---
title: "Giant Cell-Rich Soft Tissue Tumors: Clinicopathologic and Molecular Update"
id: "pubmed-42674819"
canonical_url: "https://medichelpline.com/clinical-feed/pubmed-42674819"
content_type: "clinical_feed_article"
specialty: "Oncology"
source_name: "PubMed / NCBI"
source_url: "https://pubmed.ncbi.nlm.nih.gov/42674819/"
doi: "10.21873/cgp.20607"
published_at: "2026-09-01T00:00:00.000Z"
evidence_level: "Journal Article"
license: "CC-BY-NC-4.0 / Informational Use"
---
# Giant Cell-Rich Soft Tissue Tumors: Clinicopathologic and Molecular Update
## Provenance & Clinical Metadata
- **Canonical URL:** https://medichelpline.com/clinical-feed/pubmed-42674819
- **Specialty:** [Oncology](https://medichelpline.com/clinical-feed/oncology.md)
- **Primary Source:** PubMed / NCBI
- **Source URL:** [Original Journal Publication](https://pubmed.ncbi.nlm.nih.gov/42674819/)
- **DOI:** [10.21873/cgp.20607](https://doi.org/10.21873%2Fcgp.20607)
- **Published At:** 2026-09-01T00:00:00.000Z
- **Evidence Rating:** Journal Article
## Executive GIST (TL;DR)
- Giant cell-rich tumors of soft tissue are diagnostically challenging because multiple entities show pronounced morphological overlap, especially in small or limited samples. - The review summarizes clinicopathologic and molecular features of nine tumor types: **nodular fasciitis**, **juvenile xanthogranuloma**, **phosphaturic mesenchymal tumor**, **tenosynovial giant cell tumor**, **giant cell fibroblastoma**, **giant cell-rich solitary fibrous tumor**, **giant cell tumor of soft tissue**, **keratin-positive giant cell-rich tumor**, and **undifferentiated pleomorphic sarcoma**. - Recent genomic studies have identified signature, diagnostically defining molecular drivers for several of these entities. - Characteristic gene fusions noted in the review include **USP6**, **NTRK1**, **FN1**, **CSF1**, **COL1A1-PDGFB**, **NAB2-STAT6**, and **HMGA2-NCOR2**. - Some giant cell-rich lesions are instead characterized by non-recurrent or highly complex genomic alterations rather than a single defining fusion. - The authors emphasize the growing importance of advanced **molecular testing** to resolve diagnostic ambiguity, refine classification, and potentially guide targeted therapy decisions. - The review is authored by Jun Nishio and Mikiko Aoki and published in Cancer Genomics Proteomics (2026 Sep-Oct;23[5]:899-913), PMID 42674819, DOI 10.21873/cgp.20607. - Keywords highlighted include giant cell-rich tumors, differential diagnosis, gene fusions, molecular diagnostics, and soft tissue neoplasms.
## Clinical Analysis & Structured Key Points
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Affiliations Expand ### Affiliations * 1 Section of Orthopaedic Surgery, Department of Medicine, Fukuoka Dental College, Fukuoka, Japan; nishio@fdcnet.ac.jp. * 2 Department of Pathology, Faculty of Medicine, Fukuoka University, Fukuoka, Japan. * PMID: **42674819** * DOI: [ 10.21873/cgp.20607 ](https://doi.org/10.21873/cgp.20607) Item in Clipboard Review # An Update on Selected Giant Cell-Rich Tumors of Soft Tissue Jun Nishio et al. Cancer Genomics Proteomics. 2026 Sep-Oct. Show details Display options Display options Format Abstract PubMed PMID Cancer Genomics Proteomics Actions * [ Search in PubMed ](https://pubmed.ncbi.nlm.nih.gov/?term=%22Cancer+Genomics+Proteomics%22%5Bjour%5D&sort=date&sort_order=desc) * [ Search in NLM Catalog ](https://www.ncbi.nlm.nih.gov/nlmcatalog?term=%22Cancer+Genomics+Proteomics%22%5BTitle+Abbreviation%5D) * [ Add to Search ](https://pubmed.ncbi.nlm.nih.gov/42674819/) . 2026 Sep-Oct;23(5):899-913. doi: 10.21873/cgp.20607. ### Authors [Jun Nishio](https://pubmed.ncbi.nlm.nih.gov/?term=Nishio+J&cauthor_id=42674819)[ 1 ](https://pubmed.ncbi.nlm.nih.gov/42674819/#short-view-affiliation-1 "Section of Orthopaedic Surgery, Department of Medicine, Fukuoka Dental College, Fukuoka, Japan; nishio@fdcnet.ac.jp."), [Mikiko Aoki](https://pubmed.ncbi.nlm.nih.gov/?term=Aoki+M&cauthor_id=42674819)[ 2 ](https://pubmed.ncbi.nlm.nih.gov/42674819/#short-view-affiliation-2 "Department of Pathology, Faculty of Medicine, Fukuoka University, Fukuoka, Japan.") ### Affiliations * 1 Section of Orthopaedic Surgery, Department of Medicine, Fukuoka Dental College, Fukuoka, Japan; nishio@fdcnet.ac.jp. * 2 Department of Pathology, Faculty of Medicine, Fukuoka University, Fukuoka, Japan. * PMID: **42674819** * DOI: [ 10.21873/cgp.20607 ](https://doi.org/10.21873/cgp.20607) Item in Clipboard Cite Display options Display options Format Abstract PubMed PMID ## Abstract Giant cell-rich tumors of soft tissue present a significant diagnostic challenge due to pronounced morphological overlap, particularly in limited tissue samples. This review provides a streamlined update on the clinicopathological and molecular features of nine distinct entities: nodular fasciitis, juvenile xanthogranuloma, phosphaturic mesenchymal tumor, tenosynovial giant cell tumor, giant cell fibroblastoma, giant cell-rich solitary fibrous tumor, giant cell tumor of soft tissue, keratin-positive giant cell-rich tumor, and undifferentiated pleomorphic sarcoma. While these neoplasms are unified by a prominence of multinucleated giant cells, recent genomic insights have identified signature, diagnostically defining molecular drivers. We highlight characteristic gene fusions, including USP6, NTRK1, FN1, CSF1, COL1A1-PDGFB, NAB2-STAT6, and HMGA2-NCOR2, as well as entities characterized by non-recurrent or highly complex genomic alterations. Synthesizing these data, this review underscores the critical role of advanced molecular testing in resolving diagnostic ambiguities, refining tumor classification, and guiding targeted therapeutic strategies. **Keywords:** Giant cell-rich tumors; differential diagnosis; gene fusions; molecular diagnostics; review; soft tissue neoplasms. Copyright © 2026 International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved. 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