Echinococcus multilocularis

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Echinococcus multilocularis /
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Background

Microbiology

  • Cestode in the Echinococcus family
  • Disease is caused by the larval form

Life Cycle

  • Definitive hosts are canid carnivorous animals, including urban coyotes
  • Eggs contaminate the environment, including produce
    • They are infective after a few days, and are viable for up to 16 months at 4ºC
  • Intermediate hosts (metacestode) are rodents, possibly pigs, and and incidentally humans
  • Intermediate host is eaten by the definitive host, completing the life cycle

Epidemiology

  • Estimated 18,000 cases annually
  • Worldwide in the northern hemisphere
    • Most cases in China (90%), followed by Japan and Russia
    • Rare in North America
  • Within Canada:
    • Associated with arctic foxes and wolves
    • Alberta has highest number of cases, with 19 cases from 2013 to 2021
    • 0 to 3 case reports in other provinces

Clinical Manifestations

  • Causes alveolar echinococcosis
  • Incubation period 5 to 15 years
  • Vague abdominal pain may precede diagnosis by years
  • Presents as a metastatic malignancy of unknown primary, mot commonly with malaise, weight loss, and right upper quadrant discomfort
  • Can cause cholestatic jaundice, cholangitis, portal hypertension, and Budd-Chiari syndrome

Diagnosis

  • Combination of radiologic and serologic investigations
  • A single negative serologic test does not rule out infection
  • May be confirmed by biopsy

Case Definition (Alberta)

  • Typical organ lesion detected by imaging with US or CT
  • AND histopathology compatible with alveolar echinococcosis
  • AND
    • Detection of E. multilocularis by serology
    • OR detection of E. multilocularis by molecular diagnostic methods

Imaging

  • Ultrasound or CT demonstrates lesions which typically have an irregular contour, no well-defined wall, central necrosis, and irregular intralesional and wall calcifications

PNM Classification

  • P=parasitic mass in the liver, N=neighbouring organ involvement; M=metastases
P N M
X Primary tumour cannot be assess Not evaluable Not evaluable
0 No detectable tumour in liver No regional involvement No metastasis
1 Peripheral lesions without proximal vascular and/or biliary involvement Regional involvement of contiguous organs or tissues Metastasis
2 Central lesions with proximal vascular and/or biliary invovlement of 1 lobe
3 Central lesions with hilar vascular or biliary involvement of both lobes and/or with involvement of 2 hepatic veins
4 Any liver lesion with extension along the vessels and biliary tree

Management

  • Main treatment is surgical resection followed by at least 2 years of antihelminthic therapy (e.g. albendazole)
  • Poor prognosis, with 90% 10-year mortality if untreated