Primary hepatic carcinoma with inhibin positivity in a young male patient: a rare tumor previously only reported in females-case report and review of literature.


Journal

Virchows Archiv : an international journal of pathology
ISSN: 1432-2307
Titre abrégé: Virchows Arch
Pays: Germany
ID NLM: 9423843

Informations de publication

Date de publication:
Mar 2021
Historique:
received: 18 02 2020
accepted: 03 06 2020
revised: 28 05 2020
pubmed: 14 6 2020
medline: 31 3 2021
entrez: 14 6 2020
Statut: ppublish

Résumé

Primary hepatic carcinoma with inhibin positivity is a rare aggressive liver tumor with seven cases described. The tumor presents at a younger age than primary hepatic carcinoma with all cases being females. RNA albumin ISH positivity suggests the tumor to be a primary hepatic carcinoma. The tumor is different from hepatocellular carcinoma as well as intrahepatic cholangiocarcinoma because of its distinct morphology, lack of hepatocellular differentiation, strong inhibin staining, and lack of typical mutations. A 26-year-old male presented with a 20-cm liver mass. The tumor progressed on therapy with development of multiple lung metastasis. Currently, the patient is enrolled in phase II clinical trial utilizing nivolumab and ipilumumab. While the tumor has a female preponderance, it is not exclusively found in females. Additional studies are necessary to determine the cause of inhibin staining, driving molecular alterations, natural history of this rare tumor, and to come up with consensus nomenclature.

Identifiants

pubmed: 32533342
doi: 10.1007/s00428-020-02864-y
pii: 10.1007/s00428-020-02864-y
doi:

Substances chimiques

Biomarkers, Tumor 0
Inhibins 57285-09-3

Types de publication

Case Reports Journal Article Review

Langues

eng

Sous-ensembles de citation

IM

Pagination

605-610

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Auteurs

Tom Z Liang (TZ)

Department of Pathology, Keck Medical Center, University of Southern California, Los Angeles, CA, USA.

Gilbert Whang (G)

Department of Radiology, Keck Medical Center, University of Southern California, Los Angeles, CA, USA.

Shefali Chopra (S)

Department of Pathology, Keck Medical Center, University of Southern California, Los Angeles, CA, USA. Shefali.chopra@med.usc.edu.

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Classifications MeSH