Background: Appendiceal mucinous tumour (AMT) is a very uncommon disease. We aimed to evaluate preoperative and postoperative characteristics as well as long-term outcomes of patients with appendiceal mucinous adenomas and peritoneal pseudomyxoma (PMP).
Methods: A consecutive cohort of patients diagnosed with AMT were included in the study. Tumour recurrence and the development of PMP was diagnosed based on the patients' complaints, physical examination, imaging studies, and biopsy results. The calculation of survival rates was performed using the Kaplan-Meier method.
Results: Between January 2003 and February 2013, 25 patients had AMT. 15 patients underwent appendectomy only (60%), 5 patients (20%) right hemicolectomy, 4 patients (16%) cytoreductive surgery (CRS) ± hyperthermic intraperitoneal chemotherapy (HIPEC), and 1 patient (4%) appendectomy with caecal resection. Another 2 patients underwent CRS and HIPEC 4 days and 3 years, respectively, following initial appendectomy. In the histopathologic examination, adenoma was diagnosed in 12 patients (48%); among these, low-grade PMP was found in 1 patient (4%). Low-grade appendiceal mucinous adenocarcinoma (AMCa) was diagnosed in 11 patients (44%); among these, low-grade PMP was found in seven cases (28%) and high-grade AMCa was diagnosed in 2 patients (8%). 2 of 8 PMP patients did not undergo CRS ± HIPEC because of comorbidities. The 5-year overall survival was 100% for the adenomas and 62% for PMP patients.
Conclusion: AMT is a very rare entity with a considerably good outcome even in disseminated disease. CRS and HIPEC is a gold standard treatment for PMP with excellent prognosis. However, our results could still be improved in the future.
Keywords: Appendectomy; Cytoreductive surgery; Hyperthermic intraperitoneal chemotherapy, HIPEC; Mucinous appendiceal neoplasm; Mucocele; Pseudomyxoma peritonei.