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Updated: Apr 19, 2026

Culture and Imaging of Ex Vivo Organotypic Pseudomyxoma Peritonei Tumor Slices from Resected Human Tumor Specimens
Published on: December 9, 2022
Mucinous epithelial neoplasms of the appendix and pseudomyxoma peritonei
1Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA.
Abstract:
The classification of appendiceal mucinous neoplasms has been controversial, largely focused on a particular subset of low-grade mucinous tumors that, despite their innocuous appearance, can disseminate to the peritoneal cavity as pseudomyxoma peritonei (PMP). Recent WHO classification of these tumors as low-grade appendiceal mucinous neoplasms acknowledges their unique morphologic appearance and biologic behavior. Still, debate about the use of this term and its parameters continues to impede the adoption of consensus classification for appendiceal mucinous neoplasms. The classification of PMP has also been the subject of debate, with international authorities advocating for the use of malignant terminology to describe all grades of PMP, even though some authorities consider low-grade PMP to be dissemination of adenomatous epithelium in the peritoneum. Recent data also emphasize the importance of histologic grade of the peritoneal tumors in defining prognosis of these patients.
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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