Recent advances in surgical strategies for alveolar echinococcosis of the liver

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REVIEW ARTICLE

Recent advances in surgical strategies for alveolar echinococcosis of the liver Toshiya Kamiyama1 Received: 24 May 2019 / Accepted: 16 October 2019 © Springer Nature Singapore Pte Ltd. 2019

Abstract Liver resection is the safest intervention for alveolar echinococcosis (AE), because the only potentially curative treatment is complete removal of the lesion. In combination with medical anthelmintic treatment, a safe distance of at least 1 mm is permissible in this procedure. Even when liver resection does not cure AE, good long-term survival outcomes can be achieved if most of the lesion has been removed and the disease is controlled with lifelong benzimidazole treatment. If the residual lesion is comparatively small and does not contain a closed space that may adhere to the surrounding tissue and form an abscess, complications such as sepsis arising from an abscess on the cut surface can be prevented and the required biliary drainage might be relatively simple. Larger AE lesions that invade the inferior vena cava can be treated effectively with the recent advances in reduction surgical techniques. An effective concentration of albendazole (ABZ) is found only in the periphery of AE lesions, because this drug penetrates the lesions passively. Liver transplantation, with adjuvant ABZ and the administration of appropriate immunosuppressive agents such as cyclosporin A, is indicated for patients with end-stage AE. Keywords  Alveolar echinococcosis · Liver · Hepatectomy Abbreviations AE Alveolar echinococcosis WHO World Health Organization IWGE Informal Working Group on Echinococcosis TNM Tumor–node–metastasis IVC Inferior vena cava ABZ Albendazole LT Liver transplantation LDLT Living-donor liver transplantation IS Immunosuppressive status

Background Alveolar echinococcosis (AE) is a zoonosis caused by the larval stage of Echinococcus multilocularis. Cystic echinococcosis (CE) is caused by E. granulosus. AE is prevalent in the northern hemisphere, including Central Europe and Japan, and many researchers have focused on its endemicity * Toshiya Kamiyama t‑[email protected] 1



Department of Gastroenterological Surgery I, Hokkaido University Graduate School of Medicine, North 5, West 7, Kita‑ku, Sapporo 060‑8638, Japan

[1–3], whereas CE has a global distribution. However, over the past 2 decades, extensive epidemiological research has revealed a significant expansion of AE into Northern, Eastern, and Western Europe [4]. In Japan, a gene associated with AE was recently detected in the feces of wild dogs in Saitama Prefecture (2005) [5] and Aichi Prefecture (2018) [6], although Hokkaido was the previous endemic area. AE causes liver tumors that result in infiltrative growth and distant metastases. Clinically, AE behaves like a malignant tumor and the prognosis is generally poor. AE is a serious disease with a greater than 90% mortality rate in untreated patients [7]. Chemotherapy using albendazole (ABZ) and mebendazole and surgical hepatectomy are the accepted treatment options for AE. The