Pseudomyxoma peritonei overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Parminder Dhingra, M.D. [2]
Overview
Pseudomyxoma peritonei is an uncommon tumor known for its production of mucus in the abdominal cavity.[1] The tumor is not harmful by itself, but the mucus has no place to go inside the abdominal cavity. If left untreated, it will eventually build up to the point where it compresses vital structures: the colon, the liver, kidneys, etc.
Historical Perspective
The first case of pseudomyxoma peritonei was described by Carl F. Rokitansky in 1842.[2]
Pathophysiology
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).
Causes
Pseudomyxoma peritonei is caused by a pre-existing intraperitoneal mucinous neoplasm. The K-Ras and p53 genes may be involved in the oncogenesis.
Differentiating Pseudomyxoma peritonei from other Diseases
Pseudomyxoma peritonei must be differentiated from peritoneal carcinomatosis without mucinous ascites, peritoneal sarcomatosis, and peritonitis.
Epidemiology and Demographics
The incidence of pseudomyxoma peritonei is approximately 0.5 to 1 per 100,000 individuals worldwide. Females are more commonly affected with pseudomyxoma peritonei than males. The median age at diagnosis is 50 years.
Risk Factors
There are no established risk factors for pseudomyxoma peritonei.
Natural History, Complications and Prognosis
If left untreated, patients with pseudomyxoma peritonei may progress to develop abdominal or pelvic pain, bloating, abdominal distension, weight change, and infertility. Prognosis is generally good and the 10-year survival rate of patients with pseudomyxoma peritonei is approximately 63%.
Diagnosis
History and Symptoms
Physical Examination
Laboratory Findings
CT
MRI
Ultrasound
Other Imaging Findings
Other Diagnostic Studies
Medical Therapy
Surgery
References
- ↑ Qu Z, Liu L (2006). "Management of pseudomyxoma peritonei". World J Gastroenterol. 12 (38): 6124–7. PMID 17036382.
- ↑ Pseudomyxoma peritonei. Wikipedia. https://en.wikipedia.org/wiki/Pseudomyxoma_peritonei. Accessed on December 2, 2015