Wen Xin, Zou Daopei, Ban Fazhan, Wang Lei
Department of Dermatology, Xijing Hospital, The Fourth Military Medical University, Xi'an, China.
Department of Dermatology, The First Affiliated Hospital of Chongqing Medical University, Chongqing, China.
J Dermatolog Treat. 2025 Dec;36(1):2511114. doi: 10.1080/09546634.2025.2511114. Epub 2025 Jun 16.
Benign lymphangioendothelioma is a rare tumor originating from lymphatic vessels. Its underlying mechanisms remain unclear, and effective therapeutic interventions are limited.
The goal of this study was to investigate the potential hyperactivation of the mTOR signaling pathway in benign lymphangioendothelioma. Additionally, to preliminarily assess the clinical efficacy and safety of the mTOR inhibitor sirolimus in management.
Immunohistochemical staining was utilized to evaluate the activation status of mTOR pathway-related proteins in benign lymphangioendothelioma. Three patients with confirmed hyperactivation of the mTOR signaling pathway were administered with oral sirolimus, the clinical efficacy and safety of sirolimus tablets in treating benign lymphangioendothelioma were subsequently monitored.
A total of eight patients were included in this study. Immunohistochemical analysis was conducted on seven patients, overexpression of mTOR-related molecules was observed in each case. Three patients received treatment with oral sirolimus, and all three demonstrated significant regression of their skin lesions, with no notable adverse effects.
The activation of mTOR signaling pathway is implicated in the pathogenesis of benign lymphangioendothelioma, suggesting a potential similarity in the underlying mechanisms with lymphatic malformations. The mTOR inhibitor sirolimus demonstrated promising therapeutic efficacy, accompanied by a favorable safety profile.
良性淋巴管内皮瘤是一种起源于淋巴管的罕见肿瘤。其潜在机制尚不清楚，有效的治疗干预措施有限。
本研究旨在探讨mTOR信号通路在良性淋巴管内皮瘤中是否存在潜在的过度激活。此外，初步评估mTOR抑制剂西罗莫司在治疗中的临床疗效和安全性。
采用免疫组织化学染色评估良性淋巴管内皮瘤中mTOR通路相关蛋白的激活状态。对3例确诊为mTOR信号通路过度激活的患者给予口服西罗莫司，随后监测西罗莫司片治疗良性淋巴管内皮瘤的临床疗效和安全性。
本研究共纳入8例患者。对7例患者进行了免疫组织化学分析，每例均观察到mTOR相关分子的过表达。3例患者接受口服西罗莫司治疗，所有3例患者的皮肤病变均显著消退，且无明显不良反应。
mTOR信号通路的激活与良性淋巴管内皮瘤的发病机制有关，提示其与淋巴管畸形在潜在机制上可能存在相似性。mTOR抑制剂西罗莫司显示出有前景的治疗效果，且安全性良好。
