Combined Mucinous and Neuroendocrine Tumours of the Appendix Managed with Surgical Cytoreduction and Oxaliplatin-based Hyperthermic Intraperitoneal Chemotherapy

Abstract

Appendiceal neoplasms account for 1% of appendectomy specimens. Common subtypes include mucinous cystadenoma, adenocarcinoma, and neuroendocrine tumors (NETs). The simultaneous presence of appendicular mucinous and NETs is a rare event. Depending on the tumors' morphological distribution in the affected organ, they are qualified as either "collision" or "combined" tumours. We herein present the case of a 50-year-old male who presented with acute appendicitis and who was subsequently found to have pseudomyxoma peritonei (PMP) due to a perforated combined mucinous and neuroendocrine tumours. The patient was treated by right hemicolectomy and cytoreductive surgery (CRS) with oxaliplatin-based hyperthermic intraperitoneal chemotherapy (HIPEC). He was cancer free 20 months later. Due to the limited clinical experience with this presentation, no formal recommendations exist as to its management other than those applicable to each cancer alone. The efficacity of treatment on the long-term prognosis on these combined tumors is yet to be elucidated.