Joyce WP |title=Pseudomyxoma peritonei diagnosed 19 years after appendicectomy |journal=BMJ Case Rep |volume=2015 |issue= |pages= |date=October 2015 |pmid=26494720 |pmc=4620202 |doi=10.1136/bcr-2015-211706 |url=}}</ref> | *On abdominal [[MRI]], pseudomyxoma peritonei is characterized by a mass which is hypointense on T1-weighted [[MRI]] and hyperintense on T2-weighted MRI, it can further clarify the [[cystic]] structure of mass.<ref name="pmid26494720">{{cite journal |vauthors=Wrafter PF, Connelly T, Khan JS, Joyce WP |title=Pseudomyxoma peritonei diagnosed 19 years after appendicectomy |journal=BMJ Case Rep |volume=2015 |issue= |pages= |date=October 2015 |pmid=26494720 |pmc=4620202 |doi=10.1136/bcr-2015-211706 |url=}}</ref> | ||
==Ultrasound== | ==Ultrasound== | ||
*Abdominal ultrasound scan may be helpful in the diagnosis of pseudomyxoma peritonei. | *Abdominal [[ultrasound]] scan may be helpful in the diagnosis of pseudomyxoma peritonei. | ||
*Finding on ultrasound scan suggestive of pseudomyxoma peritonei are echogenic peritoneal masses or ascites with echogenic particles which do not move.<ref name="pmid11952975">{{cite journal |vauthors=Khan S, Patel AG, Jurkovic D |title=Incidental ultrasound diagnosis of pseudomyxoma peritonei in an asymptomatic woman |journal=Ultrasound Obstet Gynecol |volume=19 |issue=4 |pages=410–2 |date=April 2002 |pmid=11952975 |doi=10.1046/j.1469-0705.2002.00611.x |url=}}</ref><ref name="pmid26564459">{{cite journal |vauthors=Qi ZH, Cai S, Li JC, L K, Zhang Y, Xiao MS, Su N |title=[Ultrasonographic Features of Pseudomyxoma Peritonei and TheirComparison with Computed Tomographic Findings] |language=Chinese |journal=Zhongguo Yi Xue Ke Xue Yuan Xue Bao |volume=37 |issue=4 |pages=424–9 |date=August 2015 |pmid=26564459 |doi=10.3881/j.issn.1000-503X.2015.04.010 |url=}}</ref><ref name="pmid23980216">{{cite journal |vauthors=Li Y, Guo A, Tang J, Wang L, Wang J, Yu D |title=Role of preoperative sonography in the diagnosis and pathologic staging of pseudomyxoma peritonei |journal=J Ultrasound Med |volume=32 |issue=9 |pages=1565–70 |date=September 2013 |pmid=23980216 |doi=10.7863/ultra.32.9.1565 |url=}}</ref> | *Finding on ultrasound scan suggestive of pseudomyxoma peritonei are echogenic peritoneal masses or ascites with echogenic particles which do not move.<ref name="pmid11952975">{{cite journal |vauthors=Khan S, Patel AG, Jurkovic D |title=Incidental ultrasound diagnosis of pseudomyxoma peritonei in an asymptomatic woman |journal=Ultrasound Obstet Gynecol |volume=19 |issue=4 |pages=410–2 |date=April 2002 |pmid=11952975 |doi=10.1046/j.1469-0705.2002.00611.x |url=}}</ref><ref name="pmid26564459">{{cite journal |vauthors=Qi ZH, Cai S, Li JC, L K, Zhang Y, Xiao MS, Su N |title=[Ultrasonographic Features of Pseudomyxoma Peritonei and TheirComparison with Computed Tomographic Findings] |language=Chinese |journal=Zhongguo Yi Xue Ke Xue Yuan Xue Bao |volume=37 |issue=4 |pages=424–9 |date=August 2015 |pmid=26564459 |doi=10.3881/j.issn.1000-503X.2015.04.010 |url=}}</ref><ref name="pmid23980216">{{cite journal |vauthors=Li Y, Guo A, Tang J, Wang L, Wang J, Yu D |title=Role of preoperative sonography in the diagnosis and pathologic staging of pseudomyxoma peritonei |journal=J Ultrasound Med |volume=32 |issue=9 |pages=1565–70 |date=September 2013 |pmid=23980216 |doi=10.7863/ultra.32.9.1565 |url=}}</ref> | ||
==Medical Therapy== | ==Medical Therapy== | ||
*The predominant therapy for pseudomyxoma peritonei is surgical resection and debulking. <ref name="pmid28540829">{{cite journal |vauthors=Mittal R, Chandramohan A, Moran B |title=Pseudomyxoma peritonei: natural history and treatment |journal=Int J Hyperthermia |volume=33 |issue=5 |pages=511–519 |date=August 2017 |pmid=28540829 |doi=10.1080/02656736.2017.1310938 |url=}}</ref> | *The predominant [[therapy]] for pseudomyxoma peritonei is surgical resection and [[debulking]]. <ref name="pmid28540829">{{cite journal |vauthors=Mittal R, Chandramohan A, Moran B |title=Pseudomyxoma peritonei: natural history and treatment |journal=Int J Hyperthermia |volume=33 |issue=5 |pages=511–519 |date=August 2017 |pmid=28540829 |doi=10.1080/02656736.2017.1310938 |url=}}</ref> | ||
*Supportive therapy for pseudomyxoma peritonei includes chemotherapy which can be used as adjuvant treatment. | *Supportive therapy for pseudomyxoma peritonei includes [[chemotherapy]] which can be used as adjuvant treatment. | ||
*The treatment of choice is complete tumor excision named cytoreductive surgery (CRS) combined with Hyperthermic | *The treatment of choice is complete tumor excision named cytoreductive surgery (CRS) combined with [[Hyperthermic intraperitoneal chemoperfusion]] (HIPEC).<ref name="pmid17197972">{{cite journal |vauthors=Smeenk RM, Verwaal VJ, Antonini N, Zoetmulder FA |title=Survival analysis of pseudomyxoma peritonei patients treated by cytoreductive surgery and hyperthermic intraperitoneal chemotherapy |journal=Ann. Surg. |volume=245 |issue=1 |pages=104–9 |date=January 2007 |pmid=17197972 |pmc=1867935 |doi=10.1097/01.sla.0000231705.40081.1a |url=}}</ref> | ||
==Surgery== | ==Surgery== | ||
Surgery is the mainstay of treatment for pseudomyxoma peritonei. The feasibility of surgery depends on the stage of pseudomyxoma peritonei at diagnosis.<ref name="AminiMasoumi-Moghaddam2014">{{cite journal|last1=Amini|first1=Afshin|last2=Masoumi-Moghaddam|first2=Samar|last3=Ehteda|first3=Anahid|last4=Morris|first4=David|title=Secreted mucins in pseudomyxoma peritonei: pathophysiological significance and potential therapeutic prospects|journal=Orphanet Journal of Rare Diseases|volume=9|issue=1|year=2014|pages=71|issn=1750-1172|doi=10.1186/1750-1172-9-71}}</ref><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> | [[Surgery]] is the mainstay of treatment for pseudomyxoma peritonei. The feasibility of surgery depends on the stage of pseudomyxoma peritonei at diagnosis.<ref name="AminiMasoumi-Moghaddam2014">{{cite journal|last1=Amini|first1=Afshin|last2=Masoumi-Moghaddam|first2=Samar|last3=Ehteda|first3=Anahid|last4=Morris|first4=David|title=Secreted mucins in pseudomyxoma peritonei: pathophysiological significance and potential therapeutic prospects|journal=Orphanet Journal of Rare Diseases|volume=9|issue=1|year=2014|pages=71|issn=1750-1172|doi=10.1186/1750-1172-9-71}}</ref><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> | ||
== References == | == References == | ||
{{Reflist|2}} | {{Reflist|2}} | ||
Revision as of 15:56, 22 January 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]Parminder Dhingra, M.D. [3]
Overview
Pseudomyxoma peritonei is a very rare tumor characterized by the presence of abundant mucus in the abdominal cavity, it's consisting of intraperitoneal mucinous tumors and ascites, most commonly arises from mucinous tumors of the appendix but it can have the ovary or colon as the primary site of origin. The tumor is not harmful by itself, but the mucus accumulation in abdominal cavity compresses vital organs such as spleen, kidney, colon. On 1842, Carl F. Rokitansky described the first case of pseudomyxoma peritonei. Pseudomyxoma peritonei may be divided into two pathological subtypes with different etiology and prognosis, these two subtypes are Peritoneal adenomucinosis and Peritoneal mucinous carcinoma. The K-Ras and p53 genes are involved in the oncogenesis. Pseudomyxoma peritonei must be differentiated from peritoneal carcinomatosis without mucinous ascites, peritoneal sarcomatosis, and tuberculosis peritonitis. The incidence of pseudomyxoma peritonei is approximately 0.1 person per 100,000 individuals worldwide. Females are more commonly affected with pseudomyxoma peritonei than males. The median age at diagnosis is 50 years. There are no established risk factors for pseudomyxoma peritonei. Patients with pseudomyxoma peritonei may develop abdominal or pelvic pain, bloating, abdominal distension, weight change, and infertility symptoms if left untreated. Common physical examination findings of pseudomyxoma peritonei include abdominal tenderness, abdominal distention. On abdominal CT scan, pseudomyxoma peritonei is characterized by low attenuation, loculated ascitic mucinous fluid in the peritoneum, omentum and mesentery with scalloping of visceral surfaces of the liver. On abdominal MRI, pseudomyxoma peritonei is characterized by a mass which is hypointense on T1-weighted MRI and hyperintense on T2-weighted MRI. Finding on ultrasound scan suggestive of pseudomyxoma peritonei are echogenic peritoneal masses or ascites which is not mobile. Cytoreductive surgery combined with hyperthermic intraperitoneal chemoperfusion is the optimal way of treatment for pseudomyxoma peritonei.
Historical Perspective
The first case of pseudomyxoma peritonei was described by Carl F. Rokitansky in 1842.[1]
Pathophysiology
Pseudomyxoma peritonei is a rare poorly understood neoplasm which may be divided into two pathological subtypes:[2]
Causes
Pseudomyxoma peritonei is caused by a pre-existing intraperitoneal mucinous neoplasm.
Differentiating Pseudomyxoma peritonei from other Diseases
Differential diagnosis of pseudomyxoma peritonei include:[4]
- Peritoneal carcinomatosis without mucinous ascites
- Peritoneal sarcomatosis
- Peritonitis
- Peritoneal mesothelioma
Epidemiology and Demographics
- The incidence of pseudomyxoma peritonei is approximately 0.1 per 100,000 individuals worldwide.
- Females are more commonly affected with pseudomyxoma peritonei than males.
- The median age at diagnosis is 50 years.[5]
Risk Factors
There are no established risk factors for pseudomyxoma peritonei.
Natural History, Complications and Prognosis
- Patients with pseudomyxoma peritonei may develop abdominal or pelvic pain, bloating, abdominal distension, weight change, and infertility.[6]
- Prognosis is generally good and the 10-year survival rate of patients with pseudomyxoma peritonei is approximately 63%.[7]
- Overall risk of complications after surgery such as urinary tract involvement due to tumor seeding in pelvis is very low. [8]
History and Symptoms
Symptoms of pseudomyxoma peritonei include:[9]
- abdominal pain
- bloating
- abdominal distention
- Shortness of breath
- Weight changes
- Menstrual irregularity in women
Physical Examination
Common physical examination findings of pseudomyxoma peritonei include: [9]
Laboratory Findings
The laboratory findings associated with pseudomyxoma peritonei include:
- Increased levels of tumor markers such as carcinoembryonic antigen (CEA), carbohydrate antigen 19.9 (CA19.9), carbohydrate antigen 125 (CA125).[10]
CT
Findings on abdominal CT scan of patients with pseudomyxoma peritonei include:[11]
- Accumulation of fluid throughout the peritoneum, omentum and mesentery seen as low attenuation mucin depositis on CT scan.
- Scalloping of visceral organs.
MRI
- On abdominal MRI, pseudomyxoma peritonei is characterized by a mass which is hypointense on T1-weighted MRI and hyperintense on T2-weighted MRI, it can further clarify the cystic structure of mass.[12]
Ultrasound
- Abdominal ultrasound scan may be helpful in the diagnosis of pseudomyxoma peritonei.
- Finding on ultrasound scan suggestive of pseudomyxoma peritonei are echogenic peritoneal masses or ascites with echogenic particles which do not move.[13][14][15]
Medical Therapy
- The predominant therapy for pseudomyxoma peritonei is surgical resection and debulking. [16]
- Supportive therapy for pseudomyxoma peritonei includes chemotherapy which can be used as adjuvant treatment.
- The treatment of choice is complete tumor excision named cytoreductive surgery (CRS) combined with Hyperthermic intraperitoneal chemoperfusion (HIPEC).[17]
Surgery
Surgery is the mainstay of treatment for pseudomyxoma peritonei. The feasibility of surgery depends on the stage of pseudomyxoma peritonei at diagnosis.[10][18]
References
- ↑ O'Connell JT, Tomlinson JS, Roberts AA, McGonigle KF, Barsky SH (August 2002). "Pseudomyxoma peritonei is a disease of MUC2-expressing goblet cells". Am. J. Pathol. 161 (2): 551–64. doi:10.1016/S0002-9440(10)64211-3. PMC 1850719. PMID 12163380.
- ↑ Noguchi R, Yano H, Gohda Y, Suda R, Igari T, Ohta Y, Yamashita N, Yamaguchi K, Terakado Y, Ikenoue T, Furukawa Y (December 2015). "Molecular profiles of high-grade and low-grade pseudomyxoma peritonei". Cancer Med. 4 (12): 1809–16. doi:10.1002/cam4.542. PMC 5123786. PMID 26475379.
- ↑ Borazanci E, Millis SZ, Kimbrough J, Doll N, Von Hoff D, Ramanathan RK (February 2017). "Potential actionable targets in appendiceal cancer detected by immunohistochemistry, fluorescent in situ hybridization, and mutational analysis". J Gastrointest Oncol. 8 (1): 164–172. doi:10.21037/jgo.2017.01.14. PMC 5334061. PMID 28280620.
- ↑ Levy, Angela D.; Shaw, Janet C.; Sobin, Leslie H. (2009). "Secondary Tumors and Tumorlike Lesions of the Peritoneal Cavity: Imaging Features with Pathologic Correlation". RadioGraphics. 29 (2): 347–373. doi:10.1148/rg.292085189. ISSN 0271-5333.
- ↑ Zhong Y, Deng M, Xu R, Kokudo N, Tang W (August 2012). "Pseudomyxoma peritonei as an intractable disease and its preoperative assessment to help improve prognosis after surgery: A review of the literature". Intractable Rare Dis Res. 1 (3): 115–21. doi:10.5582/irdr.2012.v1.3.115. PMC 4204597. PMID 25343082.
- ↑ Youssef H, Newman C, Chandrakumaran K, Mohamed F, Cecil TD, Moran BJ (March 2011). "Operative findings, early complications, and long-term survival in 456 patients with pseudomyxoma peritonei syndrome of appendiceal origin". Dis. Colon Rectum. 54 (3): 293–9. doi:10.1007/DCR.0b013e318202f026. PMID 21304299.
- ↑ Chua TC, Moran BJ, Sugarbaker PH, Levine EA, Glehen O, Gilly FN, Baratti D, Deraco M, Elias D, Sardi A, Liauw W, Yan TD, Barrios P, Gómez Portilla A, de Hingh IH, Ceelen WP, Pelz JO, Piso P, González-Moreno S, Van Der Speeten K, Morris DL (July 2012). "Early- and long-term outcome data of patients with pseudomyxoma peritonei from appendiceal origin treated by a strategy of cytoreductive surgery and hyperthermic intraperitoneal chemotherapy". J. Clin. Oncol. 30 (20): 2449–56. doi:10.1200/JCO.2011.39.7166. PMID 22614976.
- ↑ Smeenk RM, Bex A, Verwaal VJ, Horenblas S, Zoetmulder FA (January 2006). "Pseudomyxoma peritonei and the urinary tract: involvement and treatment related complications". J Surg Oncol. 93 (1): 20–3. doi:10.1002/jso.20427. PMID 16353186.
- ↑ 9.0 9.1 Wambura C, Jusabani A, Sherman O, Surani S (October 2018). "Pseudomyxoma pleurii and peritonei secondary to sigmoid colon adenocarcinoma: a rare clinico-pathologico-radiological presentation". Oxf Med Case Reports. 2018 (10): omy057. doi:10.1093/omcr/omy057. PMC 6142713. PMID 30250743.
- ↑ 10.0 10.1 Amini, Afshin; Masoumi-Moghaddam, Samar; Ehteda, Anahid; Morris, David (2014). "Secreted mucins in pseudomyxoma peritonei: pathophysiological significance and potential therapeutic prospects". Orphanet Journal of Rare Diseases. 9 (1): 71. doi:10.1186/1750-1172-9-71. ISSN 1750-1172.
- ↑ Lee NK, Kim S, Kim HS, Jeon TY, Kim GH, Kim DU, Park DY, Kim TU, Kang DH (November 2011). "Spectrum of mucin-producing neoplastic conditions of the abdomen and pelvis: cross-sectional imaging evaluation". World J. Gastroenterol. 17 (43): 4757–71. doi:10.3748/wjg.v17.i43.4757. PMC 3229624. PMID 22147976.
- ↑ Wrafter PF, Connelly T, Khan JS, Joyce WP (October 2015). "Pseudomyxoma peritonei diagnosed 19 years after appendicectomy". BMJ Case Rep. 2015. doi:10.1136/bcr-2015-211706. PMC 4620202. PMID 26494720.
- ↑ Khan S, Patel AG, Jurkovic D (April 2002). "Incidental ultrasound diagnosis of pseudomyxoma peritonei in an asymptomatic woman". Ultrasound Obstet Gynecol. 19 (4): 410–2. doi:10.1046/j.1469-0705.2002.00611.x. PMID 11952975.
- ↑ Qi ZH, Cai S, Li JC, L K, Zhang Y, Xiao MS, Su N (August 2015). "[Ultrasonographic Features of Pseudomyxoma Peritonei and TheirComparison with Computed Tomographic Findings]". Zhongguo Yi Xue Ke Xue Yuan Xue Bao (in Chinese). 37 (4): 424–9. doi:10.3881/j.issn.1000-503X.2015.04.010. PMID 26564459.
- ↑ Li Y, Guo A, Tang J, Wang L, Wang J, Yu D (September 2013). "Role of preoperative sonography in the diagnosis and pathologic staging of pseudomyxoma peritonei". J Ultrasound Med. 32 (9): 1565–70. doi:10.7863/ultra.32.9.1565. PMID 23980216.
- ↑ Mittal R, Chandramohan A, Moran B (August 2017). "Pseudomyxoma peritonei: natural history and treatment". Int J Hyperthermia. 33 (5): 511–519. doi:10.1080/02656736.2017.1310938. PMID 28540829.
- ↑ Smeenk RM, Verwaal VJ, Antonini N, Zoetmulder FA (January 2007). "Survival analysis of pseudomyxoma peritonei patients treated by cytoreductive surgery and hyperthermic intraperitoneal chemotherapy". Ann. Surg. 245 (1): 104–9. doi:10.1097/01.sla.0000231705.40081.1a. PMC 1867935. PMID 17197972.
- ↑ Harmon R, Sugarbaker P (2005). "Prognostic indicators in peritoneal carcinomatosis from gastrointestinal cancer". Int Semin Surg Oncol. 2 (1): 3. PMID 15701175.