Pseudomyxoma Peritonei Arising from Urachal Mucinous Neoplasms: a Case Series and Updated Literature Review

Niccolo Allievi1, Vasanth Mark Samuel1, Norman Carr1

  • 1Peritoneal Malignancy Insitute, Hampshire Hospitals NHS Foundation Trust, Basingstoke, UK.

Insights

Pseudomyxoma peritonei (PMP) from urachal mucinous neoplasm (UMN) is rare but treatable. Complete cytoreduction surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC) offers good prognosis for PMP patients.

Area of Science:

  • Oncology
  • Surgical Oncology
  • Gastroenterology

Background:

  • Pseudomyxoma peritonei (PMP) is a rare malignancy.
  • Urachal mucinous neoplasm (UMN) is an uncommon cause of PMP.
  • Treatment outcomes for PMP from UMN are not well-established.

Purpose of the Study:

  • To report a case series of PMP from UMN treated with CRS and HIPEC.
  • To review the current literature on PMP from UMN.
  • To evaluate the efficacy and prognosis of CRS and HIPEC for UMN-derived PMP.

Main Methods:

  • Retrospective review of patients treated between 2000-2021.
  • Literature search using MEDLINE and Google Scholar.
  • Analysis of clinical presentation, imaging, tumor markers, surgical outcomes, and survival data.

Main Results:

  • Six cases of PMP from UMN were identified.
  • Common symptoms included abdominal distension, weight loss, fatigue, and hematuria.
  • Five patients achieved complete cytoreduction with CRS and HIPEC, with survival ranging from 43-141 months.
  • Histological findings were similar to PMP from appendiceal mucinous neoplasms.

Conclusions:

  • PMP from UMN is a rare but treatable condition.
  • Complete cytoreduction via CRS and HIPEC is associated with a favorable prognosis.
  • Further research is needed for a definitive classification system.

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