Primary biliary adenosarcoma of the liver—a special and new entity

Rui Caetano Oliveira1, Luigi Terracciano2, Maria Augusta Cipriano1
1Pathology Department, Serviço de Anatomia Patológica, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal
2Pathology Department, Basel University Hospital, Basel, Switzerland

Tóm tắt

Primary sarcoma of the liver (PSL) is a rare entity accounting for less than 1% of all liver cancers, with unknown aetiology. Several subtypes have been recognized, with histology playing an essential role in patient tailoring and management. We are about to report a case of an unusual PSL, with peculiar morphologic and immunohistochemical properties. A 65-year-old female with a multicystic hepatic lesion underwent surgery due to spontaneous rupture. Pathology revealed dilated bile duct-like structures lined by benign cuboidal/columnar epithelium surrounded by neoplastic proliferation of spindle and epithelioid cells with interspersed osteoclast-like multinucleated giant cells, loosely arranged in a storiform pattern, positive for CD10. The conjugation of morphology and immunohistochemistry results provided the diagnosis of primary biliary adenosarcoma of the liver, biliary type. The patient experienced tumour relapse and died of disease 2 years and 7 months later.

Tài liệu tham khảo

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