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07:20

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Pseudomyxoma peritonei usually originates from the appendix: a review of the evidence

A Mukherjee1, A Parvaiz, T D Cecil

  • 1Pseudomyxoma Research Unit, North Hampshire Hospital, Hampshire, UK.

European Journal of Gynaecological Oncology
|August 3, 2004
PubMed

Insights

Pseudomyxoma peritonei (PMP) is a rare condition, often originating from the appendix, not exclusively the ovaries. Evidence suggests appendiceal tumors are the most frequent primary source in both males and females.

Area of Science:

  • Gastroenterology and Surgical Oncology
  • Pathology and妇科肿瘤学

Background:

  • Pseudomyxoma peritonei (PMP) is a rare neoplastic condition characterized by mucinous ascites.
  • While historically linked to ovarian tumors, its origin is debated, impacting diagnosis and treatment.

Purpose of the Study:

  • To review clinical and scientific evidence regarding the primary origin of Pseudomyxoma peritonei.
  • To evaluate the role of appendiceal versus ovarian tumors in PMP development.

Main Methods:

  • Review of four large reported series encompassing 393 patients with PMP.
  • Analysis of immunohistochemistry in women with appendiceal and ovarian tumors.

Main Results:

  • Contrary to common belief, 46% of patients in reported series were male.
  • Immunohistochemistry favors appendiceal origin in most women with PMP, even when ovarian tumors are present.
  • The distinction between adenomucinosis and mucinous adenocarcinoma is critical for prognosis.

Conclusions:

  • The appendix is the most common primary site for Pseudomyxoma peritonei.
  • PMP may represent a spectrum of disease, potentially including an "adenoma-carcinoma sequence".

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