We describe the different clinical presentations, radiology, histology and management of this unique, highly aggressive disease. Clinical presentation of appendicitis may not just be appendicitis. Appendiceal tumors must always be considered in the differential diagnosis. Dedicated radiology and histological examination in addition to aggressive surgical and oncological input improve outcome.
Keywords: adenocarcinoma ex‐goblet cell carcinoid; appendiceal neoplasm; appendiceal tumors; appendix.