Clinicopathological Spectrum and Long-Term Outcomes of Appendiceal Neoplasms: A Bicentric Cohort Study

Vollständiger Abstract

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Background: Appendiceal neoplasms are rare and biologically heterogeneous, with diverse clinical presentations, pathological features, and treatment requirements. Their rarity has limited detailed real-world outcome data, particularly from smaller health-care systems. Methods: We conducted a retrospective bicentric cohort study of patients with histologically confirmed appendiceal neoplasms treated at two tertiary surgical centres in Skopje, North Macedonia, between January 2016 and May 2026. Patients with appendiceal disease were identified through predefined ICD-10 screening, and clinical presentation, histopathology, stage, treatment, and long-term outcomes were evaluated. Overall survival was estimated using the Kaplan–Meier method. Results: Among 3,072 patients with appendiceal disease screened during the study period, 18 histologically confirmed appendiceal neoplasms were identified, corresponding to a detection rate of 0.59%. Mean age was 56.6 ± 12.7 years, and 10 (55.6%) patients were male; 12 (66.7%) presented acutely or emergently. Mucinous adenocarcinoma (38.9%) and low-grade appendiceal mucinous neoplasm (33.3%) together accounted for 72.2% of cases. Stage II was the most frequently recorded stage (10/18, 55.6%), while three patients (16.7%) had stage IV disease and pseudomyxoma peritonei was documented in two (11.1%). Management ranged from appendectomy alone to right hemicolectomy, multivisceral resection, and cytoreductive surgery; five patients (27.8%) received systemic chemotherapy. At a median follow-up of 39.3 months (IQR, 24.8–78.1), three patients had died, with no deaths attributed to appendiceal neoplasia. Estimated overall survival was 100.0% at 1 year, 93.8% at 3 years, and 84.4% at 5 years; median overall survival was not reached. Conclusions: Appendiceal neoplasms were identified in fewer than 1% of patients with appendiceal disease but demonstrated marked heterogeneity in presentation, histology, stage, and management. Mucinous neoplasms predominated, and acute surgical presentation was common. These findings support histology- and disease-extent–directed management and highlight the need for larger multicentre studies with standardised pathology and disease-specific follow-up.