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

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Abstract

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.

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Acknowledgements

The authors would like to acknowledge Alexandra Costa for her valuable contribution in language editing.

Contributions

Rui Caetano Oliveira was responsible for gathering data and manuscript design. Luigi Terracciano added discussion to the results and provided manuscript review. Maria Augusta Cipriano was responsible for work supervision, diagnosis and manuscript review. All authors read and approved the manuscript final version.

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Authors and Affiliations

  1. Pathology Department, Serviço de Anatomia Patológica, Centro Hospitalar e Universitário de Coimbra, piso-3, Praceta Mota Pinto, 3000-075, Coimbra, Portugal

    Rui Caetano Oliveira & Maria Augusta Cipriano

  2. Pathology Department, Basel University Hospital, Basel, Switzerland

    Luigi Terracciano

Authors
  1. Rui Caetano Oliveira
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  2. Luigi Terracciano
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  3. Maria Augusta Cipriano
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Corresponding author

Correspondence to Rui Caetano Oliveira.

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This study was performed in accordance with the ethical standards defined by Centro Hospitalar e Univeristário de Coimbra.

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The authors declare that they have no conflict of interest.

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Oliveira, R.C., Terracciano, L. & Cipriano, M.A. Primary biliary adenosarcoma of the liver—a special and new entity. Virchows Arch 477, 461–466 (2020). https://doi.org/10.1007/s00428-020-02783-y

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  • DOI: https://doi.org/10.1007/s00428-020-02783-y

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