Primary renal sarcoma with SS18::POU5F1 gene fusion.


Journal

Genes, chromosomes & cancer
ISSN: 1098-2264
Titre abrégé: Genes Chromosomes Cancer
Pays: United States
ID NLM: 9007329

Informations de publication

Date de publication:
09 2022
Historique:
revised: 25 04 2022
received: 12 04 2022
accepted: 01 05 2022
pubmed: 7 5 2022
medline: 14 7 2022
entrez: 6 5 2022
Statut: ppublish

Résumé

We report the first case of a primary renal undifferentiated sarcoma harboring an SS18::POU5F1 gene fusion. The patient was a 38 year-old male diagnosed with a 5 cm renal tumor which invaded the adrenal gland and extended into the renal vein. Microscopically, the neoplasm had a predominantly undifferentiated round cell morphology, with areas of rhabdoid and spindle cell growth. Similar to the previously reported cases with this fusion, by immunohistochemistry the neoplasm expressed S100 protein and epithelial markers (diffuse EMA, focal cytokeratin), suggesting the possibility of a myoepithelial phenotype. This report documents another example of a fusion-positive undifferentiated soft tissue sarcoma occurring as a primary renal neoplasm, adding to the already broad list of such entities. It highlights the crucial role of molecular analysis in establishing a specific diagnosis given the overlapping morphology and immunophenotypes such entities may exhibit.

Identifiants

pubmed: 35521683
doi: 10.1002/gcc.23053
pmc: PMC9271602
mid: NIHMS1807804
doi:

Substances chimiques

Biomarkers, Tumor 0
Octamer Transcription Factor-3 0
Oncogene Proteins, Fusion 0
POU5F1 protein, human 0
Proto-Oncogene Proteins 0

Types de publication

Case Reports Journal Article Research Support, N.I.H., Extramural

Langues

eng

Sous-ensembles de citation

IM

Pagination

572-577

Subventions

Organisme : NCI NIH HHS
ID : P30 CA008748
Pays : United States
Organisme : NCI NIH HHS
ID : P50 CA217694
Pays : United States
Organisme : NCI NIH HHS
ID : P50 CA140146
Pays : United States

Informations de copyright

© 2022 The Authors. Genes, Chromosomes and Cancer published by Wiley Periodicals LLC.

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Auteurs

Pedram Argani (P)

Departments of Pathology, The Johns Hopkins Medical Institutions, Baltimore, Maryland, USA.
Departments of Oncology, The Johns Hopkins Medical Institutions, Baltimore, Maryland, USA.

Andres Matoso (A)

Departments of Pathology, The Johns Hopkins Medical Institutions, Baltimore, Maryland, USA.
Departments of Oncology, The Johns Hopkins Medical Institutions, Baltimore, Maryland, USA.
Departments of Urology, The Johns Hopkins Medical Institutions, Baltimore, Maryland, USA.

John M Gross (JM)

Departments of Pathology, The Johns Hopkins Medical Institutions, Baltimore, Maryland, USA.
Departments of Orthopedics, The Johns Hopkins Medical Institutions, Baltimore, Maryland, USA.

Yanming Zhang (Y)

Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, United States.

Jeffrey A SoRelle (JA)

Department of Pathology, University of Texas Southwestern Medical Center, Dallas, Texas, United States.

Jeffrey Gagan (J)

Department of Pathology, University of Texas Southwestern Medical Center, Dallas, Texas, United States.

Cristina R Antonescu (CR)

Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, United States.

Doreen Palsgrove (D)

Department of Pathology, University of Texas Southwestern Medical Center, Dallas, Texas, United States.

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Classifications MeSH