ArticleVirchows Archiv : an international journal of pathology2023
Retroperitoneal malignant extra-gastrointestinal neuroectodermal tumor with EWSR1::CREM fusion and IL-6-related systemic inflammatory symptoms: a case report.
Article in Virchows Archiv : an international journal of pathology, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers.
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Who cites it
5 citing papers in PubMed, 6 citations in OpenAlex.
- Angiomatoid fibrous histiocytoma: first report of adjacent venous invasion.Journal of surgical case reports · 2026Article
- Identification of a distinct epigenetic subgroup with inferior PFS in intracranial mesenchymal tumors with FET::CREB fusion.Brain pathology (Zurich, Switzerland) · 2026Article
- A rare case of clear cell sarcoma-like/malignant gastrointestinal neuroectodermal tumor in the pancreas: case report and literature review.Frontiers in medicine · 2026Article
- A peculiar mimicker of gastro-entero-pancreatic neuroendocrine tumors: Malignant Gastrointestinal Neuroectodermal Tumor - literature review and one case report.Archive of clinical cases · 2025Review
- Malignant gastrointestinal neuroectodermal tumor: a case report and literature review.Annals of medicine and surgery (2012) · 2023Article
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Authors and funding
9 authors at 2 institutions in 1 country.
Funding
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Abstract
Malignant gastrointestinal neuroectodermal tumors (GNETs) are mesenchymal tumors that typically arise in the digestive tract and harbor EWSR1::ATF1 or EWSR1::CREB1 fusions. We report a case of primary retroperitoneal GNET in a 38-year-old woman who presented with a month-long fever with increased serum IL-6 level. A right retroperitoneal mass of 7 cm consisting of diffuse sheets of small cells with a high nuclear-to-cytoplasmic ratio and scattered osteoclast-like multinucleated giant cells was confirmed apart from the digestive tract. Peripheral lymphoid cuff and focal pseudoangiomatous spaces were present, reminiscent of angiomatoid fibrous histiocytoma. The tumor cells were positive for S100 protein and SOX10 and negative for melanocytic markers. Fluorescent in situ hybridization revealed EWSR1 and CREM gene rearrangements, consistent with EWSR1::CREM fusion, which has never been reported in GNET. The patient lives with recurrent lesions for 8 months. This case was associated with several unusual features and contributes to the evolving GNET concept.
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