Peritoneal carcinomatosis

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

Synonyms and keywords: Peritoneal metastases; Peritoneal seeding

Overview[edit]

Peritoneal carcinomatosis (also known as peritoneal metastases) is defined as a malignant tumor of the peritoneum. Metastases are the most common peritoneal malignancy, commonly metastasize from ovarian cancer, colon cancer, gastric cancer, and pancreatic cancer. Calcified peritoneal carcinomatosis may occur in serous ovarian adenocarcinoma, colon cancer, and gastric cancer. Peritoneal carcinogenesis arises from the celomic epithelium lining of the abdominal cavity (peritoneum) in response to an oncogenic stimulus. Common causes of peritoneal carcinomatosis, include: peritoneal mesothelioma, colon cancer, gastric cancer, and pancreatic cancer. Early clinical features include abdominal pain, abdominal distension, and nausea. If left untreated, the majority of patients with peritoneal carcinomatosis may progress to develop portal hypertension, pulmonary edema, and death. Common complications of peritoneal carcinomatosis include intestinal obstruction and pulmonary thromboembolism. The diagnosis of peritoneal carcinomatosis, include: Imaging findings compatible with peritoneal carcinomatosis, elevated protein concentration (more than 4.0 g/dL) in ascitic fluid, abnormal serum-ascites albumin gradient (less than 1.1 g/dL) in ascitic fluid, and high cell count (lymphocyte predominance). The mainstay medical therapy for peritoneal carcinomatosis is hyperthermic intraperitoneal chemotherapy (HIPEC), followed by cytoreductive surgery, laparotomy in conjunction with cytology testing is the most common approach for the treatment of peritoneal carcinomatosis. Prognosis is generally poor, and the 5-year survival rate of patients with peritoneal carcinomatosis is approximately 20%.

Historical Perspective[edit]

  • Peritoneal carcinomatosis was first described by Swerdlow in 1959.<ref name="PPP">Swerdlow M: Mesothelioma of the pelvic peritoneum resembling papillary cystadenocarcinoma of the ovary: Case report. Am J Obstet Gynecol 77:200, 1959.</ref>

Classification[edit]

According to the Gilly classification, peritoneal carcinomatosis may be classified according to nodule size and intraperitoneal involvement (localized or diffuse) into 4 categories:<ref name="pmid20424420">{{#invoke:Citation/CS1|citation |CitationClass=journal }}</ref>

  • 0 - No macroscopic disease
  • 1 - Malignant granulations less than 5 mm in diameter localized in one part of the abdomen
  • 2 - Malignant granulations less than 5 mm in diameter diffuse to the whole abdomen
  • 3 - Localized or diffuse malignant granulations 5–20 mm in diameter
  • 4 - Localized or diffuse large malignant masses (more than 2 cm in diameter)

Pathophysiology[edit]

  • The pathogenesis of peritoneal carcinomatosis is characterized by the malignant seeding of a tumor in the peritoneal cavity.<ref name="pmid10228488">{{#invoke:Citation/CS1|citation

|CitationClass=journal }}</ref>

  • Peritoneal carcinogenesis arises from the celomic epithelium lining of the abdominal cavity (peritoneum) in response to an oncogenic stimulus.<ref name="pmid10228488">{{#invoke:Citation/CS1|citation

|CitationClass=journal }}</ref>

  • The mutation on BRCA1/BRCA2 has been associated with the development of peritoneal carcinomatosis.
  • On gross pathology, characteristic findings of peritoneal carcinomatosis, include:<ref name="wiki">Peritoneum. Libre patholgy. https://librepathology.org/wiki/Peritoneum Accessed on April 7, 2016</ref>
    • Multilocular thin-walled cysts containing serous fluid
    • Occasionally unilocular
    • May be up to 15 cm
    • Adherent to the surface
  • On microscopic histopathological analysis, characteristic findings of peritoneal carcinomatosis, include:<ref name="wiki">Peritoneum. Libre patholgy. https://librepathology.org/wiki/Peritoneum Accessed on April 7, 2016</ref>

Causes[edit]

Common causes of peritoneal carcinomatosis, include:<ref name="wiki">Peritoneal carcinomatosis. Wikipedia. https://en.wikipedia.org/wiki/Primary_peritoneal_carcinoma Accessed on April 7, 2016</ref>

Peritoneal carcinomatosis may also be caused by a mutation in the BCRA1 or BCRA2 genes.

Differentiating Peritoneal Carcinomatosis from Other Diseases[edit]

Peritoneal carcinomatosis must be differentiated from other diseases that cause abdominal pain, ascites, and weight loss, such as:<ref name="wiki">Peritoneal metastases. Dr Henry Knipe. Radiopedia http://radiopaedia.org/articles/peritoneal-metastases Accessed on April 7, 2016</ref>

Differentiating peritoneal carcinomatosis from other diseases
Disease Prominent clinical findings Lab tests Tratment
Primary peritonitis Spontaneous bacterial peritonitis
Tuberculous peritonitis
Continuous Ambulatory Peritoneal Dialysis (CAPD peritonitis)
Secondary peritonitis Acute bacterial secondary peritonitis
Biliary peritonitis
Tertiary peritonitis
Familial Mediterranean fever (periodic peritonitis, familial paroxysmal polyserositis)
  • Colchicine prevents but does not treat acute attacks.
Granulomatous peritonitis
  • Diagnosed by the demonstration of diagnostic Maltese cross pattern of starch particles.
Sclerosing encapsulating peritonitis
Intraperitoneal abscesses
  • Diagnosed best by CT scan of the abdomen.
  • Treatment consists of prompt and complete CT or US guided drainage of the abscess, control of the primary cause, and adjunctive use of effective antibiotics. Open drainage is reserved for abscesses for which percutaneous drainage is inappropriate or unsuccessful.
Peritoneal mesothelioma
peritoneal carcinomatosis

Epidemiology and Demographics[edit]

  • The prevalence of peritoneal carcinomatosis is approximately 0.03 per 100,000 individuals worldwide.<ref name="pmid22287157">{{#invoke:Citation/CS1|citation

|CitationClass=journal }}</ref>

Age[edit]

  • Peritoneal carcinomatosis is more commonly observed among patients aged 50 - 70 years.<ref name="pmid8519536">{{#invoke:Citation/CS1|citation

|CitationClass=journal }}</ref>

  • Peritoneal carcinomatosis is more commonly observed among adults.<ref name="pmid21160812">{{#invoke:Citation/CS1|citation

|CitationClass=journal }}</ref>

Gender[edit]

  • Females are more commonly affected with peritoneal carcinomatosis than males.<ref name="pmid21160812">{{#invoke:Citation/CS1|citation

|CitationClass=journal }}</ref>

Race[edit]

  • There is no racial predilection for peritoneal carcinomatosis.<ref name="pmid21160812">{{#invoke:Citation/CS1|citation

|CitationClass=journal }}</ref>

Risk Factors[edit]

The most important risk factor in the development of peritoneal carcinomatosis include:

Natural History, Complications and Prognosis[edit]

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Diagnosis[edit]

Diagnostic Criteria[edit]

The diagnosis of peritoneal carcinomatosis is made when at least the following diagnostic criteria are met:<ref name="pmid8519536">{{#invoke:Citation/CS1|citation |CitationClass=journal }}</ref>

  • Imaging findings compatible with peritoneal carcinomatosis (see below)
  • Elevated protein concentration (more than 4.0 g/dL)
  • Abnormal serum-ascites albumin gradient (less than 1.1 g/dL)
  • High cell count (lymphocyte predominance)

Symptoms[edit]

Physical Examination[edit]

Inspection

  • Enlarged abdomen
  • Abdominal distension

Palpation

  • Bulging of the flanks or shifting dullness
  • Fluid thrill or fluid wave
  • Other physical examination findings may include:

Laboratory Findings[edit]

  • Laboratory findings consistent with the diagnosis of peritoneal carcinomatosis, include:<ref name="pmid21160812">{{#invoke:Citation/CS1|citation

|CitationClass=journal }}</ref>

Imaging Findings[edit]

  • Enhanced CT scan is the imaging modality of choice for peritoneal carcinomatosis.<ref name="pmid8519536">{{#invoke:Citation/CS1|citation

|CitationClass=journal }}</ref>

  • Smooth or nodular peritoneal thickening and enhancement.
  • Implants on the liver and the splenic surfaces are frequently seen and result in scalloping of the surface by the masses.
  • Sites of tumor implantation are the intersegmental fissure, superior recess of the lesser sac, subphrenic space, and Morison pouch.
  • Usually there is large ascites, which is often loculated.
  • The images below demonstrate a case of peritoneal carcinomatosis.

Other Diagnostic Studies[edit]

  • Peritoneal carcinomatosis may also be diagnosed using abdominal paracentesis.
  • Findings on paracentesis may include:

Treatment[edit]

Medical Therapy[edit]

  • The mainstay medical therapy for peritoneal carcinomatosis is hyperthermic intraperitoneal chemotherapy (HIPEC).<ref name="pmid19133112">{{#invoke:Citation/CS1|citation

|CitationClass=journal }}</ref>

  • Common chemotherapy agents, include:<ref name="pmid19133112">{{#invoke:Citation/CS1|citation

|CitationClass=journal }}</ref>

Surgery[edit]

  • Cytoreductive surgery is the mainstay of therapy for peritoneal carcinomatosis.<ref name="pmid19133112">{{#invoke:Citation/CS1|citation

|CitationClass=journal }}</ref>

  • Laparotomy in conjunction with cytology testing is the most common approach to the treatment of peritoneal carcinomatosis.
  • Cytoreductive surgery is composed of three steps: Exploration of the abdominal cavity, debulking and chemoperfusion.

Prevention[edit]

  • There are no primary preventive measures available for peritoneal carcinomatosis.
  • Once diagnosed and successfully treated, patients with peritoneal carcinomatosis are followed-up every 1, 3 or 6 months.
  • Follow-up testing includes ultrasound, paracentesis, and abdominal examination.<ref name="pmid8519536">{{#invoke:Citation/CS1|citation

|CitationClass=journal }}</ref>

References[edit]

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