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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. The first case of pseudomyxoma peritonei was described by Carl F. Rokitansky in 1842.[2] 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 is caused by a pre-existing intraperitoneal mucinous neoplasm. The K-Ras and p53 genes may be involved in the oncogenesis. Pseudomyxoma peritonei must be differentiated from peritoneal carcinomatosis without mucinous ascites, peritoneal sarcomatosis, and peritonitis. 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. There are no established risk factors for pseudomyxoma peritonei. 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%. Symptoms of pseudomyxoma peritonei include abdominal pain, bloating, abdominal distention, weight changes, and infertility. Common physical examination findings of pseudomyxoma peritonei include abdominal tenderness, abdominal distention, and ulcers. On abdominal CT scan, pseudomyxoma peritonei is characterized by low attenuation, often loculated fluid throughout the peritoneum, omentum and mesentery with scalloping of visceral surfaces, particularly the liver.[3] Abdominal MRI is helpful in the diagnosis of pseudomyxoma peritonei. On abdominal MRI, pseudomyxoma peritonei is characterized by a mass which is hypointense on T1-weighted MRI and hyperintense on T2-weighted MRI.[4] Abdominal ultrasound scan may be helpful in the diagnosis of pseudomyxoma peritonei. Finding on ultrasound scan suggestive of pseudomyxoma peritonei are echogenic peritoneal masses or ascites with echogenic particles which do not move. The predominant therapy for pseudomyxoma peritonei is surgical resection. Supportive therapy for pseudomyxoma peritonei includes chemotherapy. Chemotherapy is infused directly into the abdominal cavity to kill cancerous cells. Surgery is the mainstay of treatment for pseudomyxoma peritonei. The feasibility of surgery depends on the stage of pseudomyxoma peritonei at diagnosis.[4][5]
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%.
History and Symptoms
Symptoms of pseudomyxoma peritonei include abdominal pain, bloating, abdominal distention, weight changes, and infertility.
Physical Examination
Common physical examination findings of pseudomyxoma peritonei include abdominal tenderness, abdominal distention, and ulcers.
Laboratory Findings
The laboratory findings associated with pseudomyxoma peritonei are increased levels of tumor markers such as carcinoembryonic antigen (CEA), carbohydrate antigen 19.9 (CA19.9), carbohydrate antigen 125 (CA125).[4]
CT
On abdominal CT scan, pseudomyxoma peritonei is characterized by a low attenuation, often loculated fluid throughout the peritoneum, omentum and mesentery with scalloping of visceral surfaces, particularly the liver.[3]
MRI
Abdominal MRI is helpful in the diagnosis of pseudomyxoma peritonei. On abdominal MRI, pseudomyxoma peritonei is characterized by a mass which is hypointense on T1-weighted MRI and hyperintense on T2-weighted MRI.[4]
Ultrasound
Abdominal ultrasound scan may be helpful in the diagnosis of pseudomyxoma peritonei. Finding on ultrasound scan suggestive of pseudomyxoma peritonei are echogenic peritoneal masses or ascites with echogenic particles which do not move.
Medical Therapy
The predominant therapy for pseudomyxoma peritonei is surgical resection. Supportive therapy for pseudomyxoma peritonei includes chemotherapy. Chemotherapy is infused directly into the abdominal cavity to kill cancerous cells.
Surgery
Surgery is the mainstay of treatment for pseudomyxoma peritonei. The feasibility of surgery depends on the stage of pseudomyxoma peritonei at diagnosis.[4][5]
References
- ↑ Qu Z, Liu L (2006). "Management of pseudomyxoma peritonei". World J Gastroenterol. 12 (38): 6124–7. PMID 17036382.
- ↑ 2.0 2.1 Pseudomyxoma peritonei. Wikipedia. https://en.wikipedia.org/wiki/Pseudomyxoma_peritonei. Accessed on December 2, 2015
- ↑ 3.0 3.1 Pseudomyxoma peritonei. Radiopaedia. http://radiopaedia.org/articles/pseudomyxoma-peritonei. Accessed on 19 November,2015.
- ↑ 4.0 4.1 4.2 4.3 4.4 Amini, Afshin; Masoumi-Moghaddam, Samar; Ehteda, Anahid; Morris, David (2014). "Secreted mucins in pseudomyxoma peritonei: pathophysiological significance and potential therapeutic prospects". Orphanet Journal of Rare Diseases. 9 (1): 71. doi:10.1186/1750-1172-9-71. ISSN 1750-1172.
- ↑ 5.0 5.1 Harmon R, Sugarbaker P (2005). "Prognostic indicators in peritoneal carcinomatosis from gastrointestinal cancer". Int Semin Surg Oncol. 2 (1): 3. PMID 15701175.