Lundström Stadelmann, BrittaBrittaLundström Stadelmann0000-0003-2672-5766Rostami, AliAliRostamiFrey, Caroline F.Caroline F.Frey0000-0002-6478-7493Torgerson, Paul RPaul RTorgersonRiahi, Seyed MohammadSeyed MohammadRiahiBagheri, KimiaKimiaBagheriKaethner, MarcMarcKaethnerLachenmayer, AnjaAnjaLachenmayerBeldi, GuidoGuidoBeldi0000-0002-9914-3807Gasser, Robin B.Robin B.GasserHemphill, AndrewAndrewHemphill2025-03-252025-03-252025-07https://boris-portal.unibe.ch/handle/20.500.12422/206538Background Alveolar echinococcosis (AE) of humans is a disease caused by the larval (metacestode) stage of the 'fox tapeworm' Echinococcus multilocularis. AE primarily affects the liver and, like a cancer, can spread to surrounding or distant organs. Its slow progression and resemblance to malignant tumours emphasise the critical importance of early detection and intervention, as there is no highly effective vaccine against AE. Although recognized as a "neglected disease", there has been no recent, comprehensive review of the distribution, incidence and prevalence of AE in humans around the world. Objective To estimate the global, regional and national annual incidence rates (AIRs) and prevalences of human AE in endemic countries. Sources Incidence and prevalence data were obtained from published articles from 1980 to February 2024, accessible via public databases. Content This article provides a brief background on E. multilocularis, its biology and impact; reviews the geographic distribution and incidence of AE; and gives a perspective on the prevention and control of this neglected disease in humans. Implications The AE burden in humans is substantial and emphasises the need for enhanced public awareness, surveillance, prevention and control, particularly in under-resourced communities to mitigate the health, economic and psychological impact of AE. Britta Lundström-Stadelmann,Clin Microbiol Infect 2024; XX:XXX© 2025 The Author(s). Published by Elsevier Ltd on behalf of European Society of Clinical Microbiologyand Infectious Diseases. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).enAlveolar echinococcosisEchinococcusmultilocularisGeographic distributionHumansIncidencePrevalencePrevention600 - Technology::610 - Medicine & health600 - Technology::630 - Agriculturearticle10.48620/865234005477110.1016/j.cmi.2025.01.034