Mucinous epithelial neoplasms of the appendix and pseudomyxoma peritonei

Joseph Misdraji1

  • 1Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA.

Insights

The classification of appendiceal mucinous neoplasms, including pseudomyxoma peritonei (PMP), remains debated. Recent WHO classifications acknowledge low-grade appendiceal mucinous neoplasms, but consensus is hindered by ongoing discussions on terminology and PMP grading.

Area of Science:

  • Gastroenterology
  • Pathology
  • Oncology

Background:

  • Appendiceal mucinous neoplasms, particularly low-grade variants, present diagnostic challenges due to their potential to cause pseudomyxoma peritonei (PMP).
  • Existing classifications, including the recent WHO update, have attempted to standardize terminology for these tumors, recognizing their distinct morphology and behavior.
  • However, controversies persist regarding the precise classification and terminology, impacting clinical consensus and patient management.

Purpose of the Study:

  • To review the current controversies in the classification of appendiceal mucinous neoplasms and pseudomyxoma peritonei (PMP).
  • To highlight the significance of histologic grade in determining patient prognosis for PMP.
  • To discuss the implications of ongoing debates on achieving a consensus classification.

Main Methods:

  • Review of recent literature and WHO classifications pertaining to appendiceal mucinous neoplasms and PMP.
  • Analysis of the debate surrounding terminology, grading, and biologic behavior of these tumors.
  • Emphasis on the prognostic value of histologic grade in peritoneal tumors.

Main Results:

  • The classification of low-grade appendiceal mucinous neoplasms is acknowledged by the WHO but remains debated.
  • Pseudomyxoma peritonei (PMP) classification is contentious, with differing views on using malignant terminology for all grades.
  • Histologic grade of peritoneal tumors is increasingly recognized as crucial for prognostication in PMP patients.

Conclusions:

  • Achieving a consensus classification for appendiceal mucinous neoplasms and PMP is impeded by ongoing terminologic and grading debates.
  • The biologic behavior and prognostic implications of low-grade appendiceal mucinous neoplasms and PMP require further clarification and standardization.
  • Histologic grading of peritoneal disease is essential for accurate prognosis in patients with pseudomyxoma peritonei.

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