High-grade endometrial stromal sarcoma-like' sarcoma in male: Does it exist? A case report and review of the literature

Fleur Cordier; Joni Van der Meulen; Siebe Loontiens; Nadine Van Roy; Lore Lapeire; Wouter Willaert; Liesbeth Ferdinande; Koen Van de Vijver; Jo Van Dorpe; David Creytens

doi:10.1016/j.prp.2022.154228

High-grade endometrial stromal sarcoma-like' sarcoma in male: Does it exist? A case report and review of the literature

Pathol Res Pract. 2023 Jan:241:154228. doi: 10.1016/j.prp.2022.154228. Epub 2022 Nov 17.

Authors

Affiliations

¹ Department of Pathology, Ghent University Hospital, Ghent University, Ghent, Belgium.
² CRIG, Cancer Research Institute Ghent, Ghent University Hospital, Ghent University, Ghent, Belgium; Centre for Medical Genetics, Ghent University Hospital, Ghent University, Ghent, Belgium; Department of Biomolecular Medicine, Ghent University, Ghent, Belgium.
³ CRIG, Cancer Research Institute Ghent, Ghent University Hospital, Ghent University, Ghent, Belgium; Department of Medical Oncology, Ghent University Hospital, Ghent University, Ghent, Belgium.
⁴ CRIG, Cancer Research Institute Ghent, Ghent University Hospital, Ghent University, Ghent, Belgium; Department of GI Surgery, Ghent University Hospital, Ghent University, Ghent, Belgium.
⁵ Department of Pathology, Ghent University Hospital, Ghent University, Ghent, Belgium; CRIG, Cancer Research Institute Ghent, Ghent University Hospital, Ghent University, Ghent, Belgium.
⁶ Department of Pathology, Ghent University Hospital, Ghent University, Ghent, Belgium; CRIG, Cancer Research Institute Ghent, Ghent University Hospital, Ghent University, Ghent, Belgium. Electronic address: [email protected].

PMID: 36455366
DOI: 10.1016/j.prp.2022.154228

Abstract

We report an exceptional case of an undifferentiated round and spindle cell sarcoma, occurring in the periprostatic region of a 54-year-old male, with a 'high-grade endometrial stromal sarcoma-like' (HG-ESS) morphology and harboring a ZC3H7B::BCOR gene fusion identified by RNA-based next-generation sequencing. In this report, we describe the striking overlap of morphologic, immunohistochemical and molecular features of this current case and previously reported similar cases with ZC3H7B::BCOR fusion-positive HG-ESS, and discuss the differential diagnosis and possible pathogenesis of this unusual entity.

Keywords: High-grade endometrial stromal sarcoma; RNA-based next generation sequencing; Sarcoma with BCOR genetic alterations; Undifferentiated sarcoma.

Publication types

Review
Case Reports

MeSH terms

Endometrial Neoplasms* / pathology
Female
Humans
Male
Middle Aged
Proto-Oncogene Proteins / genetics
Repressor Proteins / genetics
Sarcoma* / diagnosis
Sarcoma* / genetics
Sarcoma, Endometrial Stromal* / genetics

Substances

Proto-Oncogene Proteins
Repressor Proteins