Clinicopathologic parameters and outcomes of mucinous neoplasms confined to the appendix: a benign entity with excellent prognosis.

Appendiceal mucinous neoplasms (AMNs), characterized by expansile or "pushing" growth of neoplastic epithelium through the appendix wall, are sometimes accompanied by peritoneal involvement, the extent and grade of which largely determine clinical presentation and long-term outcomes. However, the prognosis of tumors entirely confined to the appendix is still debated and confusion remains regarding their biologic behavior and, consequently, their clinical management and even diagnostic nomenclature. We evaluated AMNs limited to the appendix from 337 patients (median age: 58 years, interquartile range (IQR): 47-67), 194 (57.6%) of whom were women and 143 (42.4%) men. The most common clinical indication for surgery was mass or mucocele, in 163 (48.4%) cases. Most cases (N = 322, 95.5%) comprised low-grade epithelium, but there were also 15 (4.5%) cases with high-grade dysplasia. Lymph nodes had been harvested in 102 (30.3%) cases with a median 6.5 lymph nodes (IQR: 2-14) per specimen for a total of 910 lymph nodes examined, all of which were negative for metastatic disease. Histologic slide review in 279 cases revealed 77 (27.6%) tumors extending to the mucosa, 101 (36.2%) to submucosa, 33 (11.8%) to muscularis propria, and 68 (24.4%) to subserosal tissues. In multivariate analysis, deeper tumor extension was associated with older age (p = 0.032; odds ratio (OR): 1.02, 95% confidence intervals (CI): 1.00-1.03), indication of mass/mucocele (p < 0.001; OR: 2.09, CI: 1.41-3.11), and wider appendiceal diameter, grossly (p < 0.001; OR: 1.61, CI: 1.28-2.02). Importantly, among 194 cases with at least 6 months of follow-up (median: 56.1 months, IQR: 24.4-98.5), including 9 high-grade, there was no disease recurrence/progression, peritoneal involvement (pseudomyxoma peritonei), or disease-specific mortality. These data reinforce the conclusion that AMNs confined to the appendix are characterized by benign biologic behavior and excellent clinical prognosis and accordingly suggest that revisions to their nomenclature and staging would be appropriate, including reverting to the diagnostic term mucinous adenoma in order to accurately describe a subset of them.
(© 2022. The Author(s), under exclusive licence to United States & Canadian Academy of Pathology.)