Successful Treatment of Kaposiform Hemangioendothelioma and Tufted Angioma With Vincristine
Successful Treatment of Kaposiform Hemangioendothelioma and Tufted Angioma With Vincristine
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DOI：
10.1097/mph.0b013e3181e001a9
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发表时间：
2010-08-01
影响因子：
1.2
通讯作者：
Arbuckle, Susan
中科院分区：
文献类型：
--
作者：
Fahrtash, Farzan;McCahon, Emma;Arbuckle, Susan
背景：卡波西状血管内皮瘤和簇状血管瘤是两种罕见的局部侵袭性血管肿瘤。虽然目前被归类为单独的实体，但它们越来越被认为是同一病理学的一个谱。KHE和TA被认为是中度恶性肿瘤，因为它们具有浸润性生长、局部侵袭性和可变的预后。到目前为止，这些血管肿瘤的明确治疗已经取得了有限的success.Aim：评估的安全性，有效性和长春新碱在治疗KHE和TA.Methods的作用：病例回顾的患者档案和病理报告在儿童医院在Westmead从1995年至2009年。长春新碱治疗7例。长春新碱组的生存率为100%。平均诊断年龄为30个月（范围从出生到9岁）。女性6例（85.7%）。平均随访时间为4年（范围4个月至8年）。长春新碱治疗的7例病例中，3例患者出现相关的Kasabach-Merritt现象（43%）。完全消退1例（14%），大小消退3例（43%），镇痛改善2例（29%）。结论：阿曲斯汀是治疗KHE/TA的有效药物。它与低副作用特征相关，应被视为一线药物。
Background: Kaposiform hemangioendothelioma (KHE) and tufted angioma (TA) are rare, locally aggressive vascular tumors. Although currently classified as separate entities, they are becoming increasingly recognized as a spectrum of the same pathology. There is a well-recognized association with Kasabach-Merritt phenomenon KHE and TA are considered neoplasms of intermediate malignancy because of infiltrative growth, local aggressiveness, and variable prognosis. To date, definitive treatment for these vascular tumors has had limited success.Aim: To evaluate the safety, efficacy, and role of vincristine in the treatment of KHE and TA.Methods: Case review of patient files and pathology reports at The Children's Hospital at Westmead from 1995 to 2009.Results: Twelve cases with KHE or TA were identified. Seven cases were treated with vincristine. The survival rate in the vincristine group was 100%. Mean age of diagnosis was 30 months (range birth to 9 y). 6 patients were female (85.7%). Mean time of the follow-up was 4 years (range 4 mo to 8 y). Out of the 7 cases treated with vincristine, 3 patients had associated Kasabach-Merritt phenomenon (43%). Complete resolution, regression in size, and improvement in analgesia were found in 1 case (14%), 3 cases (43%) and 2 cases (29%), respectively. Vincristine related side effects occurred in 2 cases (29%).Conclusions: Vincristine is an effective treatment option for KHE/TA. It is associated with a low side effect profile and should be considered as the first-line agent.