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Peritoneal malignancies

Peritoneal cancers may be primary (originate in the peritoneum) or secondary (cancer spreads from another organ).

Primary peritoneal cancers include peritoneal mesotheliomas, primary peritoneal carcinoma and desmoplastic small round cell tumour. These cancers are very rare and they often have a poor prognosis.

Secondary peritoneal cancers are more common and may originate from the ovaries, appendix, colon and rectum, stomach, small bowel, pancreas, lung and breast.

Symptoms of peritoneal malignancies are often vague like abdominal pain, distension and stomach upset and thus are often diagnosed late.

Treatment options are conservative (watching), chemotherapy, surgery or a combination of both. The most aggressive and relatively new treatment of cytoreductive surgery (CRS) and heated intraperitoneal chemotherapy (HIPEC) is considered to give the best long term outcomes for suitable patients but does have a high morbidity associated with it.

Princess Alexandra Hospital offers surgery to some patients with either primary peritoneal malignancies or secondary cancers arising from the appendix, colon, rectum or small bowel.

Patients need to be seen by the specialists (surgeons and medical oncologists) at Princess Alexandra Hospital to see if they are suitable for this procedure. It is important to remember that in secondary peritoneal cancers this usually indicates aggressive disease and all steps are taken to exclude cancer spreading elsewhere in the body that this highly morbid procedure will not influence.

Pseudomyxoma peritonei (PMP)

Pseudomyxoma peritonei is a secondary peritoneal malignancy that is rare with an incidence of one to two per million per year [1] usually originating from the appendix or ovaries. It is a borderline malignancy [2] characterised by mucin-producing tumour cells that develops when an adenoma grows within the appendix. The appendix ruptures, leaking mucinous epithelial cells into the abdominal cavity resulting in a “jelly belly" [3]. It develops very slowly and the cause of PMP is unknown [4]. It generally remains within the peritoneum or abdominal cavity and rarely spreads via the blood or lymphatic systems. The fact it is usually confined to the abdominal cavity usually make it suitable for CRS and HIPEC.

Symptoms

Symptoms of pseudomyxoma peritonei may include:

  • Usually nonspecific and thus PMP is difficult to diagnose
  • Gradually increasing abdominal girth [5] or pelvic pain
  • Development of a hernia in the groin or umbilicus
  • Changes in bowel habits.

Diagnosis

The diagnosis may be difficult and it is often an unexpected finding during investigations for non-specific abdominal symptoms or found during surgery.

Treatment

PMP is typically treated in one of three ways:

  1. Watch and wait
    • This is used if there is only small volume of disease or for patients who are not fit for major surgery.
  2. Debulking surgery
    • This was the traditional surgical approach but the tumour is only partially removed. This is not curative but can provide significant relief of distressing symptoms such as abdominal distension and anorexia. This can be performed more than once but becomes increasingly difficult.
  3. Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) with care provided by surgeons and medical oncologists
    • CRS refers to attempts to completely remove or destroy all visible tumour present within the abdominal cavity. This will often require multiple organ resections and the stripping of the parietal peritoneum.
    • HIPEC delivers highly concentrated chemotherapy at 41-42 degrees.
    • It is an extensive procedure that can take over 12 hours.

Further information

Guidelines:     

Websites:

References

  1. Hinson FL, and Ambrose NS. Pseudomyxoma peritonei. British Journal of Surgery, 1998; 85 (10): p. 1332-9.
  2. Nagarajan P, et al. Sugarbaker procedure for pseudomyxoma peritonei (Protocol). The Cochrane Library, 2008; (1).
  3. Zhi-Bo Q, and Lian-Xin L. Management of pseudomyxoma peritonei. World Journal of Gastroenterol, 2006; 12 (38): p.6124-6127.
  4. Trust, T.C.N.F. Pseudomyxoma Peritonei, 2013.
  5. Sugarbaker P. Pseudomyxoma Peritonei Syndrome Defined, 2012.
Last updated 2 September 2015
Last reviewed 2 September 2015