ArticleBMC cancer2026
Integrated molecular pathology and therapeutic outcomes in dermatofibrosarcoma protuberans: Ki-67, COL1A1::PDGFB, and efficacy of Mohs surgery with postoperative imatinib in 153 Chinese patients.
Article in BMC cancer, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
backgroundDermatofibrosarcoma protuberans (DFSP) is a rare cutaneous sarcoma with a high local recurrence rate. In one of the largest single-center Asian cohorts (n = 153), we performed integrated pathologic, molecular, and long-term therapeutic analysis to define the prevalence of COL1A1::PDGFB fusion and evaluate the efficacy of Mohs micrographic surgery and postoperative imatinib.
methodsWe retrospectively analyzed 153 consecutive DFSP cases (2018-2023). COL1A1::PDGFB fusion was detected by FISH with reflex RNA sequencing in negative cases; Ki-67 expression was assessed by immunohistochemistry. Treatment outcomes were evaluated in 124 patients with a median follow-up of 26.9 months using descriptive statistics and risk ratio estimates.
resultsFusion transcripts were identified in 145 of 153 tumours (94.8%). Fibrosarcomatous DFSP showed larger size and higher Ki-67 index than conventional DFSP (5.1 vs 4.1 cm, P = 0.023; 23.4% vs 9.1%, P < 0.001). Among 122 patients followed after MMS, five relapsed (4.1%). Twenty-eight fibrosarcomatous cases received postoperative imatinib for ≥ 6 months and only two recurred (7.1%). Ki-67 ≥ 15% strongly correlated with fibrosarcomatous change.
conclusionCOL1A1::PDGFB fusion remains the molecular hallmark of DFSP. A Ki-67 index ≥ 15% indicates aggressive biology and should prompt meticulous surgical planning and consideration of postoperative imatinib in high-risk tumours.
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