ArticleVirchows Archiv : an international journal of pathology2022
Intra-abdominal EWSR1/FUS-CREM-rearranged malignant epithelioid neoplasms: two cases of an emerging aggressive entity with emphasis on misleading immunophenotype.
Article in Virchows Archiv : an international journal of pathology, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers.
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Who cites it
19 citing papers in PubMed, 28 citations in OpenAlex.
- Angiomatoid fibrous histiocytoma: a review.Journal of pathology and translational medicine · 2026Article
- Pulmonary mesenchymal cystic hamartoma with EWSR1::CREM fusion: molecular redefinition and diagnostic implications.Virchows Archiv : an international journal of pathology · 2026Article
- Clinicopathologic characteristics of uterine malignant epithelioid neoplasms with EWSR1::CREM fusion: Report of 2 cases at a novel site with literature review.Virchows Archiv : an international journal of pathology · 2026Article
- GLI2::CREBBP fusions define a subset of potentially aggressive nested-hyalinizing epithelioid soft tissue neoplasms.Virchows Archiv : an international journal of pathology · 2026Article
- ALK-Rearranged renal neoplasms: update on an institutional cohort.Virchows Archiv : an international journal of pathology · 2025Article
- Durable Response to Pazopanib (Tyrosine Kinase Inhibitor) in a Patient With EWSR1::CREM Gene Fusion Positive Intra-Abdominal Unclassified Epithelioid Sarcoma.Genes, chromosomes & cancer · 2025Review
- Malignant epithelioid tumors with EWSR1::CREB fusion involving the kidney: a report of two cases.Virchows Archiv : an international journal of pathology · 2025Article
- Expanding the spectrum of FUS::CREM-rearranged neoplasms: a case of mesenchymal malignant tumor with neuroendocrine differentiation.Virchows Archiv : an international journal of pathology · 2025Article
- EWSR1::CREM rearranged intra-abdominal malignant epithelioid neoplasm: two new cases of an emerging entity with clinicopathological characteristics and histological pitfalls.Virchows Archiv : an international journal of pathology · 2025Article
- A case report and literature review: an intra-abdominal epithelioid neoplasm with EWSR1::CREB fusions originating from the kidney.Frontiers in oncology · 2025Article
- The challenge of diagnosing neuroendocrine neoplasms: experience from a national reference center.Virchows Archiv : an international journal of pathology · 2024Article
- EWSR1::ATF1 fusions characterize a group of extra-abdominal epithelioid and round cell mesenchymal neoplasms, phenotypically overlapping with sclerosing epithelioid fibrosarcomas, and intra-abdominal FET::CREB fusion neoplasms.Virchows Archiv : an international journal of pathology · 2024Article
- Malignant Epithelioid Mesenchymal Neoplasm with FUS::CREM Gene Fusion Arising in the Tongue: A Case Report Detailing Clinicopathological, Imaging, and Molecular Features.Head and neck pathology · 2024Article
- Elucidating the Diagnostic Complexity of Round Cell Sarcoma with EWSR1-CREM Fusion: A Comprehensive Case Study.Medicina (Kaunas, Lithuania) · 2024Article
- Case report: Primary pulmonary low grade fibromyxoid sarcoma progressing to dedifferentiation: probably due toFrontiers in oncology · 2024Article
- Novel NONO::TFE3 fusion and ALK co-expression identified in a subset of cutaneous microcystic/reticular schwannoma.Virchows Archiv : an international journal of pathology · 2023Article
- CREB fusion-associated epithelioid mesenchymal neoplasms of the female adnexa: three cases documenting a novel location of an emerging entity and further highlighting an ambiguous misleading immunophenotype.Virchows Archiv : an international journal of pathology · 2023Article
- Metastatic sporadic paraganglioma with EWSR1::CREM gene fusion: A unique molecular profile that expands the phenotypic diversity of the molecular landscape of the EWSR1::CREM gene fusion positive tumors.Genes, chromosomes & cancer · 2023Article
- Mesenchymal/non-epithelial mimickers of neuroendocrine neoplasms with a focus on fusion gene-associated and SWI/SNF-deficient tumors.Virchows Archiv : an international journal of pathology · 2021Article
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9 authors at 4 institutions in 1 country.
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No grant is acknowledged in the PubMed record.
Abstract
CREB family (CREB1, ATF1, and CREM) gene fusions are defining markers in diverse mesenchymal neoplasms (clear cell sarcoma, angiomatoid fibrous histiocytoma, and others). However, neoplasms harboring EWSR1-CREM/FUS-CREM fusions are rare and poorly characterized. We describe two cases (55-year-old male with 7.5 cm renal mass and 32-year-old female with 5.5 cm mesenteric mass) illustrating their misleading immunophenotypes. Histologically, both showed eosinophilic and focally clear epithelioid cells arranged into sheets, nests, and trabeculae. Immunohistochemistry showed ALK, EMA, and AE1/AE3 immunoreactivity suggesting ALK-rearranged renal cell carcinoma (Case 1) and coexpression of keratin, EMA, synaptophysin, and chromogranin-A, suggesting neuroendocrine neoplasm (Case 2). Targeted RNA sequencing revealed EWSR1-CREM (Case 1) and FUS-CREM (Case 2) fusions. These cases add to the spectrum of CREM fusion-positive intra-abdominal epithelioid neoplasms. Their unusual immunophenotype and unexpected sites represent major pitfalls, underline a wide differential diagnosis, and emphasize the value of molecular testing in correctly diagnosing them.
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