回顾性分析浙江大学医学院附属儿童医院2022年10月确诊的1例纵隔及椎管内卡波西型血管内皮瘤(KHE)患儿的临床资料,并对纵隔KHE进行文献复习,总结其临床特征及治疗情况。患儿,男,25 d,以难治性血小板减少为主要表现,多次胸片示纵隔显著增宽,左上肺不典型肺不张表现,经胸部增强CT及磁共振成像诊断为纵隔及椎管内KHE。治疗上采用鸡尾酒疗法(糖皮质激素+长春新碱+西罗莫司)后血小板升至正常,凝血功能改善。目前随访3个月,血液参数稳定,肿块缩小,无药物不良反应。 Clinical data of one patient diagnosed with kaposiform hemangioendothelioma (KHE) in the spinal canal and mediastinum at the Children′s Hospital, Zhejiang University School of Medicine in October 2022 were retrospectively analyzed。Additionally, a literature review on mediastinal KHE was performed to summarize prior clinical characteristics and treatments。The patient, a 25-day-old boy, presented with refractory thrombocytopenia。Multiple chest radiographs suggested significant widening of the mediastinum and atypical atelectasis in the left upper lung, and enhanced chest CT and magnetic resonance imaging suggested KHE。After cocktail therapy with Prednisolone, Vincristine and Sirolimus, platelets increased to normal, and coagulation function improved。After 3 months of follow-up, hematologic parameters were stable, the mass became smaller, and no side effects occurred。
Case report of neonatal kaposiform hemangioendothelioma in the spinal canal and mediastinum with Kasabach-Merritt phenomenon
Clinical data of one patient diagnosed with kaposiform hemangioendothelioma (KHE) in the spinal canal and mediastinum at the Children′s Hospital, Zhejiang University School of Medicine in October 2022 were retrospectively analyzed.Additionally, a literature review on mediastinal KHE was performed to summarize prior clinical characteristics and treatments.The patient, a 25-day-old boy, presented with refractory thrombocytopenia.Multiple chest radiographs suggested significant widening of the mediastinum and atypical atelectasis in the left upper lung, and enhanced chest CT and magnetic resonance imaging suggested KHE.After cocktail therapy with Prednisolone, Vincristine and Sirolimus, platelets increased to normal, and coagulation function improved.After 3 months of follow-up, hematologic parameters were stable, the mass became smaller, and no side effects occurred.