Fatal Liver and Lung Alveolar Echinococcosis with Newly Developed Neurologic Symptoms due to the Brain Involvement

Abstract

The fox tapeworm Echinococcus multilocularis causes human alveolar echinococcosis, commonly affecting the liver. However, in ∼1% of cases, systematic spread of the disease involves the brain as well. A patient had a 6-year history of liver and lung alveolar echinococcosis that was considered not suitable for surgery, and treatment with albendazole was introduced. After the appearance of neurologic disturbances, an intracranial mass lesion was demonstrated by radiologic imaging. The lesion was surgically removed, and histologic analysis revealed metacestode tissue of E. multilocularis . Despite the surgical resection of the lesion, the patient died of progression of systemic alveolar echinococcosis. The authors highly recommend implementing neurologic monitoring to the follow-up algorithm for patients with systemically disseminated alveolar echinococcosis. When neurologic symptoms occur, radiologic imaging of the brain should be obtained immediately. Surgery should be considered for all intracranial echinococcal lesions, unless the lesion is located in the eloquent brain area.

Keywords: Echinococcus multilocularis; alveolar echinococcosis; cerebral alveolar echinococcosis.

Fig. 1

Abdominal computed tomography with contrast media. (Left) A mass with diffuse amorphous calcification in the right liver lobe. (Right) The lesion with calcified margins occupying the right liver lobe and involving the portal vein system.

Fig. 2

Thoracic computed tomography with contrast media. (Left) The calcified lesions with lobulated contours in the right lobe of lungs. (Right) The lesion in the left lobe of lungs.

Fig. 3

Brain magnetic resonance imaging. Axial view T2-weighted image (upper left) and coronal view T2-weighted image (bottom left) showing the irregular hypo-/hyperintense nodular lesion in the right postcentral gyrus with an extensive perifocal edema. Coronal fluid-attenuated inversion recovery image (upper right) showing the nodular iso-/hypointense lesion with a massive perifocal edema in the right parietal lobe. Coronal T1-weighted image with contrast enhancement (bottom right) showing the multilocular cystic lesion with a perifocal edema resembling a bunch of grapes.

Fig. 4

Postoperative computed tomography. This image shows the radical removal of the lesion.

Fig. 5

(Upper left) The gross pathologic specimen of the lesion removed during surgery. (Upper right) The section shows periodic acid-Schiff (PAS)-positive cuticle layer characteristic of Echinococcus multilocularis cysts (arrows). PAS stain. (Lower left) The cystic lesion with parasite surrounded by necrotic tissue. Hematoxylin and eosin stain. (Lower right) The surrounding brain tissue infiltrated by lymphocytes, plasmocytes, and multinucleated giant cells (arrow).