Sclerosing Epithelioid Fibrosarcoma of the Liver in a Pediatric Patient
| العنوان: | Sclerosing Epithelioid Fibrosarcoma of the Liver in a Pediatric Patient |
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| المؤلفون: | Amy Armstrong, Fouad Boulos, Sakil Kulkarni, Janis Stoll, Maria Bernadette Majella Doyle, Adeel Khan, Mai He |
| المصدر: | Pediatric and Developmental Pathology. 26:153-160 |
| بيانات النشر: | SAGE Publications, 2023. |
| سنة النشر: | 2023 |
| مصطلحات موضوعية: | Pediatrics, Perinatology and Child Health, General Medicine, Pathology and Forensic Medicine |
| الوصف: | Sclerosing epithelioid fibrosarcoma (SEF) is a rare but aggressive sarcoma. We report the first case of hepatic SEF in pediatric patient, which is also the second case in literature. A 17-year-old previously healthy female presented with a liver mass measuring 13.7 cm in greatest dimension and mild elevation of liver enzymes and cancer antigen 19-9. Needle biopsy revealed multiple cores of liver parenchyma mostly replaced by densely hyalinized fibrotic tissue and areas of small-to-medium sized epithelioid cells with eosinophilic and clear cytoplasm. Immunohistochemistry (IHC) demonstrated diffuse strong cytoplasmic staining of MUC4, suggesting a working diagnosis of sclerosing epithelioid fibrosarcoma (SEF)/low-grade fibromyxoid sarcoma (LGFMS). Liver explant demonstrated a well-circumscribed, nodular mass with firm, gray-white cut surface, and similar histopathology as seen in needle biopsy with no convincing evidence suggesting LGFMS. Sequencing panel revealed EWSR1::CREB3L1 gene fusion and confirmed the diagnosis of SEF. Post-operative cancer antigen 19-9 normalized 3 months after transplant; follow-up 3 and 6 months post-transplant imaging at that time showed no concern for disease recurrence. |
| تدمد: | 1615-5742 1093-5266 |
| DOI: | 10.1177/10935266221146378 |
| URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_________::7c70cbcea3599be5ad7c7361244bad19 https://doi.org/10.1177/10935266221146378 |
| Rights: | CLOSED |
| رقم الانضمام: | edsair.doi...........7c70cbcea3599be5ad7c7361244bad19 |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 16155742 10935266 |
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| DOI: | 10.1177/10935266221146378 |