A case report of a novel inflammatory and incarcerated testicular stromal tumor associated with the EWSR1::ATF1 gene fusion
- 1Department of Pathology, the First Affiliated Hospital of Shihezi University, Shihezi, Xinjiang 832000 China
- 2Department of Pathology, Hongxing Hospital of the 13th Division of Xinjiang Production and Construction Corps, Xinxing, Xinjiang 839000 China
- 3Department of Pathology, School of Medicine, Shihezi University, Shihezi, Xinjiang 832000 China
Abstract
Background: This case report describes a novel subtype of testicular sex cord-stromal tumor, termed the “inflammatory and nested testicular sex cord tumor”, which is characterized by a specific EWSR1::ATF1 gene fusion. We present the clinicopathological features of this case to enhance the understanding of this extremely rare tumor.
Case presentation: A 45-year-old male patient presented with a right testicular mass that had been present for six years without an identifiable cause. MRI conducted revealed a round nodule in the right testicular area, measuring approximately 38 × 28 mm, with relatively well-defined margins. The tumor exhibited a nodular growth, consisting of epithelial-like cells with abundant clear cytoplasm arranged in solid nests or sheets. Fibrous connective tissue surrounded the epithelial-like tumor nests, and focal inflammatory cell infiltration was observed within the stroma and tumor periphery. Immunohistochemistry showed membranous positivity for α-inhibin in the epithelial-like component. Molecular testing revealed an EWSR1::ATF1 gene fusion, leading to a final diagnosis of inflammatory and nested testicular sex cord tumor.
Conclusions: We report a novel subtype of testicular sex cord-stromal tumor, distinct from conventional forms, characterized by nest-like growth of clear epithelial-like tumor cells, focal inflammatory cell infiltration, and the presence of the EWSR1::ATF1 gene fusion. This case provides further evidence that supports the fusion pattern and clinicopathological basis for the newly proposed subtype of testicular sex cord-stromal tumor, inflammatory and nested testicular sex cord tumor.
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BibTeX
@article{wei2026case,
title = {{A case report of a novel inflammatory and incarcerated testicular stromal tumor associated with the EWSR1::ATF1 gene fusion}},
author = {Li Wei and Shasha Lv and Qin Guo and Wei Jia},
journal = {Diagnostic Pathology},
year = {2026},
month = aug,
doi = {10.1186/s13000-026-01835-2},
url = {https://doi.org/10.1186/s13000-026-01835-2},
note = {CC BY 4.0},
}
RIS
TY - JOUR
TI - A case report of a novel inflammatory and incarcerated testicular stromal tumor associated with the EWSR1::ATF1 gene fusion
AU - Li Wei
AU - Shasha Lv
AU - Qin Guo
AU - Wei Jia
PY - 2026
DA - 2026/08/10
JO - Diagnostic Pathology
DO - 10.1186/s13000-026-01835-2
UR - https://doi.org/10.1186/s13000-026-01835-2
AB - Background: This case report describes a novel subtype of testicular sex cord-stromal tumor, termed the “inflammatory and nested testicular sex cord tumor”, which is characterized by a specific EWSR1::ATF1 gene fusion. We present the clinicopathological features of this case to enhance the understanding of this extremely rare tumor. Case presentation: A 45-year-old male patient presented with a right testicular mass that had been present for six years without an identifiable cause. MRI conducted revealed a round nodule in the right testicular area, measuring approximately 38 × 28 mm, with relatively well-defined margins. The tumor exhibited a nodular growth, consisting of epithelial-like cells with abundant clear cytoplasm arranged in solid nests or sheets. Fibrous connective tissue surrounded the epithelial-like tumor nests, and focal inflammatory cell infiltration was observed within the stroma and tumor periphery. Immunohistochemistry showed membranous positivity for α-inhibin in the epithelial-like component. Molecular testing revealed an EWSR1::ATF1 gene fusion, leading to a final diagnosis of inflammatory and nested testicular sex cord tumor. Conclusions: We report a novel subtype of testicular sex cord-stromal tumor, distinct from conventional forms, characterized by nest-like growth of clear epithelial-like tumor cells, focal inflammatory cell infiltration, and the presence of the EWSR1::ATF1 gene fusion. This case provides further evidence that supports the fusion pattern and clinicopathological basis for the newly proposed subtype of testicular sex cord-stromal tumor, inflammatory and nested testicular sex cord tumor.
SN - 1746-1596
ER -