IMR Press / EJGO / Volume 25 / Issue 4 / pii/2004195

European Journal of Gynaecological Oncology (EJGO) is published by IMR Press from Volume 40 Issue 1 (2019). Previous articles were published by another publisher on a subscription basis, and they are hosted by IMR Press on imrpress.com as a courtesy and upon agreement with S.O.G.

Open Access Review

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

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1 Pseudomyxoma Research Unit, North Hampshire Hospital, Hampshire (U.K.)
Eur. J. Gynaecol. Oncol. 2004, 25(4), 411–414;
Published: 10 August 2004
Abstract

Pseudomyxoma peritonei (PMP) is a rare condition, said to be more common in females during the fourth or fifth decade of life with an incidence believed to be in the region of one per million per year. Although PMP has been reported as origi­nating from many intra-abdominal organs, in the majority of cases an ovarian or appendix cystadenoma or cystadenocarci­noma has been implicated as the primary site. Our experience suggests that most cases arise from the appendix. We have reviewed the clinical and scientific evidence. In the four largest reported series of 393 patients, 181 (46%) were males. Immunohistochemistry techniques in women with both appendical and ovarian tumours favour an appendiceal primary in most cases. The distinction between “benign” adenomucinosis and mucinous adenocarcinoma is important in both treatment and prognosis. Experience suggests that there may well be a spectrum of disease and possibly an “adenoma carcinoma sequence”.

Keywords
Pseudomyxoma peritonei
Origin
Gender distribution
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