An appendix tumour is found every 100 appendectomies. It can also be incidental discovery during a routine radiological examination and presents as a mucocele of the appendix. Appendix tumours are of varied histological type, and include mainly neuroendocrine tumours as well as epithelial tumours. The latter, when they secrete mucin, can give rise to peritoneal pseudomyxoma, a particular form of carcinosis harbouring serious complications. The management of appendix tumours responds to specific recommendations and, in advanced cases, requires the expertise of a university centre specialized in digestive oncology. The role of the primary care practitioner lies in the diagnosis and referral of patients with appendix tumours.
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