Aim This systematic review aims to comprehensively evaluate the clinicopathological and molecular features of superficial CD34-positive fibroblastic tumour (SCD34FT), a recently described intermediate-grade soft-tissue neoplasm recognised in the 5th edition of the WHO classification of soft tissue tumours. Methods Following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) 2020 guidelines, we systematically searched for English-language studies in PubMed, Scopus, Google Scholar and Web of Science. Retrospective or original case series with ≥3 histologically confirmed cases were selected for review, whereas review articles, single case reports and conference abstracts were excluded. Risk of bias was assessed using the Joanna Briggs Institute checklist. Results A total of 190 patients across 14 selected studies met the inclusion criteria. SCD34FT mainly affected middle-aged adults, with the most common site being the lower extremity. Diffuse CD34 expression was universal across all studies. A high number of cases displayed SynCAM/CADM3 positivity, while WT1 and Pan-CK (focal) were frequent. PRDM10 fusion was detected in 73% of cases, and the most common fusions were PRDM10::MED12 (66%) and PRDM10::CITED2 (28%). Only 9/169 (5.3%) had local recurrences, whereas 4/190 (2.1%) cases had lymph node metastasis, with no distant metastasis or disease-related death. Conclusion SCD34FT is a novel entity with consistent CD34 expression and recurrent PRDM10 rearrangements and shows indolent behaviour. A high index of suspicion for SCD34FT should be warranted in a superficially located tumour with striking nuclear pleomorphism but an unexpectedly low mitotic index. Molecular studies demonstrating PRDM10 rearrangement may be helpful in atypical cases.
Das et al. (2026) studied this question.