Abstract
Alveolar echinoccocosis (AE) is a rare and potentially lethal zoonosis that primarily affects the liver and is caused by larvae of the tapeworm Echinococcus multilocularis, which occurs in the northern hemisphere. AE is an illness with tumor-like characteristics, such as infiltrative growth and metastatic potential.
Diagnosis consists of imaging studies (ultrasonography, CT, and magnetic resonance) and serological tests. All patients are treated by long-term benzimidazoles therapy and if possible by radical surgery.
In exceptional cases, a liver transplantation (LTx) can be performed. LTx is considered in patients with advanced, symptomatic and radically inoperable disease.
Recurrence of AE has been linked with immunocompetence. The risk of recurrence is higher with post-transplant immunosuppressive treatment.
Currently, non-surgical interventions, such as percutaneous drainage of bile ducts are preferred, which address most complications. The number of LTx due to AE is diminishing in Europe.