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Related Concept Videos

Appendicitis-I: Introduction01:22

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The appendix, a small, narrow, blind tube extending from the inferior part of the cecum, is widely regarded as a vestigial organ, having lost much of its original function through evolution. Despite its diminished role, the appendix can become inflamed, a condition known as appendicitis.
Etiology: Appendicitis can arise from various causes, primarily rooted in the obstruction of the appendix lumen. Factors contributing to this obstruction include fecal accumulation, lymphoid hyperplasia and, in...
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Pseudomyxoma Peritonei of Extra-Appendiceal Origin: A Comparative Study.

Dario Baratti1, Shigeki Kusamura1, Massimo Milione2

  • 1Peritoneal Malicnancy Program, Fondazione IRCCS Istituto Nazionale Tumori, Milan, Italy.

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|June 29, 2016
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Summary

Pseudomyxoma peritonei (PMP) originating from extra-appendiceal sites shows similar outcomes to appendiceal PMP after cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS/HIPEC). Extra-appendiceal origin does not negatively impact patient prognosis.

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Area of Science:

  • Oncology
  • Surgical Oncology
  • Gastroenterology

Background:

  • Pseudomyxoma peritonei (PMP) typically arises from appendiceal neoplasms, but extra-appendiceal origins are less common and their clinical implications are not well understood.
  • Understanding the characteristics and outcomes of PMP from varied origins is crucial for effective treatment strategies.

Purpose of the Study:

  • To investigate the clinical, pathological, and survival differences between appendiceal and extra-appendiceal PMP.
  • To evaluate the efficacy of cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) in patients with PMP of different origins.

Main Methods:

  • A prospective database of 225 PMP patients treated with CRS and HIPEC was analyzed.
  • Patients were classified based on appendiceal versus extra-appendiceal primary origin, with histological confirmation.
  • Clinical, pathological, and immunohistochemical features were correlated with origin and survival outcomes.

Main Results:

  • 19 patients (8.4%) had extra-appendiceal PMP, originating from the ovary, uterine cervix, or mature cystic teratomas.
  • Appendiceal and extra-appendiceal PMP groups showed comparable overall survival rates (10-year survival: 63.4% vs. 62.0%, respectively).
  • High-grade histology, prior chemotherapy, extensive resections, and incomplete cytoreduction were independent predictors of poorer survival.

Conclusions:

  • PMP appears to be a relatively homogeneous disease regardless of primary site, with similar clinical-pathological features and outcomes after CRS/HIPEC.
  • Extra-appendiceal origin of PMP does not contraindicate CRS/HIPEC and does not adversely affect survival.
  • The study highlights the importance of complete cytoreduction and managing high-grade disease for improved patient outcomes.