30-yr Course and Favorable Outcome of Alveolar Echinococcosis Despite Multiple Metastatic Organ Involvement in a Non-immune Suppressed Patient

Karine Bardonnet; Dominique A Vuitton; Frédéric Grenouillet; Georges A Mantion; Eric Delabrousse; Oleg Blagosklonov; Jean-Philippe Miguet; Solange Bresson-Hadni

Disclosures

Ann Clin Microbiol Antimicrob. 2013;12(1) 

In This Article

Abstract and Introduction

Abstract

We report the 30-yr history of a well-documented human case of alveolar echinococcosis, with a lung lesion at presentation followed by the discovery of a liver lesion, both removed by surgery. Subsequently, within the 13 years following diagnosis, metastases were disclosed in eye, brain and skull, as well as additional lung lesions. This patient had no immune suppression, and did not have the genetic background known to predispose to severe alveolar echinococcosis; it may thus be hypothesized that iterative multi-organ involvement was mostly due to the poor adherence to benzimidazole treatment for the first decade after diagnosis. Conversely, after a new alveolar echinococcosis recurrence was found in the right lung in 1994, the patient accepted to take albendazole continuously at the right dosage. After serology became negative and a fluoro-deoxy-glucose-Positron Emission Tomography performed in 2005 showed a total regression of the lesions in all organs, albendazole treatment could be definitively withdrawn. In 2011, the fluoro-deoxy-glucose-Positron Emission Tomography showed a total absence of parasitic metabolic activity and the patient had no clinical symptoms related to alveolar echinococcosis.
The history of this patient suggests that multi-organ involvement and alveolar echinococcosis recurrence over time may occur in non-immune suppressed patients despite an apparently "radical" surgery. Metastatic dissemination might be favored by a poor adherence to chemotherapy. Combined surgery and continuous administration of albendazole at high dosage may allow alveolar echinococcosis patients to survive more than 30 years after diagnosis despite multi-organ involvement.

Introduction

Alveolar Echinococcosis (AE) caused by the metacestode of the "fox tapeworm" Echinococcus (E.) multilocularis is one of the most lethal helminthic diseases in humans.[1,2] Humans become infected through contact with eggs (oncospheres) present in the feces of the definitive hosts, most often foxes or dogs, but also wolves and cats, by handling the animals or by ingesting contaminated vegetables without cooking them. Only observed in the northern hemisphere, and especially in central Europe, Russia/Siberia, Central Asia, Western China, north of Japan, and Alaska, AE is also one among the rare parasitic diseases with a surgical treatment, because of its "tumor-like" progression. The only available antiparasitic chemotherapy, i.e. high doses of albendazole (ABZ) or mebendazole (MBZ) given continuously, is only parasitostatic in this disease and the complete resection of the parasitic lesions is thus recommended whenever possible.[3,4] In most of the cases, the parasitic lesions are initially located in the liver and may then invade adjacent organs; however, true metastases may also be seen in any organ or tissue. Extra-hepatic locations were already present at diagnosis in 34% of cases in those cases recorded in the EurEchinoReg European registry from 1982 to 2000.[5] When the clinical symptoms at presentation are related to such extra-hepatic locations, the diagnosis of AE is difficult and very often it is confirmed after surgery on the pathological aspects of the lesions and/or evidence of its parasitic nature by PCR.[6,7] Except for lung metastases, AE metastatic dissemination is often associated with immune deficiency of the host.[8] "Multi-organ AE" usually qualifies cases with hepatic, pulmonary and cerebral locations;[9,10] metastases to more than 2 organs/tissues are extremely rare, either simultaneously or successively, in immune-competent subjects. The involvement of several organs is among the main causes of poor prognosis.[11,12] We report here the case of an immune-competent man with an AE discovered from a lung metastasis in 1981 who had 5 different locations of the disease, either simultaneously or successively, underwent 4 surgical operations on 6 different organs/tissues in 4 different locations, and who may nevertheless be considered cured from the disease after a unique follow-up of more than 30 years.

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