Pseudomyxoma peritonei pathophysiology: Difference between revisions

	Pseudomyxoma peritonei Microchapters
Home
Patient Information
Overview
Historical Perspective
Pathophysiology
Causes
Differentiating Pseudomyxoma peritonei from other Diseases
Epidemiology and Demographics
Risk Factors
Screening
Natural History, Complications and Prognosis
Diagnosis
History and Symptoms
Physical Examination
Laboratory Findings
Electrocardiogram
CT
MRI
Ultrasound
Other Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
Surgery
Primary Prevention
Secondary Prevention
Cost-Effectiveness of Therapy
Future or Investigational Therapies
Case Studies
Case #1
Pseudomyxoma peritonei pathophysiology On the Web
Most recent articles
Most cited articles
Review articles
CME Programs
Powerpoint slides
Images
American Roentgen Ray Society Images of Pseudomyxoma peritonei pathophysiology All Images X-rays Echo & Ultrasound CT Images MRI
Ongoing Trials at Clinical Trials.gov
US National Guidelines Clearinghouse
NICE Guidance
FDA on Pseudomyxoma peritonei pathophysiology
CDC on Pseudomyxoma peritonei pathophysiology
Pseudomyxoma peritonei pathophysiology in the news
Blogs on Pseudomyxoma peritonei pathophysiology
Directions to Hospitals Treating Pseudomyxoma peritonei
Risk calculators and risk factors for Pseudomyxoma peritonei pathophysiology

VisualWikitext

Latest revision as of 20:30, 1 April 2019

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 tumor known for its production of abundant mucinous ascites in the abdominal cavity. It can have a mass impact on vital organs such as spleen, pancreas, and kidney. Pseudomyxoma peritonei may be divided into two pathological subtypes: peritoneal adenomucinosis and peritoneal mucinous carcinoma.

Pathogenesis

The pathogenesis of the disease is related to biomarkers and molecular genetic alterations.

Immunohistochemical markers and genetic alterations involved in the pathogenesis of pseudomyxoma peritonei include:^[1]
- CK 20
- CDX2 and MUC2 are found to be positive in these tumors.
- KRAS mutation and loss of heterozygosity in some gene loci.
- Losses of alleles in chromosomes 18q, 17p, 5q.

Pathology

Pseudomyxoma peritonei may be divided into two pathological subtypes:^[2]

Disseminated peritoneal adenomucinosis (DPAM) which has charracteristic features of the following:
- Peritoneal lesions with abundant extracellular mucin
- Proliferative mucinous epithelium
- Less mitotic activity as compared with peritoneal mucinous carcinomatosis
Peritoneal mucinous carcinomatosis (PMCA) which is characterized by:
- Peritoneal lesions having more abundant mucinous epithelium
- Characteristic cytologic features of carcinoma, with high mitotic activity.

Immunohistology

Immunohistochemical markers can help identify the organ of origin.

The tumor is positive for cytokeratin 20 (CK20), CEA, caudal-type homeobox protein 2 (CDX-2), and MUC2 as well as negative cytokeratin 7 (CK7) and CA125.
Studies have shown that mucin MUC2 and MUC5AC is extensively positive in pseudomyxoma peritonei patients. ^[3]

References

↑ Szych C, Staebler A, Connolly DC, Wu R, Cho KR, Ronnett BM (June 1999). "Molecular genetic evidence supporting the clonality and appendiceal origin of Pseudomyxoma peritonei in women". Am. J. Pathol. 154 (6): 1849–55. doi:10.1016/S0002-9440(10)65442-9. PMC 1866622. PMID 10362811.
↑ Ronnett BM, Zahn CM, Kurman RJ, Kass ME, Sugarbaker PH, Shmookler BM (December 1995). "Disseminated peritoneal adenomucinosis and peritoneal mucinous carcinomatosis. A clinicopathologic analysis of 109 cases with emphasis on distinguishing pathologic features, site of origin, prognosis, and relationship to "pseudomyxoma peritonei"". Am. J. Surg. Pathol. 19 (12): 1390–408. PMID 7503361.
↑ Nonaka D, Kusamura S, Baratti D, Casali P, Younan R, Deraco M (October 2006). "CDX-2 expression in pseudomyxoma peritonei: a clinicopathological study of 42 cases". Histopathology. 49 (4): 381–7. doi:10.1111/j.1365-2559.2006.02512.x. PMID 16978201.

Template:WikiDoc Sources

[pmid10362811-1] Szych C, Staebler A, Connolly DC, Wu R, Cho KR, Ronnett BM (June 1999). "Molecular genetic evidence supporting the clonality and appendiceal origin of Pseudomyxoma peritonei in women". Am. J. Pathol. 154 (6): 1849–55. doi:10.1016/S0002-9440(10)65442-9. PMC 1866622. PMID 10362811.

[pmid7503361-2] Ronnett BM, Zahn CM, Kurman RJ, Kass ME, Sugarbaker PH, Shmookler BM (December 1995). "Disseminated peritoneal adenomucinosis and peritoneal mucinous carcinomatosis. A clinicopathologic analysis of 109 cases with emphasis on distinguishing pathologic features, site of origin, prognosis, and relationship to "pseudomyxoma peritonei"". Am. J. Surg. Pathol. 19 (12): 1390–408. PMID 7503361.

[pmid16978201-3] Nonaka D, Kusamura S, Baratti D, Casali P, Younan R, Deraco M (October 2006). "CDX-2 expression in pseudomyxoma peritonei: a clinicopathological study of 42 cases". Histopathology. 49 (4): 381–7. doi:10.1111/j.1365-2559.2006.02512.x. PMID 16978201.

[1]

[2]

[3]

@@ Line 1: / Line 1: @@
 __NOTOC__
 {{Pseudomyxoma peritonei}}
-{{CMG}}{{AE}}{{PSD}}
+{{CMG}}{{AE}}{{Nnasiri}}
 ==Overview==
-The remarkable feature of pseudomyxoma peritonei is that this neoplastic, progressive process often arises from a seemingly benign or well differentiated primary tumor. Pseudomyxoma peritonei may be divided into two pathological subtypes which have aetiological and prognostic significance ( Peritoneal adenomucinosis and Peritoneal mucinous carcinoma).
+Pseudomyxoma peritonei (PMP) is a rare [[tumor]] known for its production of abundant [[mucinous]] [[ascites]] in the [[abdominal cavity]]. It can have a mass impact on vital organs such as [[spleen]], [[pancreas]], and [[kidney]]. Pseudomyxoma peritonei may be divided into two [[pathological]] subtypes: [[peritoneal]] adenomucinosis and [[Peritoneal mucinous carcinomatosis|peritoneal mucinous carcinoma.]]
 ==Pathogenesis==
-* The pathological process starts with neoplastic transformation of the appendiceal goblet cells and subsequent formation of a primary mucinous [[tumor]].
+The pathogenesis of the disease is related to [[biomarkers]] and molecular [[genetic]] alterations.
-* While proliferating, tumor cells maintain their constitutive level of mucin expression. As a result, the overall secretion of mucin dramatically rises.
+*[[Immunohistochemistry|Immunohistochemica]]<nowiki/>[[Immunohistochemistry|l markers]] and [[genetic]] alterations involved in the [[pathogenesis]] of pseudomyxoma peritonei include:<ref name="pmid10362811">{{cite journal |vauthors=Szych C, Staebler A, Connolly DC, Wu R, Cho KR, Ronnett BM |title=Molecular genetic evidence supporting the clonality and appendiceal origin of Pseudomyxoma peritonei in women |journal=Am. J. Pathol. |volume=154 |issue=6 |pages=1849–55 |date=June 1999 |pmid=10362811 |pmc=1866622 |doi=10.1016/S0002-9440(10)65442-9 |url=}}</ref>
-* This is followed by intraluminal accumulation of mucin and eventual development of an appendiceal [[mucocele]].
+**CK 20
-* A small perforation or rupture of the mucocele is the key event towards the development of pseudomyxoma peritonei through which tumor cells gain access into the peritoneal cavity.
+**[[CDX2]] and [[MUC2]] are found to be positive in these [[Tumor|tumors]].
-* Lacking cell surface adhesion molecules, the exfoliated tumor cells passively circulate with the peritoneal fluid and redistribute throughout the peritoneal cavity.
+**[[KRAS]] [[Mutations|mutation]] and [[loss of heterozygosity]] in some [[Loci|gene loci]].
-* As a result, tumor implants and mucin collections form at the peritoneal fluid re-absorption sites as well as within the dependent portions of the peritoneal cavity to create pseudomyxoma peritonei’s characteristic pattern of the peritoneal dissemination.
+**Losses of [[alleles]] in [[chromosomes]] [[18q syndrome|18q]], 17p, 5q.
-* Accumulating mucin increases intraabdominal pressure and compresses visceral organs.
-* Furthermore, extensive involvement of the peritoneal surface promotes variable inflammatory and fibrotic responses in the peritoneal environment and hence the development of bowel obstruction as a fatal complication of the disease.<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>
 ==Pathology==
-* The remarkable feature of pseudomyxoma peritonei is that this neoplastic, progressive process often arises from a seemingly benign or well differentiated primary tumor.
+Pseudomyxoma peritonei may be divided into two pathological subtypes:<ref name="pmid7503361">{{cite journal |vauthors=Ronnett BM, Zahn CM, Kurman RJ, Kass ME, Sugarbaker PH, Shmookler BM |title=Disseminated peritoneal adenomucinosis and peritoneal mucinous carcinomatosis. A clinicopathologic analysis of 109 cases with emphasis on distinguishing pathologic features, site of origin, prognosis, and relationship to "pseudomyxoma peritonei" |journal=Am. J. Surg. Pathol. |volume=19 |issue=12 |pages=1390–408 |date=December 1995 |pmid=7503361 |doi= |url=}}</ref>
-* Pseudomyxoma peritonei may be divided into two pathological subtypes which have aetiological and prognostic significance:
+*[[Disseminated peritoneal adenomucinosis]] (DPAM) which has charracteristic features of the following:
-** Peritoneal adenomucinosis
+**[[Peritoneal]] lesions with abundant [[extracellular]] [[mucin]]
-** Peritoneal mucinous carcinoma
+**Proliferative [[mucinous]] [[epithelium]]
-* Not all cases may exactly fit into these categories where many of the patients have intermediate or discordant features.
+**Less [[mitotic]] activity as compared with [[Peritoneum|peritoneal]] [[mucinous]] [[carcinomatosis]]
+*[[Peritoneal mucinous carcinomatosis]] (PMCA) which is characterized by:
-===Peritoneal adenomucinosis===
+**[[Peritoneal]] lesions having more abundant [[mucinous]] [[epithelium]]
-*A peritoneal neoplasm composed largely of mucin associated with fibrosis with minimal cytologic atypia and mitoses.
+**Characteristic [[Cytological|cytologic]] features of [[carcinoma]], with high [[Mitotic|mitotic activity]].
-*The primary tumor is generally an adenoma.
-===Peritoneal mucinous carcinoma===
-*A peritoneal neoplasm characterised by proliferative epithelium, glands, nests, or individual cells with marked cytologic atypia.
-*The primary tumor is a mucinous adenocarcinoma.
 ==Immunohistology==
-Immunohistochemical markers can help identify the organ of origin. These include positive cytokeratin 20 (CK20), CEA, caudal-type homeobox protein 2 (CDX-2), and MUC2 as well as negative cytokeratin 7 (CK7) and CA125. Of particular interest is the secreted mucin MUC2 that is extensively positive in pseudomyxoma peritonei patients. Although MUC2 has been suggested as a biological marker of pseudomyxoma peritonei, its significance as a prognostic factor is a matter of controversy.
+[[Immunohistochemistry|Immunohistochemical markers]] can help identify the organ of origin.
+*The tumor is positive for [[cytokeratin]] 20 (CK20), [[CEA]], caudal-type homeobox protein 2 (CDX-2), and [[MUC2]] as well as negative [[cytokeratin]] 7 (CK7) and [[CA125]].
+*Studies have shown that mucin [[MUC2]] and MUC5AC is extensively positive in pseudomyxoma peritonei patients. <ref name="pmid16978201">{{cite journal |vauthors=Nonaka D, Kusamura S, Baratti D, Casali P, Younan R, Deraco M |title=CDX-2 expression in pseudomyxoma peritonei: a clinicopathological study of 42 cases |journal=Histopathology |volume=49 |issue=4 |pages=381–7 |date=October 2006 |pmid=16978201 |doi=10.1111/j.1365-2559.2006.02512.x |url=}}</ref>
 == References ==
-{{Reflist|1}}
+{{Reflist|2}}
 [[Category:Types of cancer]]
@@ Line 41: / Line 37: @@
 {{WikiDoc Help Menu}}
 {{WikiDoc Sources}}
+ [[Category:Up-To-Date]]
+[[Category:Oncology]]
+[[Category:Medicine]]
+[[Category:Gastroenterology]]
+[[Category:Surgery]]