Danz Beate, Hellmann Andrea, Stadie Volker, Dunst Jürgen, Richter Christine, Marsch Wolfgang Ch, Helmbold Peter
Department of Dermatology Martin Luther University Halle-Wittenberg, Klinik und Poliklinik für Hautkrankheiten Ernst-Kromayer-Str. 5 D-06097 Halle (Saale), Germany.
Eur J Dermatol. 2005 Nov-Dec;15(6):474-7.
We report an 80-year-old woman, suffering from a recurrence of a multilocalized lymphedema-associated angiosarcoma of the right arm. The tumor consisted of solid tumor cell formations and "classical" spongiform tumor complexes. In the tumor periphery, pathological endothelial cell proliferates on pre-existing dilated lymphatic capillaries were detectable, which, together with immunohistology (CD 31+/Desmoplakin-1-2.17+/CD 34-), supported the diagnosis of lymphangiosarcoma. Complete remission was achieved under radioimmunotherapy (54 Gy/Interferon beta). A further recurrence 3 months later outside the primary therapy fields was successfully treated with radiotherapy alone. During a follow-up observation period of 3 years, there was neither local recurrence nor metastasis. This case demonstrates for the first time the long-lasting efficacy of photon radiation in a case of histologically-defined lymphangiosarcoma. Further studies should elucidate the suitability of radio monotherapy as first-line therapy in lymphedema-associated angiosarcoma with lymphatic endothelium-like immunohistology.
我们报告了一名80岁女性，患有右臂多部位淋巴水肿相关血管肉瘤复发。肿瘤由实体肿瘤细胞结构和“经典”海绵状肿瘤复合体组成。在肿瘤周边，可检测到先前扩张的淋巴管上有病理内皮细胞增殖，这与免疫组织化学（CD 31+/桥粒斑蛋白-1 - 2.17+/CD 34-）一起支持淋巴管肉瘤的诊断。在放射免疫治疗（54 Gy/干扰素β）下实现了完全缓解。3个月后在初次治疗区域外的再次复发仅通过放射治疗成功治愈。在3年的随访观察期内，既无局部复发也无转移。该病例首次证明了光子辐射在组织学确诊的淋巴管肉瘤病例中的长期疗效。进一步的研究应阐明放射单药治疗作为具有淋巴管内皮样免疫组织学的淋巴水肿相关血管肉瘤一线治疗的适用性。
