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Clinicopathological Features and Management of Appendiceal Mucoceles: A Systematic Review

William F Morano, Elizabeth M Gleeson, Sean H Sullivan

    The American Surgeon
    |March 28, 2018
    PubMed

    Insights

    Appendiceal mucoceles (AMs) are rare neoplasms. Initial treatment should be appendectomy, with further management guided by pathology to address the low malignancy risk.

    Area of Science:

    • Gastroenterology
    • Surgical Oncology
    • Pathology

    Background:

    • Appendiceal mucoceles (AMs) are uncommon mucin-containing neoplasms with malignant potential.
    • Limited evidence-based data exists for managing AMs based on clinicopathological features.

    Purpose of the Study:

    • To systematically review patient, pathologic, and treatment characteristics of non-perforated appendiceal mucoceles.
    • To define evidence-based guidelines for the management of AMs.

    Main Methods:

    • A MEDLINE search was conducted between 1995 and 2015 using the term "Appendix mucocele."
    • Systematic review and data analysis of patient, pathologic, and treatment variables from 276 non-perforated AM cases.

    Main Results:

    • Female patients comprised 59% of cases (163/276).
    • Pathologic findings included cystadenoma/low-grade appendiceal mucinous neoplasm (54%), unspecified/benign (25%), retention cyst (14.1%), cystadenocarcinoma (4.2%), and mucosal hyperplasia (2.9%).
    • Malignancy (cystadenocarcinoma) was diagnosed in 4.2% of cases, predominantly in females, with a higher risk of synchronous colonic malignancy (27%).

    Conclusions:

    • Appendiceal mucoceles generally have a low risk of malignancy.
    • Initial treatment should involve appendectomy, with subsequent management determined by the final pathologic diagnosis.

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