伴Kasabach-Merritt现象卡波西形血管内皮瘤和丛状血管瘤的临床及病理特征

目的 探讨伴Kasabach-Merritt现象(KMP)的卡波西形血管内皮瘤(KHE)和丛状血管瘤(TA)临床病理特征.方法 对7例伴KMP的KHE和TA病例进行临床及病理学分析.结果 诊断为KHE 5例,TA 2例,均伴有血小板降低.7例均在出生后1月内发病,病灶均位于躯干或四肢.2例快速增大,2例缓慢进展,3例稳定或逐渐消退.二者病灶外观多样,典型表现为暗红或紫红色肿块或斑片.组织学上,KHE和TA均由结节状梭形血管内皮细胞团构成.区别在于KHE内皮细胞团边界不清,红细胞淤滞,含铁血黄素沉积;TA内皮细胞团界限清晰,呈弹坑样分布.口服泼尼松治疗2例,切除植皮1例,效果满意;随访观察4例,病情无进展.结论 KMP病理性质为KHE或TA,前者为中间性肿瘤,具局部侵袭性,后者为良性肿瘤,二者均可部分白行退化,需与其他类型血管性肿瘤鉴别.     

西罗莫司联合泼尼松治疗卡波西样血管内皮瘤伴Kasabach-Merritt现象初探

【摘要】 目的 初步探讨西罗莫司联合泼尼松治疗卡波西样血管内皮瘤（KHE）伴Kasabach-Merritt现象（KMP）的临床价值。方法 回顾性分析2011年1月至2018年1月于四川大学华西医院小儿外科接受西罗莫司联合泼尼松治疗的36例KHE伴KMP患儿的一般临床资料、治疗效果以及不良反应发生情况。结果 36例KHE伴KMP患儿中,男女比例为1∶0.8;平均年龄15个月（4.0 ~ 60.0个月）;平均发病年龄6.3个月（0.8 ~ 48.0个月）;混合型KHE 32例（88.9%）,深部型KHE 4例（11.1%）;瘤体大小为2.5 cm × 4.0 cm ~ 20.0 cm × 24.0 cm。在联合治疗中,泼尼松平均用药时间为6.4周（范围5.0 ~ 9.0周）,西罗莫司平均用药时间为19.3个月（范围13.0 ~ 27.0个月）。联合治疗1 ~ 5周,36例KHE伴KMP患者的血小板和纤维蛋白原逐渐恢复正常。在短期使用泼尼松联合长期使用西罗莫司治疗6、12个月后,疾病严重性平均评分由治疗前4.0分分别下降到2.4分、1.6分。在治疗12个月后,32例患者瘤体达到大部分消退的标准,3例达到部分消退的标准,1例无变化。治疗期间,常见的不良反应为胃肠道不适和口腔溃疡,无患儿因为严重不良反应而中止治疗,也未发生药物毒性相关性死亡。结论 西罗莫司联合泼尼松治疗KHE伴KMP安全有效。     

卡波西样血管内皮瘤/丛状血管瘤发病机制

卡波西样血管内皮瘤(kaposiform hemangioendothelioma,KHE)/丛状血管瘤(tufted angioma,TA)为血管内皮细胞肿瘤,具有局部侵袭性,部分伴发卡梅现象(Kasabach-Merritt phenomenon,KMP),是一种具有潜在死亡风险的危重症.目前已报道的KHE/TA的...     

卡波西形血管内皮瘤一例

患者男,21岁,因左下肢肿胀、疼痛 4个月,伴结节、肿块2个月入院。4个月前患者出现左下肢肿胀,轻度疼痛。2个月前左下肢、腹股沟、会阴部出现蓝紫色及暗红色结节,逐渐增大。同时左下肢肿胀加剧,阴囊及阴茎出现水肿,左下肢活动受限。在外院病理检查怀疑卡波西肉瘤,为进一步诊治而收入院。家族中无类     

梭形细胞血管内皮瘤

报告1例梭形细胞血管内皮瘤.患者男,16岁.右跖部结节10余年,相继波及左足和双小腿下段,无自觉症状.皮损组织病理检查可见真皮中下层由海绵状血管腔隙和梭形细胞组成的团块.依据临床表现和组织病理学改变,诊断为梭形细胞血管内皮瘤.     

梭形细胞血管内皮瘤一例

患者女,43岁。因右下肢皮下结节1年于2013年7月来我院就诊。患者1年前右下肢出现一花生米大蓝青色结节,无明显自觉症状,结节生长缓慢,无发热等全身症状。患者否认既往患有其他皮肤疾病,否认家族成员有类似病史及遗传病史……     

多发性丘疹型血管角皮瘤伴海绵状血管瘤

患儿男,5岁. 主诉:左肩部起多个红色丘疹40余天. 现病史:40余天前家人发现患儿左肩部有2个粟粒大红色丘疹,随后邻近部位渐出现20余个类似的红色丘疹,渐增至绿豆或黄豆大,无自觉症状,部分皮损擦破后易出血.发疹前局部无外伤史.     

先天性毛细血管瘤恶变致血管内皮瘤1例

报告一例先天性毛细血管瘤恶变致血管内皮瘤。患者女，60岁，左膝关节红斑60年，结节、破溃4年。组织病理：皮肤溃疡形成，真皮及皮下血管增生明显，血管壁增厚，管腔闭塞，血管内皮细胞偶见分裂相。免疫组化染色：CD31（＋）。病理诊断：血管内皮瘤。     

卡波西样血管内皮瘤117例临床分析

目的总结分析卡波西样血管内皮瘤(Kaposiform hemangioendothelioma,KHE)患者的临床特点及治疗经验。方法选择并分析2008年1月-2018年12月在四川大学华西医院就诊的117例KHE患者的临床资料,结合相关文献,回顾性总结分析其临床特征、并发症、治疗及预后等特点。结果共入组117例KHE患者,男64例、女53例,比例约为1.20∶1,患者年龄1 d^5岁,平均年龄2.74个月,年龄<1岁的患者105例(89.74%)。伴有KMP(Kasabach-Merritt phenomenon,KMP)者83例,主要临床表现为皮肤损害、皮下或骨关节肌肉的浸润性肿物、伴有运动受限、急性心衰、胸腔积液、心包积液或凝血出血相关疾病等并发症。病灶直径范围2~26 cm,平均直径为9.04 cm。提示年龄、病灶大小与患者是否发生KMP有关(P均<0.05)。采用西罗莫司、泼尼松、长春新碱等药物治疗55例,手术治疗25例,联合治疗32例,未治疗行观察随访5例。转归:临床治疗有效103例(88.03%),9例(7.69%)因急性心衰、呼吸衰竭、弥散性血管内凝血等并发症死亡。结论绝大多数KHE患者在1岁以前发病,并且发生KMP风险更高。KHE临床表现主要与各种并发症有关,早期明确诊断和及时治疗对减少并发症的发生、改善预后十分重要。     

小儿皮肤血管瘤和血管畸形的临床病理特征和鉴别诊断

小儿血管瘤可以自然消退逐渐得到国内外诸多学者的证实和肯定。但关于小儿血管瘤和血管畸形的鉴别诊断,各家标准和意见不一［１～３］。我们详细观察分析了１９９例小儿血管病变的临床病理特征,以利于诊断和临床治疗。材料和方法对我院１９８６年～１９９６年手术切除有...     

Kaposiform hemangioendothelioma without Kasabach-Merritt phenomenon

Kasabach-Merritt phenomenon is a serious coagulopathy associated with kaposiform hemangioendothelioma (KHE), tufted angioma, and possibly other vascular neoplasms. KHE presenting in the absence of Kasabach-Merritt phenomenon is rare, although tufted angioma frequently occurs without thrombocytopenia. We retrospectively reviewed 10 cases of KHE without Kasabach-Merritt phenomenon. The tumors appeared as soft tissue masses with the overlying skin being either normal, erythematous, or violaceous. There were no radiologic or microscopic differences in noncoagulopathic KHE as compared with coagulopathic KHE. Evidence of platelet trapping and hemosiderin deposition was seen histologically, despite normal serum platelet levels. All KHE were less than 8 cm in diameter, suggesting that tumors that grow no larger than this size are less likely to trap platelets in sufficient quantity to cause thrombocytopenia. Our series confirms that KHE appears with a wide spectrum of behavior and response to treatment. The decision as to whether or not to treat a noncoagulopathic KHE should be based on the size and location of the tumor and the possible side effects of therapy.     

Transformation between Kaposiform hemangioendothelioma and tufted angioma

Kaposiform hemangioendothelioma and tufted angioma are viewed as two separate disease entities in the current classification of vascular tumors. However, the concurrence of features of both vascular tumors in the same specimen of some patients raises the question whether these tumors exist on a continuum. Herein, we report a dynamic transformation between both tumors within a single patient, which suggests they are two variants of the same vascular tumor.     

Ultrasonography and magnetic resonance imaging features of kaposiform hemangioendothelioma and tufted angioma

Kaposiform hemangioendothelioma (KHE) and tufted angioma (TA) primarily occurring in infants are difficult to distinguish. This study evaluated ultrasonography (US) and magnetic resonance imaging (MRI) features of KHE and TA. Pathologically proven TA (n = 21) and KHE (n = 40 [11 KHE + Kasabach–Merritt phenomenon [KMP]]) occurring between January 2015 and December 2017 were reviewed. US (n = 61) and MRI (n = 50) findings were retrospectively evaluated. On US, KHE and TA lesions were subcutaneous, while 40% of KHE exhibited an infiltrative pattern extending into adjacent muscles. Of TA lesions, 42.9% were hyperechoic and 96.15% of KHE lesions exhibited mixed echogenicity. Of TA lesions, 76.2% exhibited well‐defined margins and all KHE lesions exhibited ill‐defined margins. The depth and vascular density of KHE and KHE + KMP were significantly increased compared with TA. The arterial peak systolic blood flow velocity of KHE + KMP was significantly higher than that in TA and KHE. KHE and KHE + KMP were significantly harder than TA on elastography. 3‐D color Doppler revealed branch‐shape blood flow for KHE and KHE + KMP lesions; for TA, it revealed a dot‐like and striped pattern. Considering MRI findings, KHE and KHE + KMP were more likely to exhibit diffuse heterogeneous enhancement after contrast than TA. KHE was infiltrative and more likely to be thick, hypoechoic, ill‐defined, richly vascular and hard than TA on US. KHE lesions were subcutaneous and reticular, with heterogeneous enhancement on MRI. Awareness of these features should prompt radiologists in the differential diagnosis of pediatric masses.     

Retroperitoneal kaposiform hemangioendothelioma complicated by Kasabach-Merritt phenomenon and obstructive jaundice: A retrospective series of 3 patients treated with sirolimus

We present a retrospective case series of 3 patients with retroperitoneal kaposiform hemangioendothelioma (KHE) complicated by Kasabach-Merritt phenomenon (KMP) and biliary obstruction. We found sirolimus to be a safe and effective treatment for these patients who were refractory to other treatment modalities. However, our patients were slow to respond in comparison to published reports of sirolimus use for KHE without biliary obstruction. We postulate that therapeutic serum levels of sirolimus may be affected by biliary obstruction and improved with surgical alleviation of the obstruction.     

Acquired tufted angioma associated with pregnancy

Acquired tufted angioma is a distinctive condition that is different from other types of acquired vascular proliferation. Despite the progressive spread of these angiomas, they appear to be benign, and malignant change has not been encountered. We describe a case of recurrent acquired tufted angioma associated with pregnancy, an association which has not been previously recorded.     

Kasabach-Merritt综合征13例临床分析

目的了解Kasbach-Merritt综合征的临床特点,以减少误诊,并探讨其治疗方案。方法对本院2001年3月～2008年2月诊治的13例Kasabach-Merritt综合征患者的临床资料进行回顾性分析。结果13例患者中男4例,女9例,年龄均<6个月,均表现为不同部位较大的血管瘤,合并有血小板减少及出血症状,3例有弥漫性血管内凝血(DIC)。3例于大剂量糖皮质激素冲击治疗的基础上予支持对症治疗,效果较好。结论Kasabach-Merritt综合征临床少见,发病早,易误诊,DIC发生率高。根据患儿的皮损及实验室检查结果临床特点,采取不同的治疗方案,缓解病情,可为择期手术切除瘤体和局部注射硬化剂治疗赢得时机。     

综合性手术对Kasabach-Merritt现象的疗效分析

【摘要】 目的 分析综合性外科手术治疗Kasabach-Merritt现象（KMP）的疗效。方法 回顾性分析2017年11月至2019年3月在河南省人民医院血管瘤科接受外科治疗的18例KMP患者的临床资料。术前使用糖皮质激素及免疫球蛋白提升患者血小板计数,对糖皮质激素不敏感者术前12 ~ 24 h予单次输注大剂量血小板, 血小板 < 30 × 109/L者按0.3个治疗量/kg输注,血小板 ≥ 30 × 109/L者按0.2个治疗量/kg输注,总量不超过1个治疗量（1个治疗量总液体量为200 ~ 250 ml,约含血小板2.5 × 1011个）。术中尽可能剥离肿瘤组织,对大面积肿瘤术中缝合困难者,使用自体原位植皮术,对术后有功能障碍可能性的患儿请康复师会诊,予个体化功能锻炼,并促进功能恢复。结果 纳入18例患者,男9例,女9例,平均年龄73 d（范围7 ~ 354 d）。皮损位于四肢3例,躯干11例,头面颈部4例。手术成功17例,1例死亡。手术成功的患儿中,16例血小板及凝血功能于术后1周内均恢复至正常,1例患儿术后经规律口服西罗莫司后血小板及凝血功能也恢复正常;术后患儿均能保持良好运动能力。结论 综合性外科手术治疗KMP疗效肯定,起效迅速,不良反应少。