Publications by authors named "E G Demicco"
Background: Differentiation of benign myxomas and malignant myxoid sarcomas can be difficult with an overlapping spectrum of morphologic MR findings.
Purpose: To assess the diagnostic utility of MRI radiomics in the differentiation of musculoskeletal myxomas and myxoid sarcomas.
Study Type: Retrospective.
The aim of the study was to report the outcome of primary localized low-grade fibromyxoid sarcoma (LGFMS), sclerosing epithelioid fibrosarcoma (SEF), and hybrid LGFMS/SEF (H-LGFMS/SEF). Patients with primary localized LGFMS, SEF, or H-LGFMS/SEF, surgically treated with curative intent from January 2000 to September 2022, were enrolled from 14 countries and 27 institutions. Pathologic inclusion criteria were predefined by expert pathologists.
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- Solitary fibrous tumor (SFT) is a rare type of tumor that can occur in various body parts and is often linked to specific genetic fusions, with 10-30% of cases becoming metastatic.
- A study involving DNA methylation analysis of 79 SFTs revealed distinct epigenetic changes linked to their primary sites, identifying key genes such as EGFR and TBX15 that showed differing levels of expression based on the tumor's location and genetic fusion type.
- TBX15 emerged as a significant marker, with changes in its methylation and expression strongly correlating to the tumor's tissue of origin, suggesting it could help differentiate between new tumors and metastases without needing extensive genomic analysis.
Article Synopsis
- A retrospective study by the Ultra-Rare Sarcoma Working Group analyzed data from 101 patients with metastatic low-grade fibromyxoid sarcoma (LGFMS), sclerosing epithelioid fibrosarcoma (SEF), and hybrid (H)-LGFMS/SEF across 28 centers from January 2000 to September 2022.
- The study measured progression-free survival (PFS) from the detection of metastasis and after starting treatment, revealing varying effectiveness among the different sarcoma types and treatments.
- Findings showed that metastatic LGFMS and SEF had distinct survival outcomes, with systemic treatments showing limited effectiveness, suggesting a need for new therapeutic options in future research.
Article Synopsis
- Acral fibrochondromyxoid tumor (AFCMT) is a recently identified, likely benign tumor found in the hands and feet, characterized by distinct histological features and a specific genetic fusion (THBS1::ADGRF5).
- In a study of 37 cases, the tumors predominantly affected middle-aged individuals (median age 47) and displayed well-defined structures with vascularity and chondrocyte-like cells in a chondromyxoid environment.
- Immunohistochemical tests showed most tumors positive for CD34 and ERG but negative for S100 protein, indicating that AFCMT is a unique entity distinct from other similar tumors.