ArticleModern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc2020
Recurrent novel THBS1-ADGRF5 gene fusion in a new tumor subtype "Acral FibroChondroMyxoid Tumors".
Article in Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 8 papers.
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8 citing papers in PubMed, 25 citations in OpenAlex.
- Chondroid Soft Tissue Tumors With FOS::PABPN1 Fusion: A New Entity? About Two Cases.Genes, chromosomes & cancer · 2026Article
- Acral Mesenchymal Spindle Cell Neoplasm With a Novel HMGA2::NCOA2 Fusion.Journal of cutaneous pathology · 2026Article
- CD34-Positive Acral Chondromyxoid Mesenchymal Neoplasm Harboring a Novel TCF4::ERG Fusion.Genes, chromosomes & cancer · 2025Article
- Superficial acral calcified chondroid mesenchymal neoplasm harboring an FN1::FGFR2 fusion and review of the literature.Journal of cutaneous pathology · 2024Review
- Recurrent Inflammatory Myofibroblastic Tumor of Larynx Harboring a Novel THBS1::ALK Fusion.International journal of genomics · 2024Review
- The clinical relevance of the adhesion G protein-coupled receptor F5 for human diseases and cancers.Biochimica et biophysica acta. Molecular basis of disease · 2023Review
- Acral FibroChondroMyxoid tumor: imaging features of a new entity.Skeletal radiology · 2021Article
- Acral Fibrochondromyxoid Tumor Presenting as Enlarging Nodule Involving the Distal Fingertip and Hyponychium: A Case Report.Case reports in dermatologyArticle
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Authors and funding
14 authors at 10 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Acral soft tissue tumors are common neoplasms, a subset of which pose a diagnostic challenge. We report 10 cases of a previously unrecognized acral benign soft tissue tumor. These tumors arose on the fingers and toes and involved bone in half of cases. Histologically, the tumors were lobulated and displayed an abundant stroma made of variable fibrous, chondroid and myxoid material reminiscent of cartilaginous or myoepithelial differentiation. Tumor cells harbored small round to reniform nuclei with clear chromatin and inconspicuous nucleoli along with scant eosinophilic cytoplasm. The cells were mostly arranged haphazardly in the stroma but also in small clusters. No mitotic activity was detected. No specific feature was identified in recurrent cases. By immunohistochemistry, the cells consistently stained for CD34 (10/10), ERG (9/10), and SOX9 (7/10). Whole RNA sequencing identified a previously undescribed recurrent in frame THBS1-ADGRF5 gene fusion in all cases. The transcript was confirmed by RT-PCR and was not found in the control group of mimickers including soft tissue chondromas. We propose the name of Acral FibroChondroMyxoid Tumors for this new entity.
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