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<p>Alveolar and cystic echinococcosis are rare zoonotic diseases caused by the larval stages of <i>E.&#xa0;multilocularis</i> and <i>E.&#xa0;granulosus</i>. Due to their tumour-like growth, it is often necessary to differentiate them from neoplasms of the liver both macroscopically and histologically. The larval stage is localized predominantly, but not exclusively, in the liver and is characterized for <i>Echinococcus multilocularis</i> macroscopically by a&#xa0;necrotic, “bread-like” ill-defined lesion with intermingled microcysts; in contrast, lesions of <i>E.&#xa0;granulosus</i> show well-defined “noodle- and grape-like” macrocystic lesions. Histologically, the lesions of <i>E.&#xa0;multilocularis</i> are characterized by a&#xa0;tubular, infiltrative growth accompanied by extensive necrosis and a&#xa0;rim composed of epithelioid cells, a&#xa0;fibrotic zone, and a&#xa0;lymphoplasmacytic infiltrate at the transition to non-infected liver tissue. In <i>E.&#xa0;granulosus</i> the necrotic area is less pronounced, without tubular growth and is followed by a&#xa0;rim area identical to lesions caused by <i>E.&#xa0;multilocularis,</i> with the fibrosis zone being significantly wider. The identification of the strongly PAS-positive fragmented lamellar body in the necrotic area is essential. This structure is narrow and fragmented in the larval stage of <i>E.&#xa0;multilocularis</i>, whereas it is distinctly wider in <i>E.&#xa0;granulosus</i>. The positive immunohistochemical staining with monoclonal antibody (mAb) EmG3 confirms the diagnosis of echinococcosis. Regarding the differential diagnosis by immunohistochemistry, the laminated layer of <i>E.&#xa0;multilocularis</i> stains positive with the monoclonal antibodies (mAb) EmG3 and Em2G11, whereas the laminated layer of <i>E.&#xa0;granulosus</i> sensu is only positive with mAb EmG3. Immunohistochemically, fragments of the lamellar body can be detected in the germinal centres of draining lymph nodes infections caused by <i>E.&#xa0;multilocularis </i>and <i>E.&#xa0;granulosus</i> (small particles of <i>E.&#xa0;multilocularis</i>, SPEMs; and small particles of <i>E.&#xa0;granulosus</i>, SPEGs), thus allowing the diagnosis even on small biopsies without lamellar body.</p>

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Pathologie der Echinokokkose

  • 期刊论文

  • 发表日期：

    2025年8月6日

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  • Thomas F. E. Barth,
  • Michael Reinehr,
  • Hanspeter Frey,
  • Achim Weber

摘要

Alveolar and cystic echinococcosis are rare zoonotic diseases caused by the larval stages of E. multilocularis and E. granulosus. Due to their tumour-like growth, it is often necessary to differentiate them from neoplasms of the liver both macroscopically and histologically. The larval stage is localized predominantly, but not exclusively, in the liver and is characterized for Echinococcus multilocularis macroscopically by a necrotic, “bread-like” ill-defined lesion with intermingled microcysts; in contrast, lesions of E. granulosus show well-defined “noodle- and grape-like” macrocystic lesions. Histologically, the lesions of E. multilocularis are characterized by a tubular, infiltrative growth accompanied by extensive necrosis and a rim composed of epithelioid cells, a fibrotic zone, and a lymphoplasmacytic infiltrate at the transition to non-infected liver tissue. In E. granulosus the necrotic area is less pronounced, without tubular growth and is followed by a rim area identical to lesions caused by E. multilocularis, with the fibrosis zone being significantly wider. The identification of the strongly PAS-positive fragmented lamellar body in the necrotic area is essential. This structure is narrow and fragmented in the larval stage of E. multilocularis, whereas it is distinctly wider in E. granulosus. The positive immunohistochemical staining with monoclonal antibody (mAb) EmG3 confirms the diagnosis of echinococcosis. Regarding the differential diagnosis by immunohistochemistry, the laminated layer of E. multilocularis stains positive with the monoclonal antibodies (mAb) EmG3 and Em2G11, whereas the laminated layer of E. granulosus sensu is only positive with mAb EmG3. Immunohistochemically, fragments of the lamellar body can be detected in the germinal centres of draining lymph nodes infections caused by E. multilocularis and E. granulosus (small particles of E. multilocularis, SPEMs; and small particles of E. granulosus, SPEGs), thus allowing the diagnosis even on small biopsies without lamellar body.