肝脏婴儿型血管内皮瘤二例

肝脏婴儿型血管内皮瘤（infantile hemangioendthelioma，IHE）是较少见的肝脏良性肿瘤，文献报道少见。现就我院收治的2例肝脏IHE结合有关文献进行分析讨论。     

婴儿型肝脏血管内皮细胞瘤并发心力衰竭2 例分析

目的探讨婴儿型肝脏血管内皮细胞瘤(IHHE)合并心力衰竭的临床特征、治疗及预后。方法回顾分析2例婴儿型IHHE合并心力衰竭患儿的临床资料,并复习相关文献。结果例1女性,产前超声示胎儿左腹部不均质回声团,生后第5天经磁共振成像、CT检查确诊,生后7天出现心力衰竭,于出生15天手术切除并经病理检查确诊IHHE,2月龄时复查无异常。例2男性,出生第15天时因新生儿黄疸入院,并很快出现心力衰竭,经磁共振、CT检查临床确诊IHHE。因病情进展迅速,于术前合并失代偿心力衰竭、呼吸衰竭死亡。结论婴儿型IHHE合并心力衰竭者病死率高,早识别、早干预、多学科干预可改善预后。     

罕见婴儿胰头血管内皮瘤干扰素治疗一例

患儿：男，9个月，因全身皮肤黄染，拟肝炎在外院治疗1月余，无好转，CT检查肝外胆管扩张，以阻塞性黄疸收入我科。入院体检：巩膜及全身皮肤重度黄染，腹部膨隆。肝肋下平脐，质中，心肺无特殊。辅助检查，Hb49g／L，细胞压积17．8％，ALT：120U／L。AST：167U／L，r-谷氨酰转肽酶134U／L，总胆红素389．6μmol／L，直接胆红素218μmol／L，总蛋白55g／L，HBS-Ab( )，甲胎蛋白7．2ng／mL，癌胚抗原1．8μg／L，MRI及CT、B超均提示胰头占位，直径约4．5cm，胰母细胞瘤可能。     

小儿肝脏血管内皮细胞瘤组织中VEGF表达的研究

目的：研究血管内皮生长因子（VEGF）、CD34在小儿肝脏血管内皮细胞瘤（IHHE）组织中的表达,探讨VEGF在血管内皮细胞瘤诊断和治疗中的作用。方法14例肝脏血管内皮细胞瘤石蜡标本来自2002年7月至2009年7月北京儿童医院手术切除并经病理诊断的临床病例,术前未行其他治疗,并选取小儿肝脏恶性肿瘤组织（肝母细胞瘤20例）,其他肝脏良性肿瘤组织（结节样增生10例,错构瘤10例）及IHHE瘤旁组织作为对照组;空白对照选取良性肿瘤术中切取部分肝脏组织。组织切片SP法进行免疫组织化学染色,兔抗VEGF和CD34相关抗原标记;计算出VEGF表达阳性率,CD34相关抗原抗体检测出肿瘤间质血管,分析其表达的不同及相互关系。应用统计软件进行数据分析。结果①VEGF在肝母细胞瘤组织中表达率为90.0％（18/20）,在良性肿瘤组中表达阳性率为10.0％（2/20）;VEGF在正常肝组织、瘤旁组织中呈低水平、稳定表达,染色浅,而在IHHE肿瘤组织中则呈阴性表达。在肝母细胞瘤组阳性表达率明显高于其他组,差异有显著性（P＜0.05）,其余两两比较,差异均无显著性（P＞0.05）。②CD34在已知正常肝组织肝窦处未见阳性表达;在IHHE组织中的阳性表达率为100.0％（14/14）,肝母细胞瘤为90.0％（18/20）,肝脏良性肿瘤为15.0％（3/20）,瘤旁组织未见表达。MVD在IHE,肝母细胞瘤组,良性肿瘤组及瘤旁组织分别为31.55±4.86,29.75±5.56,11.23±3.97。结论 IHHE中,高表达CD34,低表达VEGF,表明其不是单纯的血管、内皮细胞病变,很有可能是血管瘤与血管畸形的混合体。IHE增殖期抗血管生成治疗可能促进肿瘤退化。     

卡波西样血管内皮瘤的药物治疗现状和进展

卡波西样血管内皮瘤(kaposiform hemagioendothelioma,KHE)是一种少见的主要发生于婴幼儿和儿童的真性血管性肿瘤,往往呈现局灶性进展,一般不发生远处转移,累及部位主要有头颈部、躯干和四肢,可累及一个或多个部位。与婴儿血管瘤不同,目前国内尚没有针对卡波西样血管内皮瘤的标准化治疗方案,国内外也没有明确的治疗指南。因此,本文就卡波西样血管内皮瘤的诊断、药物治疗共识的现状及新进展进行简要的回顾和总结,希望通过多中心大样本前瞻性研究来制定卡波西样血管内皮瘤药物治疗的指证、指南及共识。     

卡波西样血管内皮瘤的研究进展

卡波西样血管内皮瘤(Kaposiform hemangioendothelioma,KHE)是一种好发于婴幼儿及儿童的、罕见的内皮源性、具有局部侵袭性的脉管肿瘤,表现为弥漫性血管与淋巴管的侵袭性增殖。KHE合并血小板减少、低纤维蛋白血症和凝血因子消耗,称为卡梅现象(Kasabach-Merritt phenomenon,KMP),可致严重并发症甚至死亡。目前,随着大家对KHE发病机制的认识,临床诊疗水平的不断提高,采用手术、动脉栓塞或长春新碱、西罗莫司等药物治疗可使得大多数患儿预后较好;但部分长期合并运动功能障碍与慢性疼痛的患儿,仍是治疗难点。为进一步提高临床医生对KHE的认识,本文拟对其临床特征、主要并发症及危险因素、发病机制和治疗等进行深入阐述,为临床治疗KHE提供经验。     

小儿肝脏血管内皮细胞瘤一例

小儿肝脏血管内皮细胞瘤少见,虽然其组织学上类属良性,但其内在增殖的特点却使其具有潜在恶性的可能,所以应积极治疗,日前本院治疗1例,疗效良好,现报告如下。     

后腹膜卡波西样血管内皮瘤诊疗分析

目的 通过分析后腹膜卡波西样血管内皮瘤的诊治过程,探讨该类疾病的诊断与治疗方法.方法 复旦大学附属儿科医院外科近年来成功治疗后腹膜卡波西洋血管内皮瘤伴血小板减少患儿2例,回顾性分析2例患儿的病史及诊疗经过.结果 1例患儿肿瘤位于后腹膜,表现为后腹膜巨大占位性病变,伴血小板减少,被误诊为神经母细胞瘤.1例表现为顽固血便,血小板减少,被误诊为出血性肠炎.2例患儿诊断过程复杂,经多科会诊得以确诊.在治疗初期激素治疗有效,但迅速出现耐药和病情反复.采用长春新碱（Vincristine,VCR）治疗后病情得到控制,撤离激素,血小板均恢复至正常范围,肿块消失.结论 后腹膜卡波西样血管内皮瘤临床罕见,最常见的并发症是血小板减少.该病诊断困难,容易误诊.VCR治疗有效,部分患儿可完全缓解.规范化和个体化的治疗非常必要.     

小儿阑尾上皮样血管内皮瘤一例

患儿 :女 ,6岁 ,因右下腹痛伴发热2ｄ急诊入院。入院前 2ｄ晨起后脐周腹痛 ,阵发性能忍受 ,腹痛过后不影响活动。当天下午及夜间未诉腹痛 ,但有低热。 1ｄ后诉右下腹痛 ,也不重。入院当日上午因腹痛加重就诊。病后无食欲 ,无恶心呕吐 ,无腹泻便秘。既往健康 ,食欲欠佳。否认腹痛史。Ｔ 37℃ ,Ｐ 96次/ｍｉｎ ,ＢＰ 8/ 12ｋＰａ,体重 16ｋｇ。发育营养欠佳 ,自动体位 ,意识清楚 ,检查合作。无明显贫血貌 ,浅表淋巴结不大。心肺正常。腹部平坦 ,肝脾不大。专科检查 :右下腹明显肌紧张和压痛 ,触之饱满感。肛诊 :直肠内温度不高 ,右上方有触痛 …     

Unusual presentation of infantile hemangioendothelioma

Infantile hemangioendothelioma is a rare benign hepatic tumor arising from mesenchymal tissue. Most of the cases present with congestive heart failure and asymptomatic abdominal mass were before 6 months of age. We described a patient with projectile vomiting, which is quite an unusual presentation. Following surgical resection, neither recurrence nor symptoms were seen in 2 years of follow-up.     

Potential response to curcumin in infantile hemangioendothelioma of the liver

A case of life‐threatening infantile hemangioendothelioma of the liver in a 6‐month‐old infant treated successfully with the dietary supplement curcumin is reported, with 6‐year follow‐up. Implications for pathogenesis based on sites of action of curcumin are considered. Pediatr Blood Cancer. 2010;55:377–379. © 2010 Wiley–Liss, Inc.     

Thyroid abnormalities in infantile hepatic hemangioendothelioma

We report an infant with hepatic hemangioendothelioma (HAE) associated with compensated hypothyroidism. The hepatic lesions regressed with steroid therapy and his thyroid function normalized with high doses of thyroxine supplement.     

Multifocal epithelioid hemangioendothelioma in a 16-year-old boy

We report on a 16-year-old white male presenting with a 4-month history of syncopal episodes and occasional headaches. The CT and MRI studies revealed numerous lytic lesions of the skull base and cervical spine; subsequently, similar lesions were demonstrated in all areas of the skeleton, and CT showed numerous lesions in the lungs, liver, and kidneys. Excisional biopsy from several sites confirmed the diagnosis of epithelioid hemangioendothelioma. We conclude that epithelioid hemangioendothelioma should be added to the differential diagnosis for lytic lesions of bone that are clustered in the same anatomic region and that might also present with visceral involvement.     

Faecal calprotectin levels in infants with infantile colic, healthy infants, children with inflammatory bowel disease, children with recurrent abdominal pain and healthy children

This study investigated faecal calprotectin concentration, a measure of intestinal inflammation, in infants and children with abdominal pain. Faecal calprotectin was measured by an enzyme-linked immunosorbent assay kit in spot stool samples in 76 infants with typical infantile colic, 7 infants with transient lactose intolerance and 27 healthy infants. All infants were 2-10 wk of age. In addition, 19 children with recurrent abdominal pain (RAP; mean age 11.5 y), 17 with inflammatory bowel disease (IBD; mean age 11.1 y; 10 had Crohn's disease and 7 ulcerative colitis) and 24 healthy children (mean age 5.3 y) were studied. In infants with infantile colic the mean faecal calprotectin concentration was not different from that in healthy infants (278 +/- 105 vs 277 +/- 109 mg kg(-1), p = 0.97) or in infants with transient lactose intolerance (300.3 +/- 124 mg kg(-1), p = 0.60). The calprotectin level was similar in boys and girls and fell significantly with age (p = 0.04). Children with IBD had faecal calprotectin levels (293 +/- 218 mg kg(-1)) much higher than healthy children (40 +/- 28 mg kg(-1), p < 0.0001) and children with RAP without identified organic disease (18 +/- 24 mg kg(-1), p < 0.0001). Conclusion: Faecal calprotectin may differentiate between functional abdominal pain and IBD in school-aged children. In young infants high faecal calprotectin levels are normal.