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Updated: Dec 12, 2025
Culture and Imaging of Ex Vivo Organotypic Pseudomyxoma Peritonei Tumor Slices from Resected Human Tumor Specimens
Published on: December 9, 2022
Mucinous appendiceal neoplasms with or without pseudomyxoma peritonei: a review
Mikael L Soucisse1,2, Joshua Lansom1, Mohammad S Alshahrani1,3
1Department of Surgery, University of New South Wales, St George Hospital, Sydney, Australia.
Abstract:
Mucinous appendiceal neoplasms (MANs) are rare tumours and the primary cause of pseudomyxoma peritonei. These tumours have a much more benign course than typical colorectal cancers, generally growing for many years before giving any clinical signs. The spectrum of presentations, tumour stages and the underlying cytology is very wide, warranting from the simplest operation like an appendicectomy to the most complicated operation like a complete cytoreduction surgery and hyperthermic intraperitoneal chemotherapy. Fortunately, most patients can be offered a curative treatment, but limiting operative morbidity without compromising oncologic outcomes is the biggest challenge in managing these patients. Histopathology is the cornerstone of decision making for MANs, but is also subject to ongoing debate because of a lack of terminology consensus amongst pathologists. Combined with the rarity of this disease, the multiple histopathologic classification updates of MANs explain the ongoing confusion amongst clinicians in regard to individual optimal treatment. This review will cover the most recent histological classification of MANs and attempt to clarify optimal management of patients with different clinical presentation and histologic combinations.
Insights
Mucinous appendiceal neoplasms (MANs) are rare tumors causing pseudomyxoma peritonei. This review clarifies their histology and management, aiming to optimize treatment for better patient outcomes.
Area of Science:
- Gastroenterology and Surgical Oncology
Background:
- Mucinous appendiceal neoplasms (MANs) are rare tumors, often presenting insidiously and leading to pseudomyxoma peritonei.
- MANs exhibit a wide spectrum of presentations, stages, and cytologic features, necessitating tailored surgical approaches from appendicectomy to cytoreduction with hyperthermic intraperitoneal chemotherapy.
Purpose of the Study:
- To review the most recent histological classifications of MANs.
- To clarify optimal management strategies for MANs based on clinical presentation and histological subtypes.
Main Methods:
- Literature review focusing on recent advancements in MANs classification and treatment guidelines.
- Analysis of histopathological findings and their correlation with clinical management.
Main Results:
- Histopathological classification of MANs is complex and subject to ongoing debate due to terminology inconsistencies.
- Despite challenges in classification, curative treatment is achievable for most patients, with the primary goal being to minimize operative morbidity while maintaining oncologic control.
Conclusions:
- Standardizing histopathological terminology is crucial for consistent diagnosis and treatment planning of MANs.
- A clear understanding of MANs histology and recent classifications is essential for clinicians to provide optimal, individualized patient management.
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