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Pseudomyxoma peritonei

MedGen UID:
18726
Concept ID:
C0033822
Neoplastic Process
Synonyms: Gelatinous ascites; Syndrome of pseudomyxoma peritonei
SNOMED CT: Pseudomyxoma peritonei (112679004); Pseudomyxoma peritonei (307601000)
Modes of inheritance:
Unknown inheritance
MedGen UID:
989040
Concept ID:
CN307042
Finding
Source: Orphanet
Hereditary clinical entity whose mode of inheritance is unknown.
 
HPO: HP:0040414
Monarch Initiative: MONDO:0017048
Orphanet: ORPHA26790

Definition

A type of ascites characterized by copious amounts of mucinous ascites and mucinous peritoneal implants, leading to progressive obliteration of the peritoneal cavity and intestinal obstruction. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPseudomyxoma peritonei
Follow this link to review classifications for Pseudomyxoma peritonei in Orphanet.

Professional guidelines

PubMed

Govaerts K, Lurvink RJ, De Hingh IHJT, Van der Speeten K, Villeneuve L, Kusamura S, Kepenekian V, Deraco M, Glehen O, Moran BJ; PSOGI
Eur J Surg Oncol 2021 Jan;47(1):11-35. Epub 2020 Feb 28 doi: 10.1016/j.ejso.2020.02.012. PMID: 32199769
Chicago Consensus Working Group
Cancer 2020 Jun 1;126(11):2525-2533. Epub 2020 Apr 13 doi: 10.1002/cncr.32881. PMID: 32282073
Carr NJ, Bibeau F, Bradley RF, Dartigues P, Feakins RM, Geisinger KR, Gui X, Isaac S, Milione M, Misdraji J, Pai RK, Rodriguez-Justo M, Sobin LH, van Velthuysen MF, Yantiss RK
Histopathology 2017 Dec;71(6):847-858. Epub 2017 Sep 19 doi: 10.1111/his.13324. PMID: 28746986

Recent clinical studies

Etiology

Sugarbaker PH, Chang D
Eur J Surg Oncol 2022 Dec;48(12):2369-2377. Epub 2022 Jul 31 doi: 10.1016/j.ejso.2022.07.018. PMID: 35941031
Govaerts K, Lurvink RJ, De Hingh IHJT, Van der Speeten K, Villeneuve L, Kusamura S, Kepenekian V, Deraco M, Glehen O, Moran BJ; PSOGI
Eur J Surg Oncol 2021 Jan;47(1):11-35. Epub 2020 Feb 28 doi: 10.1016/j.ejso.2020.02.012. PMID: 32199769
Chicago Consensus Working Group
Cancer 2020 Jun 1;126(11):2525-2533. Epub 2020 Apr 13 doi: 10.1002/cncr.32881. PMID: 32282073
Carr NJ, Cecil TD, Mohamed F, Sobin LH, Sugarbaker PH, González-Moreno S, Taflampas P, Chapman S, Moran BJ; Peritoneal Surface Oncology Group International
Am J Surg Pathol 2016 Jan;40(1):14-26. doi: 10.1097/PAS.0000000000000535. PMID: 26492181
Smeenk RM, Bruin SC, van Velthuysen ML, Verwaal VJ
Curr Probl Surg 2008 Aug;45(8):527-75. doi: 10.1067/j.cpsurg.2008.04.003. PMID: 18590843

Diagnosis

Juárez de la Garza N, Juárez-Durán J, Gómez-Rodríguez IE, Rivera-Silva G
Med Clin (Barc) 2023 Dec 7;161(11):506. Epub 2023 Jul 7 doi: 10.1016/j.medcli.2023.05.015. PMID: 37423876
Shukla J, Sharma V
J R Coll Physicians Edinb 2023 Sep;53(3):184-185. Epub 2023 Jun 17 doi: 10.1177/14782715231179254. PMID: 37329264
Govaerts K, Lurvink RJ, De Hingh IHJT, Van der Speeten K, Villeneuve L, Kusamura S, Kepenekian V, Deraco M, Glehen O, Moran BJ; PSOGI
Eur J Surg Oncol 2021 Jan;47(1):11-35. Epub 2020 Feb 28 doi: 10.1016/j.ejso.2020.02.012. PMID: 32199769
Carr NJ, Cecil TD, Mohamed F, Sobin LH, Sugarbaker PH, González-Moreno S, Taflampas P, Chapman S, Moran BJ; Peritoneal Surface Oncology Group International
Am J Surg Pathol 2016 Jan;40(1):14-26. doi: 10.1097/PAS.0000000000000535. PMID: 26492181
Smeenk RM, Verwaal VJ, Zoetmulder FA
Cancer Treat Rev 2007 Apr;33(2):138-45. Epub 2006 Dec 19 doi: 10.1016/j.ctrv.2006.11.001. PMID: 17182192

Therapy

Rodríguez-Ortiz L, Vázquez-Borrego MC, Bura FI, Granados-Rodríguez M, Valenzuela-Molina F, Rufián-Andújar B, Martínez-López A, Ortega-Salas R, Espejo-Herrero JJ, Romero-Ruiz A, Arjona-Sánchez Á
Br J Surg 2024 Mar 2;111(3) doi: 10.1093/bjs/znae045. PMID: 38477594
Govaerts K, Lurvink RJ, De Hingh IHJT, Van der Speeten K, Villeneuve L, Kusamura S, Kepenekian V, Deraco M, Glehen O, Moran BJ; PSOGI
Eur J Surg Oncol 2021 Jan;47(1):11-35. Epub 2020 Feb 28 doi: 10.1016/j.ejso.2020.02.012. PMID: 32199769
Kahn AB, Tulla KA, Tzvetanov IG
Gastroenterol Clin North Am 2019 Dec;48(4):575-583. Epub 2019 Oct 4 doi: 10.1016/j.gtc.2019.08.010. PMID: 31668184
García KM, Flores KM, Ruiz A, González FL, Rodríguez ÁM
J Gastrointest Cancer 2019 Dec;50(4):1037-1042. doi: 10.1007/s12029-018-00192-8. PMID: 30618002
Sugarbaker PH, Ronnett BM, Archer A, Averbach AM, Bland R, Chang D, Dalton RR, Ettinghausen SE, Jacquet P, Jelinek J, Koslowe P, Kurman RJ, Shmookler B, Stephens AD, Steves MA, Stuart OA, White S, Zahn CM, Zoetmulder FA
Adv Surg 1996;30:233-80. PMID: 8960339

Prognosis

Sugarbaker PH, Chang D
Eur J Surg Oncol 2022 Dec;48(12):2369-2377. Epub 2022 Jul 31 doi: 10.1016/j.ejso.2022.07.018. PMID: 35941031
Sugarbaker PH
Surg Oncol Clin N Am 2018 Jul;27(3):413-424. doi: 10.1016/j.soc.2018.02.001. PMID: 29935680
Carr NJ, Cecil TD, Mohamed F, Sobin LH, Sugarbaker PH, González-Moreno S, Taflampas P, Chapman S, Moran BJ; Peritoneal Surface Oncology Group International
Am J Surg Pathol 2016 Jan;40(1):14-26. doi: 10.1097/PAS.0000000000000535. PMID: 26492181
Smeenk RM, Bruin SC, van Velthuysen ML, Verwaal VJ
Curr Probl Surg 2008 Aug;45(8):527-75. doi: 10.1067/j.cpsurg.2008.04.003. PMID: 18590843
Parsons J, Gray GF, Thorbjarnarson B
Arch Surg 1970 Nov;101(5):545-9. doi: 10.1001/archsurg.1970.01340290001001. PMID: 5479698

Clinical prediction guides

Kusamura S, Barretta F, Yonemura Y, Sugarbaker PH, Moran BJ, Levine EA, Goere D, Baratti D, Nizri E, Morris DL, Glehen O, Sardi A, Barrios P, Quénet F, Villeneuve L, Gómez-Portilla A, de Hingh I, Ceelen W, Pelz JOW, Piso P, González-Moreno S, Van Der Speeten K, Deraco M; Peritoneal Surface Oncology Group International (PSOGI) and the French National Registry of Rare Peritoneal Surface Malignancies (RENAPE)
JAMA Surg 2021 Mar 1;156(3):e206363. Epub 2021 Mar 10 doi: 10.1001/jamasurg.2020.6363. PMID: 33502455Free PMC Article
Carr NJ, Cecil TD, Mohamed F, Sobin LH, Sugarbaker PH, González-Moreno S, Taflampas P, Chapman S, Moran BJ; Peritoneal Surface Oncology Group International
Am J Surg Pathol 2016 Jan;40(1):14-26. doi: 10.1097/PAS.0000000000000535. PMID: 26492181
Honoré C, Caruso F, Dartigues P, Benhaim L, Chirica M, Goéré D, Elias D
Anticancer Res 2015 Sep;35(9):4943-7. PMID: 26254392
Brueggen C, Baird G, Meisheid A
Clin J Oncol Nurs 2007 Aug;11(4):525-32. doi: 10.1188/07.CJON.525-532. PMID: 17723965
Madwed D, Mindelzun R, Jeffrey RB Jr
AJR Am J Roentgenol 1992 Jul;159(1):69-72. doi: 10.2214/ajr.159.1.1609724. PMID: 1609724

Recent systematic reviews

Murage NW, Ahmed NM, Underwood TJ, Walters ZS, Breininger SP
Cancer Metastasis Rev 2023 Mar;42(1):335-359. Epub 2023 Feb 1 doi: 10.1007/s10555-023-10088-0. PMID: 36723696Free PMC Article
Floriano I, Silvinato A, Reis JC, Cafalli C, Bernardo WM
Clinics (Sao Paulo) 2022;77:100039. Epub 2022 May 13 doi: 10.1016/j.clinsp.2022.100039. PMID: 35576869Free PMC Article
Stein A, Strong E, Clark Gamblin T, Clarke C, Tsai S, Thomas J, George B, Mogal H
Ann Surg Oncol 2020 Jan;27(1):85-97. Epub 2019 Oct 3 doi: 10.1245/s10434-019-07879-7. PMID: 31583543
Tempfer CB, Solass W, Reymond MA
Wien Med Wochenschr 2014 Dec;164(23-24):519-28. Epub 2014 Sep 24 doi: 10.1007/s10354-014-0312-y. PMID: 25249025
McBride K, McFadden D, Osler T
J Surg Res 2013 Jul;183(1):246-52. Epub 2013 Jan 10 doi: 10.1016/j.jss.2012.12.040. PMID: 23490141

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