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The risk of developing pseudomyxoma peritonei from a non-perforated low grade appendiceal mucinous neoplasm found at

Chamila Lakmal1, Bipasha Chakrabarty1, Christine Tan2

  • 1Colorectal and Peritoneal Oncology Centre, The Christie NHS Foundation Trust, UK.

European Journal of Surgical Oncology : the Journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology
|August 21, 2024
PubMed
Abstract

Insights

Low-grade appendiceal mucinous neoplasms (LAMNs) classified as pTis/pT3 have a negligible risk of developing pseudomyxoma peritonei (PMP). These LAMNs require low-intensity surveillance and no further surgery if completely excised.

Area of Science:

  • Gastroenterology
  • Surgical Oncology
  • Pathology

Background:

  • Low-grade appendiceal mucinous neoplasms (LAMNs) are precursors to pseudomyxoma peritonei (PMP).
  • Classification of LAMNs includes non-perforated (pTis, pT3) and perforated (pT4) types.
  • Quantifying PMP risk in pTis and pT3 LAMNs is crucial for management.

Purpose of the Study:

  • To determine the risk of developing PMP from pTis and pT3 LAMNs.
  • To establish appropriate surveillance strategies for these low-risk appendiceal neoplasms.

Main Methods:

  • Retrospective analysis of a prospectively collected database of LAMN patients (2004-2019).
  • Exclusion of pT4 LAMNs and other appendix tumors; specialist review of pathology, operation notes, and CT scans.
  • Standardized follow-up including surveillance CTs and tumor markers (CEA, CA19-9, CA125).

Main Results:

  • 193 patients with pT3/pTis LAMNs were included (pTis=153, pT3=40).
  • Median follow-up was 6.45 years; 8 patients (4%) were lost to follow-up.
  • None of the remaining 185 patients developed PMP; 5 patients (2.5%) had R1 resections.

Conclusions:

  • pTis/pT3 LAMNs, after careful specialist review, have a negligible risk of PMP development.
  • Low-intensity surveillance is recommended for correctly classified pTis/pT3 LAMNs.
  • Complete excision obviates the need for further surgery; R1 resections require individualized assessment at specialist centers.
Keywords:
Low grade appendiceal mucinous neoplasmPseudomyxoma peritonei

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