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Curr Oncol. 2014 Feb;21(1):e155-65. doi: 10.3747/co.21.1695.

Pseudomyxoma peritonei originating from urachus-case report and review of the literature.

Author information

  • 1Second Department of General and Oncological Surgery, Wroclaw Medical University, Wroclaw, Poland.
  • 2Department of General Surgery ii , Lower-Silesian Specialised Hospital, Wroclaw, Poland.
  • 3Wroclaw Medical University, Wroclaw, Poland.
  • 4Division of Pathomorphology and Oncological Cytology, Wroclaw Medical University, Wroclaw, Poland.

Abstract

Pseudomyxoma peritonei (pmp) is a rare clinical condition defined as extensive intraperitoneal spread of mucus associated with a variety of mucinous tumours of varying biologic behavior. Although appendix or ovaries have usually been implicated as the primary site, cases have been reported in association with neoplastic lesions of other sites. Pseudomyxoma peritonei originating from urachal remnants is a unique entity, reported only 18 times in the English literature thus far. Considering the rarity of the lesion, we report the case of a 50-year-old man surgically treated for pmp associated with a low-grade mucinous urachal neoplasm. Unique aspects of case are the low histologic aggressiveness of the causative lesion (reported only twice worldwide) and the early stage of the disease, with a relatively small amount of intraperitoneal free mucin. Review of the literature about pmp in general and a collation of previously reported cases of pmp originating from the urachus are presented and discussed.

KEYWORDS:

Pseudomyxoma peritonei; mucinous neoplasms; urachal adenocarcinoma; urachus

PMID:
24523614
PMCID:
PMC3921041
DOI:
10.3747/co.21.1695
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