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A 45-year-old male presented with jaundice, abdominal discomfort, and weight loss. Abdominal ultrasound revealed intrahepatic cholestasis and cholelithiasis indicative of Caroli-syndrome. Subsequent magnetic resonance imaging demonstrated a corresponding 5 × 4 cm polycyclic, calcified mass and a distant 12 mm subhilar stenosis of the common bile duct resembling cholangiocarcinoma. Calcifications seemed unusual and brought up differential diagnosis of echinococcosis. Serology revealed an echinococcus antibody-titer of 33 U (cut-off > 11.5 U). However, the clinical presentation with jaundice, weight loss, and a distant subhilar stenosis of the common bile duct without association to the intrahepatic process led to underestimation of the positive antibody-titer. Thus, the patient underwent right hemihepatectomy for potential cholangiocarcinoma. Dense infiltrative small cysts interspersed the resected right lobe of the liver. Histopathology revealed a granulomatous inflammation surrounding typical cuticulas, confirming hepatobiliary alveolar echinococcosis. Alveolar echinococcosis is considered as one of the neglected diseases by the World Health Organization, although incidence is rising worldwide and specifically in Germany. Our case illustrates hurdles in diagnosing alveolar echinococcosis and underlines the importance to be aware of classical and rare clinical manifestations., Competing Interests: The authors declare that they have no conflict of interest., (Thieme. All rights reserved.)

A 19-year-old woman presented with liver capsule pain and a liver lesion on sonography, which contained microvesicular cystic, necrotic and solid fibrotic formations typical for alveolar echinococcosis (AE). The diagnosis was confirmed by serology and histopathology. This parasitic infection which is endemic in Germany is feared because of its malignant growth. The increasing expansion of E. multilocularis in Europe will lead to a higher incidence of AE with an occurrence of cases outside classical endemic regions.

Purpose of Review: Human alveolar echinococcosis is caused by the larval stage of Echinococcus multilocularis, occurring in at least 42 countries of the northern hemisphere. Recent studies in Europe and Asia have shown that the endemic area of E. multilocularis is larger than previously known and the parasite has regionally expanded from rural to urban areas. Diagnosis of alveolar echinococcosis is supported by results from imaging studies, histopathology and/or nucleic acid detection, and serology. The present review summarizes current understanding of clinical features, knowledge on appropriate treatment, and discusses ways to improve standards of care., Recent Findings: High prevalences of this deadly disease have been discovered in surveys in parts of China. Clinical manifestations, diagnostic tools and the burden of disease were described, and are based on high case numbers. In Europe, excellent tools have been introduced, which improve disease management. Long-term observations in Switzerland provide an optimistic view, as the infection can be well controlled, if patients are cared for in specialized centres. An expert consensus summarizes the current recommendation for diagnosis and treatment of alveolar echinococcosis by the Informal Working Group on Echinococcosis of the WHO., Summary: Diagnosis and treatment of alveolar echinococcosis remains a challenge for clinicians. The updated WHO-recommendations aim to support decisions on diagnosis and treatment of alveolar echinococcosis. Anti-infective therapy is the backbone of treatment; surgery should be restricted to patients at an early stage of the disease. For the majority of cases continuous chemoprophylaxis with benzimidazoles is cost-effective and leads to a good quality of life for patients with this chronic disease.

Echinococcus granulosus, an intestinal tapeworm of dogs and other canids, infects humans in its larval stage and causes human echinococcosis or hydatid disease. In the Republic of Korea, 31 parasite-proven human echinococcosis cases have been reported, most of which were imported from the Middle East. We recently examined a 61-year-old Korean man who had a large cystic mass in his liver. ELISA was negative for tissue parasitic infections, including echinococcosis, cysticercosis, paragonimiasis, and sparganosis. The patient underwent surgery to remove the cyst, and the resected cyst was processed histopathologically for microscopic examinations. In sectioned cyst tissue, necrotizing protoscolices with disintegrated hooklets of E. granulosus were found. In some areas, only freed, fragmented hooklets were detected. The patient had traveled to western and central Europe in 1996, and had no other history of overseas travel. We report our patient as a hepatic echinococcosis case which was probably imported from Europe.

Human alveolar echinococcosis is a parasitic zoonosis with intermediate (rodents and small lagomorphs) and final (carnivores) hosts. The latter can transmit the parasite to humans, by their feces, which are contaminated by the oncospheres of Echinococcus multilocularis, the larvae of which develop in the liver like a slow cancer. Political, socioeconomic, and ecological factors can affect the intermediate and final hosts and thus influence the long-term emergence and stability of endemic areas. The past 20 years have been marked by: Epidemiologic changes: discovery of an endemic area in China, the largest in the world; extension of the European endemic range to the east and north; extension of the French endemic range to the west and south; and the settlement of contaminated foxes in urban centers, which will modify the populations at risk over time. Progress in diagnosis: initial use of liver ultrasound to screen for asymptomatic disease; value of magnetic resonance imaging to confirm diagnosis and assess extension of disease before beginning treatment; mastery of immunological and genetic diagnosis; and the introduction of positron emission tomography combined with computed tomography to assess the functional activity of the lesions and monitor treatment. Consensus has defined the main directions of a multidisciplinary treatment approach: radical liver resection when possible; avoidance of palliative surgical procedures; use of interventional radiology or endoscopy to treat biliary and vascular complications, whenever possible; consideration of liver transplantation only when all other treatment possibilities have been exhausted; and treatment of all patients, without exception, by albendazole for at least two years after radical surgery and long term (at least several years) in other cases. In France, survival and quality of life have improved very substantially, due to earlier diagnosis, clinical classification of cases, and rigorous follow-up as part of the FrancEchino network. Better understanding of the immunological and immunogenetic mechanisms that underlie the course of disease may open up the possibility of immunomodulation that could replace or round out the current treatments. These are far from satisfactory, in view of the constraints of prolonged, most often life-long treatment by benzimidazoles and their side effects., (Copyright 2009. Published by Elsevier Masson SAS.)

Infection caused by Echinococcus multilocularis is a rare helminthiasis, human cases have not been diagnosed in Hungary until now. The endemic region is Central Europe; the occurrence of this infection has been reported from most of the neighbouring countries; however, E. multilocularis has been found in the red fox population in Hungary. Summarizing the recent knowledge concerning epidemiological, clinical patterns and therapeutic options, the authors describe the first Hungarian case of alveolar echinococcosis. In the presence of appropriate clinical findings, the possibility of this rare infection has to be considered in the differential diagnosis of infiltrative hepatic lesions.

Human alveolar echinococcosis, caused by the metacestode stage of Echinococcus multilocularis, is one of the most dangerous zoonoses in the temperate and arctic areas of northern hemisphere. The mortality of the disease exceeds 95% in untreated or inadequately treated patients. In the past three decades, the spread of this parasite was observed in Europe as a consequence of human interventions resulting in the population size increase of foxes. The authors demonstrated the presence of E. multilocularis in foxes in all northern counties of Hungary and the existence of hyperendemic regions in the Counties Nógrád and Gyor-Moson-Sopron. The aim of the present paper is to summarize the current knowledge on the life cycle and distribution of E. multilocularis, and the epidemiology, clinical features, diagnosis, treatment and control of human alveolar echinococcosis.

Surveillance for alveolar echinococcosis in central Europe was initiated in 1998. On a voluntary basis, 559 patients were reported to the registry. Most cases originated from rural communities in regions from eastern France to western Austria; single cases were reported far away from the disease-"endemic" zone throughout central Europe. Of 210 patients, 61.4% were involved in vocational or part-time farming, gardening, forestry, or hunting. Patients were diagnosed at a mean age of 52.5 years; 78% had symptoms. Alveolar echinococcosis primarily manifested as a liver disease. Of the 559 patients, 190 (34%) were already affected by spread of the parasitic larval tissue. Of 408 (73%) patients alive in 2000, 4.9% were cured. The increasing prevalence of Echinococcus multilocularis in foxes in rural and urban areas of central Europe and the occurrence of cases outside the alveolar echinococcosis-endemic regions suggest that this disease deserves increased attention.