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.
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 aetiological and prognostic significance 4:
- 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%.
It is typically characterized by loculated collections of fluid causing scalloping of abdominopelvic organs.
May show evidence of ascites with centrally displaced small bowel loops and scattered punctate or curvilinear calcifications.
- 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
- 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 localised to the peritoneal cavity
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.
General imaging differential considerations include:
- 1. Brant WE, Helms CA. Fundamentals of diagnostic radiology. Lippincott Williams & Wilkins. (2007) ISBN:0781761352. Read it at Google Books - Find it at Amazon
- 2. Federle MP, Jeffrey RB, Woodward PJ et-al. Diagnostic Imaging: Abdomen, Published by Amirsys®. Lippincott Williams & Wilkins. (2009) ISBN:1931884714. Read it at Google Books - Find it at Amazon
- 3. Misdraji J. Appendiceal mucinous neoplasms: controversial issues. Arch. Pathol. Lab. Med. 2010;134 (6): 864-70. Arch. Pathol. Lab. Med. (link) - Pubmed citation
- 4. Ronnett BM, Yan H, Kurman RJ et-al. Patients with pseudomyxoma peritonei associated with disseminated peritoneal adenomucinosis have a significantly more favorable prognosis than patients with peritoneal mucinous carcinomatosis. Cancer. 2001;92 (1): 85-91. Cancer (link) - Pubmed citation
- 5. Yoo E, Kim JH, Kim MJ et-al. Greater and lesser omenta: normal anatomy and pathologic processes. Radiographics. 27 (3): 707-20. doi:10.1148/rg.273065085 - Pubmed citation
- 6. Hanbidge AE, Lynch D, Wilson SR. US of the peritoneum. Radiographics. 23 (3): 663-84. doi:10.1148/rg.233025712 - Pubmed citation
- 7. Ros PR, Mortele KJ. CT and MRI of the abdomen and pelvis, a teaching file. Lippincott Williams & Wilkins. (2006) ISBN:0781772370. Read it at Google Books - Find it at Amazon
- 8. Buy JN, Malbec L, Ghossain MA et-al. Magnetic resonance imaging of pseudomyxoma peritonei. Eur J Radiol. 1989;9 (2): 115-8. - Pubmed citation
- 9. Sugiyama K, Ito N. Mucinous cystadenocarcinoma of the urachus associated with pseudomyxoma peritonei with emphasis on MR findings. Magn Reson Med Sci. 2009;8 (2): 85-9. Magn Reson Med Sci (link) - Pubmed citation
- 10. Takeuchi M, Matsuzaki K, Yoshida S et-al. Imaging findings of urachal mucinous cystadenocarcinoma associated with pseudomyxoma peritonei. Acta Radiol. 2004;45 (3): 348-50. Acta Radiol (link) - Pubmed citation
- 11. Semelka RC. Abdominal-Pelvic MRI. Wiley-Blackwell. (2010) ISBN:0470487755. Read it at Google Books - Find it at Amazon
- 12. Eurorad teaching files : Case 1239
- 13. Leonards LM, Pahwa A, Patel MK, Petersen J, Nguyen MJ, Jude CM. Neoplasms of the Appendix: Pictorial Review with Clinical and Pathologic Correlation. Radiographics : a review publication of the Radiological Society of North America, Inc. 37 (4): 1059-1083. doi:10.1148/rg.2017160150 - Pubmed