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Appendiceal adenocarcinoma: Current concepts & challenges
1Department of Pathology, Cleveland Clinic, Cleveland Clinic 9500 Euclid Ave, Cleveland, OH 44195, United States.
Abstract:
Appendiceal adenocarcinoma (ApAC) is a rare malignancy, comprising less than 1 % of all gastrointestinal tumors. The current World Health Organization classifies ApAC as mucinous or nonmucinous. Mucinous ApAC are composed of pools of mucin lined by cells with low- and high-grade cytology and areas of infiltrative invasion. Nonmucinous ApAC histologically resemble conventional colorectal adenocarcinomas and have a worse prognosis than their mucinous counterpart. Unfortunately, the nomenclature and histologic classification of ApAC, specifically the mucinous subtype, has changed several times throughout the years, contributing to diagnostic confusion for pathologists. The treatment for mucinous ApAC differs from that of other appendiceal mucinous neoplasms, thus accurate diagnosis is key to patient management and outcome. This review discusses the current classification and staging of ApAC with a particular emphasis on the mucinous subtype and peritoneal disease, as these areas are the most challenging for practicing surgical pathologists.
Insights
Appendiceal adenocarcinoma (ApAC) diagnosis is challenging due to evolving classifications, especially for mucinous subtypes. Accurate staging and classification are crucial for effective patient management and improved outcomes in this rare cancer.
Area of Science:
- Gastrointestinal Pathology
- Surgical Oncology
Background:
- Appendiceal adenocarcinoma (ApAC) is a rare gastrointestinal malignancy, accounting for less than 1% of all such tumors.
- Current World Health Organization classification divides ApAC into mucinous and nonmucinous subtypes, with distinct histological features and prognoses.
Purpose of the Study:
- To review the current classification and staging of appendiceal adenocarcinoma.
- To emphasize the diagnostic challenges associated with the mucinous subtype and peritoneal disease.
Main Methods:
- Literature review of appendiceal adenocarcinoma classification and staging.
- Focus on histological features of mucinous and nonmucinous ApAC.
- Discussion of diagnostic challenges and their impact on patient management.
Main Results:
- Nomenclature and classification of ApAC, particularly the mucinous subtype, have undergone significant changes, leading to diagnostic confusion.
- Mucinous ApAC exhibits pools of mucin with varying cytology and infiltrative invasion.
- Nonmucinous ApAC resembles colorectal adenocarcinoma and has a poorer prognosis.
Conclusions:
- Accurate diagnosis of ApAC, especially the mucinous subtype, is critical due to differing treatment protocols.
- Understanding current classification and staging is essential for optimal patient management and outcomes.
- Peritoneal disease in ApAC presents significant challenges for surgical pathologists.
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