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Background <p>Dermatofibrosarcoma 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 <i>COL1A1</i>::<i>PDGFB</i> fusion and evaluate the efficacy of Mohs micrographic surgery and postoperative imatinib.</p> Methods <p>We retrospectively analyzed 153 consecutive DFSP cases (2018–2023). <i>COL1A1</i>::<i>PDGFB</i> 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&#xa0;months using descriptive statistics and risk ratio estimates.</p> Results <p>Fusion 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&#xa0;cm, P = 0.023; 23.4% vs 9.1%, P &lt; 0.001). Among 122 patients followed after MMS, five relapsed (4.1%). Twenty-eight fibrosarcomatous cases received postoperative imatinib for ≥ 6&#xa0;months and only two recurred (7.1%). Ki-67 ≥ 15% strongly correlated with fibrosarcomatous change.</p> Conclusion <p><i>COL1A1</i>::<i>PDGFB</i> 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.</p>

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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

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  • 发表日期：

    2026年4月30日

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  • Jinrui Guo,
  • Yongmei Cui,
  • Nana Zhang,
  • Jing Liang,
  • Jian-Ning Chen,
  • Xiangqi Huang,
  • Dan He

摘要

Background

Dermatofibrosarcoma 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.

Methods

We 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.

Results

Fusion 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.

Conclusion

COL1A1::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.