Pseudomyxoma peritonei pathophysiology

Jump to navigation Jump to search

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

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

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).

Pathogenesis

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

  • Immunohistochemical markers 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

  • 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
    • Peritoneal mucinous carcinoma
  • Not all cases may exactly fit into these categories where many of the patients have intermediate or discordant features.

Peritoneal adenomucinosis

  • A peritoneal neoplasm composed largely of mucin associated with fibrosis with minimal cytologic atypia and mitoses.
  • 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.

References

  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.


Template:WikiDoc Sources