Revision 36 for 'Pseudomyxoma peritonei'

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Pseudomyxoma peritonei

Pseudomyxoma peritonei refers to the intraperitoneal accumulation of a gelatinous ascites secondary to rupture of a mucinous tumor. The most common cause is a ruptured mucinous tumor of the appendix/appendiceal mucocoele 10.  

Occasionally, mucinous tumors of the colon, rectum, stomach, pancreas and urachal tumors 9,10,13 are implicated. There is some ongoing contention as to whether primary ovarian tumors are a frequent source in their own right, or whether in these cases the appendix is the primary site and the ovarian lesion is metastatic 2,3.

Pathology

The remarkable feature of pseudomyxoma peritonei is that this neoplastic, progressive process often arises from a seemingly benign or well-differentiated primary tumor 3. With the rupture of these mucinous tumors, the mucus and cells will flow along with the peritoneal fluid and spread with deposits commonly seen at the pelvis, paracolic gutters, on the liver capsule, and on the omentum 13

Pseudomyxoma peritonei may be divided into two pathological subtypes which have etiological and prognostic significance 4:

  • peritoneal adenomucinosis
    • a peritoneal neoplasm composed largely of mucin associated with fibrosis with minimal cytological atypia and mitoses
    • the primary tumor is generally an adenoma
  • peritoneal mucinous carcinoma
    • characterized by proliferative epithelium, glands, nests, or individual cells with marked cytologic atypia
    • the primary is a mucinous adenocarcinoma

Not all cases fit neatly into these categories, and many patients have intermediate or discordant features.  

However, a long term follow-up study 4 of 109 patients found 5-year survival rates were markedly different: 75% for the adenomucinosis group and 14% for the mucinous carcinoma group. The intermediate/discordant group 5 year survival was 50%.

Radiographic features

It is typically characterized by loculated collections of fluid causing scalloping of abdominopelvic organs.

Plain radiograph

May show evidence of ascites with centrally displaced small bowel loops and scattered punctate or curvilinear calcifications.

Ultrasound 
  • echogenic peritoneal masses or ascites with echogenic particles which (unlike other forms of particulate ascites such as hemoperitoneum or pus in the peritoneum) do not move 6
  • small bowel loops displaced medially
  • may show scalloping of the liver, spleen and at times other organs
CT
  • low attenuation, often loculated fluid throughout the peritoneum, omentum and mesentery
  • scalloping of visceral surfaces, particularly the liver 5
  • scattered (curvilinear or punctate) calcifications may be frequently present 5
  • tends to remain localized to the peritoneal cavity
MRI

Reported signal characteristics of the collections include 8

  • T1: typically low signal
  • T2: typically high signal
  • T1 C+ (Gd): may show enhancement 12

Treatment and prognosis

This is a progressive and often fatal disease. Recurrent bowel obstructions are commonly due to the fibrosis and adhesions in advanced disease 13.

Treatment is surgical debulking, followed by infusion of intraperitoneal chemotherapy. Aggressive surgical treatment has been considered based on the calculation of the peritoneal cancer index (PCI), which is a prognostic indicator based on the amount of tumor found at laparoscopy. This index has been performed using CT or MRI 13

Differential diagnosis

General imaging differential considerations include:

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