Pseudomyxoma peritonei overview: Difference between revisions

Jump to navigation Jump to search
No edit summary
 
Line 49: Line 49:
==Surgery==
==Surgery==
[[Surgery]] is the mainstay of treatment for pseudomyxoma peritonei. The feasibility of [[surgery]] depends on the stage of pseudomyxoma peritonei at diagnosis.  
[[Surgery]] is the mainstay of treatment for pseudomyxoma peritonei. The feasibility of [[surgery]] depends on the stage of pseudomyxoma peritonei at diagnosis.  
== References ==
{{Reflist|2}}


[[Category:Types of cancer]]
[[Category:Types of cancer]]

Latest revision as of 20:14, 1 April 2019

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 overview On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Pseudomyxoma peritonei overview

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 overview

CDC on Pseudomyxoma peritonei overview

Pseudomyxoma peritonei overview in the news

Blogs on Pseudomyxoma peritonei overview

Directions to Hospitals Treating Pseudomyxoma peritonei

Risk calculators and risk factors for Pseudomyxoma peritonei overview

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Nima Nasiri, M.D.[2]

Overview

Pseudomyxoma peritonei is a very rare tumor characterized by the presence of abundant mucus in the abdominal cavity, consisting of intraperitoneal mucinous tumors and ascites, most commonly arising from mucinous tumors of the appendix, however, the ovary or the colon may be the primary site of origin. The tumor is not harmful by itself, but the mucus accumulation in abdominal cavity compresses vital organs such as spleen, kidney, colon. On 1842, Carl F. Rokitansky described the first case of pseudomyxoma peritonei. Pseudomyxoma peritonei may be divided into two pathological subtypes with different etiology and prognosis, these two subtypes are peritoneal adenomucinosis and peritoneal mucinous carcinoma. The K-Ras and p53 genes are involved in the oncogenesis. Pseudomyxoma peritonei must be differentiated from peritoneal carcinomatosis without mucinous ascites, peritoneal sarcomatosis, and tuberculosis peritonitis. The incidence of pseudomyxoma peritonei is approximately 0.1 person 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. Patients with pseudomyxoma peritonei may develop abdominal or pelvic pain, bloating, abdominal distension, weight change, and infertility symptoms if left untreated. Common physical examination findings of pseudomyxoma peritonei include abdominal tenderness, abdominal distention. On abdominal CT scan, pseudomyxoma peritonei is characterized by low attenuation, loculated ascitic mucinous fluid in the peritoneum, omentum and mesentery with scalloping of visceral surfaces of the liver. On abdominal MRI, pseudomyxoma peritonei is characterized by a mass which is hypointense on T1-weighted MRI and hyperintense on T2-weighted MRI. Finding on ultrasound scan suggestive of pseudomyxoma peritonei are echogenic peritoneal masses or ascites which is not mobile. Cytoreductive surgery combined with hyperthermic intraperitoneal chemoperfusion is the optimal way of treatment for pseudomyxoma peritonei.

Historical Perspective

The first case of pseudomyxoma peritonei was described by Carl F. Rokitansky in 1842.

Pathophysiology

Pseudomyxoma peritonei is a rare poorly understood neoplasm which may be divided into two pathological subtypes: Disseminated peritoneal adenomucinosis (DPAM), and Peritoneal mucinous carcinomatosis (PMCA).

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

Differential diagnosis of pseudomyxoma peritonei include peritoneal carcinomatosis without mucinous ascites, peritoneal sarcomatosis, Peritonitis, peritoneal mesothelioma.

Epidemiology and Demographics

The incidence of pseudomyxoma peritonei is approximately 0.1 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

Patients with pseudomyxoma peritonei may 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%.Overall risk of complications after surgery such as urinary tract involvement due to tumor seeding in pelvis is very low.

History and Symptoms

Symptoms of pseudomyxoma peritonei include: abdominal pain, bloating, abdominal distention, Shortness of breath, Weight change, Menstrual irregularities in women.

Physical Examination

Common physical examination findings of pseudomyxoma peritonei include: Abdominal tenderness, Abdominal distention.

Laboratory Findings

The laboratory findings associated with pseudomyxoma peritonei include: increased levels of tumor markers such as carcinoembryonic antigen (CEA), carbohydrate antigen 19-9 (CA19-9), carbohydrate antigen 125 (CA125).

CT

Findings on abdominal CT scan of patients with pseudomyxoma peritonei include: accumulation of fluid throughout the peritoneum, omentum and mesentery seen as low attenuation mucin depositis on CT scan, scalloping of visceral organs.

MRI

On abdominal MRI, pseudomyxoma peritonei is characterized by a mass which is hypointense on T1-weighted MRI and hyperintense on T2-weighted MRI, it can further clarify the cystic structure of mass.

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 and debulking. Supportive therapy for pseudomyxoma peritonei includes chemotherapy which can be used as adjuvant treatment. The treatment of choice is complete tumor excision named cytoreductive surgery (CRS) combined with Hyperthermic intraperitoneal chemoperfusion (HIPEC).

Surgery

Surgery is the mainstay of treatment for pseudomyxoma peritonei. The feasibility of surgery depends on the stage of pseudomyxoma peritonei at diagnosis.


Template:WikiDoc Sources