Desmoplastic small round cell tumor of the liver: diagnosing a rare case on liver biopsy.
Desmoplastic small round cell tumor
EWSR1
Liver
WT1
Journal
Diagnostic pathology
ISSN: 1746-1596
Titre abrégé: Diagn Pathol
Pays: England
ID NLM: 101251558
Informations de publication
Date de publication:
29 Jul 2023
29 Jul 2023
Historique:
received:
05
06
2023
accepted:
11
07
2023
medline:
31
7
2023
pubmed:
30
7
2023
entrez:
29
7
2023
Statut:
epublish
Résumé
Desmoplastic small round-cell tumors (DSRCT) frequently develop in the retroperitoneum, pelvis, omentum, and mesentery. Here, we present an unusual case of primary DSRCT in the liver. The patient was an 11-year-old boy with multiple solid masses in the liver parenchyma. The tumor in the needle biopsy had a histology revealing a small round cell morphology and desmoplasia. It shows the immunohistochemical features of DSRCT and documentation of EWSR1-WT1 fusion.A potential diagnostic pitfall is exerted when evaluating liver biopsy, in which DSRCT is a great mimicker and may be easily confused with more common liver malignancies of childhood, such as hepatoblastoma, calcifying nested stromal-epithelial tumor, undifferentiated embryonal sarcoma, and other small round cell tumors, as well as the fibrolamellar variant of hepatocellular carcinoma. This distinction is critical because an accurate therapeutic approach requires a correct diagnosis.
Identifiants
pubmed: 37516860
doi: 10.1186/s13000-023-01373-1
pii: 10.1186/s13000-023-01373-1
pmc: PMC10386280
doi:
Types de publication
Case Reports
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
84Subventions
Organisme : National Regional Medical Center Opening Project
ID : No. NRMC0103
Informations de copyright
© 2023. The Author(s).
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