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Ovarian Mucinous Cystic Tumor Associated with Pseudomyxoma Peritonei

MedGen UID:
309512
•Concept ID:
C1518726
•
Neoplastic Process

Definition

The presence of abundant mucoid or gelatinous, cellular or acellular material in the pelvis and abdominal cavity (pseudomyxoma peritonei) that is associated with the presence of an ovarian mucinous cystic tumor. In the vast majority of cases, the presence of pseudomyxoma peritonei is the result of metastasis from a primary appendiceal or gastrointestinal tumor site, and the ovarian mucinous tumor is metastatic rather than primary. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVOvarian Mucinous Cystic Tumor Associated with Pseudomyxoma Peritonei

Professional guidelines

PubMed

Mucinous appendiceal neoplasms: Incidence, diagnosis and surgical treatment.
Nutu OA, Marcacuzco Quinto AA, Manrique Municio A, Justo Alonso I, Calvo Pulido J, García-Conde M, Cambra Molero F, Jiménez Romero LC
Cir Esp 2017 Jun-Jul;95(6):321-327. Epub 2017 Jun 24 doi: 10.1016/j.ciresp.2017.05.008. PMID: 28655402
Mucinous borderline ovarian tumors: points of general agreement and persistent controversies regarding nomenclature, diagnostic criteria, and behavior.
Ronnett BM, Kajdacsy-Balla A, Gilks CB, Merino MJ, Silva E, Werness BA, Young RH
Hum Pathol 2004 Aug;35(8):949-60. doi: 10.1016/j.humpath.2004.03.006. PMID: 15297962
Diagnostic criteria and behavior of ovarian seromucinous (endocervical-type mucinous and mixed cell-type) tumors: atypical proliferative (borderline) tumors, intraepithelial, microinvasive, and invasive carcinomas.
Shappell HW, Riopel MA, Smith Sehdev AE, Ronnett BM, Kurman RJ
Am J Surg Pathol 2002 Dec;26(12):1529-41. doi: 10.1097/00000478-200212000-00001. PMID: 12459620

Recent clinical studies

Etiology

Ovarian tumors: a survey of selected advances of note during the life of this journal.
Young RH
Hum Pathol 2020 Jan;95:169-206. Epub 2019 Oct 22 doi: 10.1016/j.humpath.2019.09.002. PMID: 31654691
Mucinous appendiceal neoplasms: Incidence, diagnosis and surgical treatment.
Nutu OA, Marcacuzco Quinto AA, Manrique Municio A, Justo Alonso I, Calvo Pulido J, García-Conde M, Cambra Molero F, Jiménez Romero LC
Cir Esp 2017 Jun-Jul;95(6):321-327. Epub 2017 Jun 24 doi: 10.1016/j.ciresp.2017.05.008. PMID: 28655402
Pseudomyxoma peritonei: review on a cluster of peritoneal mucinous diseases.
Jacquemin G, Laloux P
Acta Chir Belg 2005 Apr;105(2):127-33. PMID: 15906901
Mucinous cystadenocarcinoma of the appendix. The controversy persists: a review.
Lo NS, Sarr MG
Hepatogastroenterology 2003 Mar-Apr;50(50):432-7. PMID: 12749241
Pseudomyxoma peritonei: new concepts in diagnosis, origin, nomenclature, and relationship to mucinous borderline (low malignant potential) tumors of the ovary.
Ronnett BM, Shmookler BM, Sugarbaker PH, Kurman RJ
Anat Pathol 1997;2:197-226. PMID: 9575376

Diagnosis

Peritoneal Pathology Review: Mullerian, Mucinous and Mesothelial Lesions.
Kiyokawa T
Surg Pathol Clin 2022 Jun;15(2):259-276. Epub 2022 May 18 doi: 10.1016/j.path.2022.02.005. PMID: 35715161
Pathologic diagnosis, origin, and natural history of pseudomyxoma peritonei.
Buell-Gutbrod R, Gwin K
Am Soc Clin Oncol Educ Book 2013:221-5. doi: 10.14694/EdBook_AM.2013.33.221. PMID: 23714507
Pathology of primary and metastatic mucinous ovarian neoplasms.
Leen SL, Singh N
J Clin Pathol 2012 Jul;65(7):591-5. Epub 2011 Nov 10 doi: 10.1136/jclinpath-2011-200162. PMID: 22075188
Mucinous cystadenoma of the appendix.
Iswariah H, Metcalfe M, Lituri D, Maddern GJ
ANZ J Surg 2004 Oct;74(10):918-9. doi: 10.1111/j.1445-1433.2004.03180.x. PMID: 15456452
Pseudomyxoma peritonei.
Harshen R, Jyothirmayi R, Mithal N
Clin Oncol (R Coll Radiol) 2003 Apr;15(2):73-7. doi: 10.1053/clon.2002.0163. PMID: 12708714

Therapy

Pseudomixoma peritonei, a rare entity difficult to diagnose and treat - case report.
Mavrodin C, Pariza G, Iordache V, Iorga P, Sajin M
Chirurgia (Bucur) 2014 Nov-Dec;109(6):846-9. PMID: 25560512
Cytoreductive surgery plus bicavitary chemohyperthermia as treatment of pseudomixoma peritonei with pleural extension. A case report and review of the literature.
Saladino E, Famulari C, La Monaca E, Fortugno A, Fleres F, Macrì A
Ann Ital Chir 2014 Jul-Aug;85(4):372-6. PMID: 25263588
Acute hyperglycemia following intraperitoneal irrigation with 10% dextrose in a patient with pseudomyxoma peritonei.
Roy WJ Jr, Thomas BL, Horowitz IR
Gynecol Oncol 1997 May;65(2):360-2. doi: 10.1006/gyno.1997.4657. PMID: 9159352
Pseudomyxoma peritonei: treatment with intraperitoneal 5-fluorouracil.
Nasr MF, Kemp GM, Given FT Jr
Eur J Gynaecol Oncol 1993;14(3):213-7. PMID: 8508878

Prognosis

Ovarian tumors: a survey of selected advances of note during the life of this journal.
Young RH
Hum Pathol 2020 Jan;95:169-206. Epub 2019 Oct 22 doi: 10.1016/j.humpath.2019.09.002. PMID: 31654691
Pathologic diagnosis, origin, and natural history of pseudomyxoma peritonei.
Buell-Gutbrod R, Gwin K
Am Soc Clin Oncol Educ Book 2013:221-5. doi: 10.14694/EdBook_AM.2013.33.221. PMID: 23714507
Pathology of primary and metastatic mucinous ovarian neoplasms.
Leen SL, Singh N
J Clin Pathol 2012 Jul;65(7):591-5. Epub 2011 Nov 10 doi: 10.1136/jclinpath-2011-200162. PMID: 22075188
Pseudomyxoma peritonei: review on a cluster of peritoneal mucinous diseases.
Jacquemin G, Laloux P
Acta Chir Belg 2005 Apr;105(2):127-33. PMID: 15906901
Borderline epithelial tumors of the ovary.
Hart WR
Mod Pathol 2005 Feb;18 Suppl 2:S33-50. doi: 10.1038/modpathol.3800307. PMID: 15761465

Clinical prediction guides

Ovarian tumors: a survey of selected advances of note during the life of this journal.
Young RH
Hum Pathol 2020 Jan;95:169-206. Epub 2019 Oct 22 doi: 10.1016/j.humpath.2019.09.002. PMID: 31654691
Mucinous appendiceal neoplasms: Incidence, diagnosis and surgical treatment.
Nutu OA, Marcacuzco Quinto AA, Manrique Municio A, Justo Alonso I, Calvo Pulido J, García-Conde M, Cambra Molero F, Jiménez Romero LC
Cir Esp 2017 Jun-Jul;95(6):321-327. Epub 2017 Jun 24 doi: 10.1016/j.ciresp.2017.05.008. PMID: 28655402
Pseudomyxoma peritonei of 92 Chinese patients: clinical characteristics, pathological classification and prognostic factors.
Guo AT, Li YM, Wei LX
World J Gastroenterol 2012 Jun 28;18(24):3081-8. doi: 10.3748/wjg.v18.i24.3081. PMID: 22791943Free PMC Article
Ovarian mature teratomas with mucinous epithelial neoplasms: morphologic heterogeneity and association with pseudomyxoma peritonei.
McKenney JK, Soslow RA, Longacre TA
Am J Surg Pathol 2008 May;32(5):645-55. doi: 10.1097/PAS.0b013e31815b486d. PMID: 18344868
Current issues in the pathology of ovarian cancer.
Rabban JT, Bell DA
J Reprod Med 2005 Jun;50(6):467-74. PMID: 16050571

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