- Rare borderline malignant condition
- Approximately 100 cases per year in UK
- More common in women than men
- Characterised by production of large volumes of mucinous ascites
- Often presents with advanced disease
- It is often associated with ovarian or appendicular pathology
- Depending on degree of cytological atypia the pathology has been
classified as:
- Disseminated peritoneal adenomucinosis
- Peritoneal mucinous carcinomatosis
Clinical features
- Features of raised intrabdominal pressure
- Bloating
- Abdominal wall hernia
- Uterovaginal prolapse
- Feature mimicking appendicitis
- Palpable abdominal mass
- Non-specific abdominal pain
- Features of advanced malignancy
- Anorexia
- Weight loss
- Ascites
Investigations
- CT is the first line investigation
- Omental cake often apparent
- Scalloping if the diaphragmatic surface of the liver is
characteristic
- Segmental narrowing of the small bowel is a poor prognostic sign
- Patients are often anaemic
- Serum inflammatory markers are raised
- Tumours markers (CEA, CA19.9 and CA125) are often elevated
Management
- Management is controversial
- Surgery consists of either
- Complete cytoreduction with curative intent
- Palliative debulking
- Complete cytoreduction is usually combined with intraperitoneal
chemotherapy
- Careful patient selection is required
- Complete cytoreduction is major undertaking
- Postoperative mortality is about 5%
- Intrabdominal sepsis occurs in about 30% patients
- Surgery is indicated if:
- Complete removal of tumour is achievable
- Palliative debulking will improve quality of life
Debulking
- Involves removal of mucin and tumour bulk
- Limited resectional procedures may be performed
Cytoreduction
- The aim is to remove all macroscopic disease
- No tumour deposits more than 3 mm should be left
- This will maximise effect of chemotherapy
- Six peritonectomy procedures may be necessary:
- Greater omentectomy and splenectomy
- Stripping of left hemidiaphragm
- Stripping of right hemidiaphragm
- Cholecystectomy and lesser omentectomy
- Distal gastrectomy
- Pelvic peritonectomy and anterior resection
Intraperitoneal chemotherapy
- Systemic chemotherapy if of limited value
- Intraperitoneal chemotherapy should be give after adequate
cytoreduction
- Of limited benefit if significant residual disease
- Intraoperative Mitomycin C is followed by postoperative 5FU
- Chemotherapeutic agents are heated to 41 degrees
- Heat seems to have a synergistic effect to the drugs
- Increases risk of fistula formation and anastomotic leak
Bibliography
Bryant J, Clegg A J, Sindhu M K et al. Systematic
review of the Sugarbaker procedure for pseudomyxoma peritonei. Br
J Surg 2005; 92: 153-158.
Moran B J, Cecil T D. The etiology, clinical presentation
and management of pseudomyxoma peritonei. Surg Clin North Am
2003; 12: 585-603.
Murphy E M, Farquharson S M, Moran B J. Management of
unexpected appendiceal neoplasm. Br J Surg 2006; 93:
783-792.
Sugarbaker P H. New standard of care for appendiceal epithelial
neoplasm and pseudomyxoma peritonei syndrome. Lancet Oncol
2006; 7: 69-76. |