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  • Title: [Role of master transcriptional factor Prox-1 in lymphatic endothelial differentiation of Kaposiform hemangioendothelioma].
    Author: Ke ZY, Yang SJ.
    Journal: Zhonghua Bing Li Xue Za Zhi; 2017 Mar 08; 46(3):176-181. PubMed ID: 28297758.
    Abstract:
    Objective: To analyze the clinical and pathological features of Kaposiform hemangioendothelioma (KHE), and to investigate the role of master transcriptional factor Prox-1 in the regulation of lymphatic differentiation. Methods: Nine cases of KHE (during the period from October 2009 to June 2016) were collected with clinical and pathological data. H&E stained section review and immunohistochemietry using the Dako EnVision method were performed. Results: There were 6 female and 3 male patients with age ranging from 2 months to 8 years (median 3 years and 4 months). The patients presented with either single subcutaneous soft tissue mass, or bone tumors, with the duration of disease onset ranging from 1 month to 1 year. The sites of involvement included the skins of neck (2 cases), nose root (1 case), inguinal (1 case), thigh root (1 case), humerus (2 cases), lumbar vertebrae(1 case), and mesentery (1 case). These tumors were histologically composed of nodules of densely packed spindle or ovoid cells and deformed small blood vessels in an invasive growth pattern. The tumor cells were immunohistochemically positive for both blood vessels and lymphatic endothelial markers, including Prox-1, the master transcriptional factor, and VEGFR-3. With followed-up from 1 to 60 months (median 26 months), two patients died of the disease, while the remaining patients were alive without recurrence. Conclusions: KHE is a rare vascular tumor with at least partial lymphatic endothelial differentiation, in which Prox-1 may act as a master regulator for such differentiation. KHE is an aggressive tumor of intermediate malignant potential, with local invasion and recurrence tendency, and long term follow-up is required. 目的： 分析Kaposi样血管内皮细胞瘤(KHE)的临床病理学特征及预后，并探讨关键性转录因子Prox-1在KHE淋巴管内皮分化中的作用。 方法： 收集2009年10月至2016年6月间9例KHE的临床病理资料，其中男3例，女6例。年龄2个月～8岁，平均年龄约3岁4个月。5例发生于皮肤(颈部2例、鼻根部1例、腹股沟1例、大腿根部1例)，2例发生于肱骨，1例发生于腰3椎骨，1例发生于肠系膜。临床上多表现为单发软组织肿块，术前病程1个月至1年。采用EnVision两步法免疫组织化学染色，对其进行临床病理分析。 结果： 该组病例组织形态上由球状或丛状分布的梭形细胞或卵圆形细胞组成，呈浸润性生长。免疫组织化学结果显示，血管及淋巴管内皮标志物均阳性，包括淋巴管内皮细胞关键性转录因子Prox-1和血管内皮细胞生长因子受体3。9例患者随访时间1～ 60个月(平均26个月)，其中，除2例患者死亡外，其余均无病生存。 结论： KHE是一种罕见的血管肿瘤，具有部分淋巴管内皮分化，其中，关键性转录因子Prox-1可能发挥重要转录调节作用。该肿瘤具有侵袭性和复发倾向，在条件允许的情况下，完整切除是其首选的治疗方法。.
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