博来霉素-电硬化疗法（BEST）治疗淋巴管畸形（LMs）的安全性及临床疗效
Safety and Clinical Outcome of Bleomycin-Electrosclerotherapy (BEST) Treating Lymphatic Malformations (LMs).
作者信息
Obereisenbuchner Florian, Borisch Elena, Puhr-Westerheide Daniel, Deniz Sinan, Cay Ferdi, Schirren Mirjam, Biechele Gloria, Schregle Robin, Häberle Beate, Haehl Julia, Hartl Alexandra, Fröba-Pohl Alexandra, Kashani Fatemeh, Ricke Jens, Seidensticker Max, Wildgruber Moritz, Schmidt Vanessa F
机构信息
Department of Radiology, LMU University Hospital, LMU Munich, Marchioninistraße 15, 83177, Munich, Germany.
Interdisciplinary Center for Vascular Anomalies (IZGA), LMU University Hospital, LMU Munich, Munich, Germany.
出版信息
Cardiovasc Intervent Radiol. 2025 Sep 4. doi: 10.1007/s00270-025-04169-6.
INTRODUCTION
Bleomycin-electrosclerotherapy (BEST) is a novel treatment for slow-flow vascular malformations (SFVMs), most studied in venous malformations. This study specifically evaluated its safety and clinical outcome in lymphatic/lymphatic-dominant lympho-venous malformations (LMs/ld-LVMs).
MATERIALS AND METHODS
A monocentric cohort with symptomatic LMs or ld-LVMs treated by BEST was retrospectively assessed. A treatment-specific, patient-reported questionnaire assessed overall clinical response (complete response: symptom-free, partial response: improved symptoms, no response: unchanged symptoms, progression of symptoms), subjective health-related quality of life (QoL; optimal, improved, unchanged, worsening), pain (numerical rating scale, NRS), and postprocedural skin discolouration (yes/no). Pre and postprocedural lesion size was measured in three planes on MRI.
RESULTS
Twenty-seven treatments were performed in 20 patients with 14 LMs and six ld-LVMs (11 microcystic, five mixed, and four macrocystic subtypes). Patients received 1.4 ± 0.6 treatments with a median bleomycin dose of 7 mg (range 2-15 mg). After BEST, 7/20 (35%) patients reported complete response, 10/20 (50%) partial response, and 3/20 (15%) no response. Health-related QoL was stated as optimal in 10/20 (50%) and as improved in 4/20 (20%) patients. Median pain NRS was reduced from 6 (3-10) to 2 (0-6). Postprocedural skin discolouration occurred in 11/20 (55%) patients. Follow-up MR imaging revealed lesion size reduction from mean maximum volume of 793 cm (IQR 155-2199 cm) to 548 cm (IQR 71-1059 cm). Total complication rate (CIRSE grade 3-4) was 11.1%. No differences in all outcome parameters regarding LMs subtypes were assessed.
CONCLUSION
BEST demonstrates efficacy and acceptable safety treating LMs and ld-LVMs, including challenging microcystic lesions previously considered difficult to treat.
LEVEL OF EVIDENCE
3b, Retrospective Cohort Study.
引言
博来霉素-电硬化疗法（BEST）是一种治疗缓慢血流性血管畸形（SFVMs）的新方法，在静脉畸形方面研究最多。本研究专门评估了其在淋巴管/以淋巴管为主的淋巴静脉畸形（LMs/ld-LVMs）中的安全性和临床结果。
材料与方法
对一组接受BEST治疗的有症状LMs或ld-LVMs的单中心队列进行回顾性评估。一份针对治疗的患者报告问卷评估了总体临床反应（完全缓解：无症状，部分缓解：症状改善，无反应：症状无变化，症状进展）、主观健康相关生活质量（QoL；最佳、改善、无变化、恶化）、疼痛（数字评分量表，NRS）和术后皮肤变色（是/否）。在MRI上于三个平面测量术前和术后病变大小。
结果
对20例患者进行了27次治疗，其中14例为LMs，6例为ld-LVMs（11例微囊型、5例混合型和4例大囊型亚型）。患者接受了1.4±0.6次治疗，博来霉素中位剂量为7mg（范围2-15mg）。BEST治疗后，7/20（35%）的患者报告完全缓解，10/20（50%）部分缓解，3/20（15%）无反应。10/20（50%）的患者表示健康相关生活质量最佳，4/20（20%）的患者表示有所改善。疼痛NRS中位数从6（3-10）降至2（0-6）。11/20（55%）的患者出现术后皮肤变色。随访MRI显示病变大小从平均最大体积793cm（IQR 155-2199cm）减小至548cm（IQR 71-1059cm）。总并发症发生率（CIRSE 3-4级）为11.1%。未评估LMs各亚型在所有结局参数上的差异。
结论
BEST在治疗LMs和ld-LVMs方面显示出疗效和可接受的安全性，包括先前认为难以治疗的具有挑战性的微囊型病变。
证据水平
3b，回顾性队列研究。
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