angiosarcoma of liver ICD-10: C22.3; ICD-O: 9120/3
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Mann, 58 J., Chemiearbeiter, u.a. mit der Synthese von Polyvinylchlorid
beschäftigt. Autopsiepräparat, Leber stark vergrössert,
von teils nekrotischen und hämorrhagischen Tumorknoten durchsetzt
- Fokale Dilatation der Sinusoide ("Peliosis
hepatis") mit hypertrophen und hyperplastischen, dysplastischen,
polymorphen, proliferativen Sinusendothelien, welche die Leberzellen
umhüllen.
- Zwischen sarkomatösen Sinusendothelien und hyperplastischen
Leberzellplatten vermehrt Kollagenfasern
- Zunehmende Atrophie der Leberzellplatten
- Herdförmig blutgefüllte Kavernen,
umgeben von dicken, bindegewebigen Mauern, austapeziert mit Sarkomzellen
- Herdförmig kompaktes Muster: Fischzugartig gelagerte Sarkomzellen
- Nekroseherde
Der endotheliale Phänotyp der Tumorzellen
kann durch immunzytochemischen Nachweis von Faktor VIII-related antigen
nachgewiesen werden
58-year-old male used to work in a chemical plant where he was involved in the synthesis of vinyl chloride (specimen from autopsy).
- Tumor cells grow along pre-existing vascular channels (sinusoids, portal vein branches).
- Sinusoids are focally dilated (Peliosis
hepatis) and lined by spindle-shaped, rounded or irregular eosinophilic cells with hyperchromatic nuclei. The latter may be elongated or irregular in shape.
- Collagen between sinus endothelium and hyperplastic hepatocytes (Leberzellplatten).
- Sinusoidal growth of tumor cells leads to atrophy of neighboring liver cells.
- Blood-filled caverns (Kavernen) surrounded by a thick, fibrotic wall are lined by sarcoma cells.
- In some areas solidly packed tumor cells arranged in a herring-bone pattern are distinguished, reminiscent of a fibrosarcoma.
- Extensive areas of necrosis (Nekroseherde).
- Remaining liver parenchyma with ductular hyperplasia along portal tracts. Focal hyperplasia of liver parenchyma. Thrombosis in a branch of the portal vein.
Tumor cells (Tumorzellen) express factor VIII-related antigen by immunohistochemistry, in accordance with their endothelial phenotype. CD31 and CD34 are two additional useful markers.
Macroscopy: The liver is diffusely enlarged and pervaded by multiple in part grayish-white in part red-brown hemorrhagic tumor nodules.