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 …     

小儿直肠上皮样血管内皮瘤1例报告

1临床资料1.1一般资料患儿,女,7岁。因排便不畅20余天入院,20d前患儿出现频繁便意感,排便量少,肛门常伴有稀便溢出。无呕吐、无腹痛、无发热,排便时肛门口见一肿物坠出,拟诊直肠肿物收入院。既往体健,无特殊家族史,入院体查:营养发育中等,轻度贫血貌,心肺无异常体征。腹平软,左下腹触及一约6cm×5cm肿物,可活动,无压痛,肛门指检:肛周皮肤红、潮湿,直肠内触及一圆形肿物,光滑,可推动、轻压痛,指套有血染。B超检查:膀胱后壁后方探及一实质性等回声肿物,界清有包膜回声、大小约7cm×5cm,子宫附件检查不清。1.2临床诊断直肠肿物性质待查1.3手术…     

会阴部上皮样血管肉瘤一例

患儿 :男 ,11岁。 1999年 1月发现会阴左侧有一痛性肿物 ,约成人拇指大小 ,触痛明显 ,局部皮肤无红肿 溃烂。曾在外院治疗无效 ,5月 2 1日就诊我院。体检 :会阴左侧有一痛性肿物 ,约3.0ｃｍ× 3.0ｃｍ ,边界不清 ,质硬 ,表面光滑 ,较固定 ,压痛明显。Ｂ超显示 :会阴左侧探及 2 .0ｃｍ× 1.7ｃｍ低回声团 ,团块内有丰富血流信号。骨盆Ｘ线片显示 :骨及关节未见异常。 5月 2 7日手术所见 :会阴左侧肿物约 4 .0ｃｍ×4 .0ｃｍ× 3.0ｃｍ ,位于肌肉组织内 ,边界欠清 ,固定 ,质脆易烂 ,混有血凝块。术后病理 :中间型血管内皮瘤。术后未行放疗和…     

左心房内血管周上皮样细胞分化肿瘤一例

患儿女,2岁,因"睡眠差10 d,精神差伴气促5 d,加重1 d"于2009年3月18日入院.入院前10 d无明显诱因出现夜间烦吵,近5 d精神差,伴气促,不喜活动,无口周青紫,入院前1 d始纳差,不喜下地活动,间有轻咳,双下肢轻度水肿,尿量减少,至当地医院给予"多巴胺"、"多巴酚丁胺"、"西地兰"等治疗后症状稍改善,行超声心动图示"左房占位性病变:黏液瘤?"急诊转入我科.     

儿童胸腺淋巴上皮瘤样癌1例报道并文献复习

目的加强对儿童胸腺淋巴上皮瘤样癌(LELC)的认识。方法对1例诊断为儿重胸腺LELC患儿临床资料进行回顾性分析,并结合文献报道的10例儿童LELC进行分析。结果惠儿,12岁,男,表现为夜间睡眠不宁、胸闷;外周血白细胞升高,胸部CT示右中上纵膈可见类圆形软组织密度影;手术切除大部分肿物,病理报告为胸腺LELC;化疗后临床症状消失,6个月后原位复发。近20年来文献报道胸腺LELC患儿共10例,其中男6例,女4例。起病症状为胸痛、呼吸困难者6例,肥大性骨关节病(HOA)5例,证实EBV感染6例;8例在发病1年左右死亡。结论胸腺LELC是儿童时期罕见的恶性肿瘤,常合并EBV感染,存在免疫功能紊乱,临床特点为咳嗽、胸闷、胸腔巨大占位和HOA,手术难以完整切除,恶性程度高,预后极差。     

肺上皮样血管内皮细胞瘤一例

患儿男,12岁,主因咳嗽2个月,加重15d入院。患儿于入院前2个月开始咳嗽,咯白色黏痰,量不多,伴乏力,无发热、咯血、喘息等伴随症状,间断应用止咳药物治疗。入院前15d咳嗽加重,自觉呼吸不顺畅,胸部X线片提示左肺大部分不张。收入院后发现,患儿有短暂低热,咳嗽为声咳,声音嘹亮、高亢,呈金属声。     

CT,magnetic resonance imaging and clinicopathological characteristics of perivascular epithelioid cell tumor in children北大核心CSCD

目的评价儿童血管周上皮样细胞瘤(PEComa)的CT、磁共振成像(MRI)和临床病理特征,以提高对该肿瘤的诊断水平。方法回顾性分析2019年3月至2022年4月首都医科大学附属北京儿童医院经病理确诊的6例儿童PEComa的CT、MRI和临床病理特征。结果患儿年龄1.7~11.9岁(平均7.6岁)。男女比例为1∶2。6例单发肿瘤发病部位分别见于肾脏(2例)、腹腔(2例)、盆腔(1例)和腹股沟(1例)。肿瘤边界清晰,呈类椭圆形(5例)或多结节样(1例),边缘见花边样或结节样改变(3例)。肿瘤长径4.8~15.9 cm(平均9.3 cm)。5例肿瘤表现出周围侵犯,2例存在淋巴结转移。2例患儿合并结节性硬化症。1例肿瘤为术后复发。CT平扫(6例)示肿瘤呈等/低密度,增强后呈不均匀渐进性强化。多数(5例)肿瘤见瘤内迂曲增粗血管影,少数(2例)见沙砾样钙化。肿瘤中心在MRI(3例)T1WI及T2WI上呈斑片样或结节样高信号,周边呈等肌肉信号,弥散加权成像(DWI)(b=800 s/mm2)上病灶周边部分弥散受限且增强后强化明显,中心部分呈等信号,增强后不强化或仅轻微强化。1例组织学分类为良性PEComa,5例为恶性PEComa。Melan-A和SMA的表达率分别为100.0%、83.3%。结论儿童PEComa的CT、MRI和病理表现存在一些特异性,常表现为腹盆腔内边界清楚的类椭圆形肿块,伴结节样边缘,可见瘤内迂曲增粗血管,增强后以周边强化为主,可有局部侵犯和远处转移,病理上以恶性病变居多,免疫组织化学上呈黑色素-肌肉双源性表达。     

卡波西样血管内皮瘤伴卡梅综合征1例报告与文献复习

目的 了解卡波西样血管内皮瘤（Kaposiformhemangio-endothelioma,KHE）伴卡梅综合征（Kasabach-Merritt syndrome,KMS）的诊断、临床特点及治疗方法.方法 分析采用甲强龙＋长春新碱（VCR）＋环磷酰胺（CTX）治疗1例2012年2月我院收治的经病理诊断KHE伴KMS的临床疗效,并复习相关文献.结果 （1）化疗方案采用甲强龙＋ VCR＋ CTX.2周血小板恢复正常.观察至2012年6月患儿血小板及凝血功能恢复正常,左侧肩关节活动改善,复查肩部CT提示肿瘤明显缩小.（2）化疗后患儿出现双上眼睑下垂,经营养神经治疗后恢复正常.长春新碱毒副作用可逆.结论 KHE是一种临床罕见,但以儿童和婴幼儿为主要发病年龄的低度恶性血管源性肿瘤,临床上常伴有KMS.本病例提示对于难以手术治疗的患儿可给予激素及免疫抑制剂的联合治疗,能改善KMS并肿瘤缩小.     

儿童母细胞性浆细胞样树突细胞肿瘤5例病例系列报告

目的 分析儿童母细胞性浆细胞样树突细胞肿瘤(BPDCN)的临床特征、病理表现、治疗和预后。方法 纳入2014年1月1日至2019年1月31日北京大学人民医院收治的5例BPDCN患儿,截取患儿的一般资料、起病年龄、确诊年龄、临床表现、辅助检查结果、治疗情况和随访资料。结果 5例BPDCN患儿纳入本文分析,男2例,女3例,诊断年龄8~14岁。中位随访时间37(15~51)月。5例患儿起病时均有皮肤受累,皮肤外受累器官包括肝脾、淋巴结、椎体、外周血,未发现中枢神经系统受累。5例患儿均经皮肤或骨髓病理检查确诊,均表达CD4、CD56和CD123,均无特异性基因表达,1例染色体核型检测异常。诱导化疗采用ALL或NHL方案,均诱导缓解,2例NHL方案巩固化疗中出现局灶复发。5例患儿后续均行异基因造血干细胞移植,目前无病存活4例,1例为治疗相关死亡,5例患儿均无疾病相关死亡。结论 儿童BPDCN临床侵袭性弱于成人,诱导化疗敏感,后续行异基因造血干细胞移植预后较好。     

儿童腹膜后肿瘤累及重要血管的手术处理

目的总结儿童常见腹膜后肿瘤累及重要血管时的手术处理经验。方法选择47例儿童腹膜后肿瘤,其中包括进展期神经母细胞瘤22例,神经节细胞瘤6例,肾母细胞瘤19例,应用“血管骨骼化”的方法解剖血管,并对受累血管采用多种方法处理,最终完成一期手术切除。结果儿童常见腹膜后肿瘤如神经母细胞瘤、肾母细胞瘤、神经节细胞瘤在血管骨骼化理念的指导下,一期手术完全切除率分别达95．45％、100％及100％。除1例术后早期发生急性。肾功能衰竭经透析治疗痊愈外,其余均顺利恢复,无围手术期死亡病例。结论儿童常见腹膜后肿瘤通过骨骼化血管及其它相应方法处理后,能提高一期手术完全切除率。     

Perivascular Epithelioid Cell Tumor of the Orbit: A Case Report and Review of the Literature

We present a unique case of a perivascular epithelioid cell tumor (PEComa) in the orbit of a 9-year-old female patient. The entity of PEComas has been described only recently. Characteristic histologic features and an immunohistochemical profile of negativity for epithelial markers and positivity for melanogenesis-related markers define the tumors. In children and young adults, this tumor has a predilection for the falciform ligament and ligamentum teres of the liver. It is associated with, but not exclusive to, tuberous sclerosis. To the best of our knowledge, this is the first reported case of a PEComa of the orbit in a child or adult. The main differential diagnoses for this melanin pigment-producing lesion include melanoma and pigmented paraganglioma. The histologic features, immunohistochemical profile, ultrastructural studies, and molecular studies led us to favor a diagnosis of PEComa. The prognosis of this entity is undetermined due largely to the small number of reported cases.     

润滑油中毒1例

患儿，男，3岁，因误服润滑油5h，伴昏睡4h入院。患儿于入院前5h误服润滑油约200mL，1h后出现恶心、呕吐，共2次呕吐胃内容物，量多，有机油味，呕吐为非喷射性，且出现全身冷汗，随后患儿出现昏睡状，面色苍白，呼气有润滑油味，无惊厥，无腹泻，5h无尿。体检：体温37．1℃，心率150次／min，呼吸60次／min，血压12／8kPa，体质量12kg，面色苍白，急性重病容，呈昏睡状态，语言不清，神志模糊，查体不合作，皮肤苍白，双侧瞳孔等大等圆，对光反射灵敏。口唇发绀，呼吸急促，鼻翼扇动，呼气有机油味，吸气时三凹征明显，双肺未闻及干湿性哕音。心音有力，律齐，未闻及杂音，     

脂肪母细胞瘤1例

脂肪母细胞瘤临床较为少见,主要发生在儿童,文献以个案报告为主。国内报告甚少,最近本院收治1例。现报告如下。1临床资料患儿,女,2岁3个月,因腹部无痛性包块进行性增大1 a余入院。体检:腹膨隆,右中腹可扪及16 cm×9 cm包块,质中,边界清楚,活动度差,无压痛及反跳痛。CT:右     

阵发性睡眠性血红蛋白尿1例

患儿，女，12岁，以间断面色苍白4年余，发热伴血尿5d为主诉入院。患儿4年前因面色苍白，皮肤散在出血点0．5个月在当地儿童医院骨髓检查诊断为：再生障碍性贫血（AA），按AA口服药物治疗至今，病情无好转。病初及治疗期间多次血常规检查网织红细胞计数均为0．02～0．03，未曾复查骨髓像，亦未作其他相关检查。     

Treadmill Training in a Child with Cerebral Palsy: A Case Report

This case report describes the use of treadmill training without body weight support to improve walking speed in a child with diplegic cerebral palsy. The child was a six-year-old girl with spastic diplegic cerebral palsy. She walked short distances independently using a posterior support walker but was unable to keep up with her peers walking long distances. Speed of walking 50 feet and speed of walking from her classroom to the cafeteria (458 feet) were timed pretreadmill, posttreadmill, and 3 weeks following treadmill intervention. The GMFM-88 was administered pre- and posttreadmill intervention. Following treadmill intervention, the child's speed of walking increased and she was able to walk to the cafeteria daily with her peers. Improvements in the percent of maximum total score of the GMFM-88 were also observed. The outcomes of this case suggest that the use of short-term, task-specific treadmill intervention might increase a child's walking speed enough to increase their participation in the school setting.     

A Case Report of Paroxysmal Nocturnal Hemoglobinuria in a Child

A ten-year-old boy with paroxysmal nocturnal hemogulobinuria (PNH) is described. Although hemolytic anemia was evident, Ham's test was negative and the erythorocyte acetylcholinesterase (ACHE) activity was normal at the first admission. The diagnosis of hemolytic anemia of unknown etiology was made. Methylprednisolone pulse therapy was started, but could not prevent the hemolytic crises associated with infections. Twelve months later Ham's test turned positive and ACHE activity decreased. The diagnosis of PNH was confirmed. As the disease is insidious in onset, we emphasize that a high index of suspicion and Ham's test and sucrose water test repeated at regular intervals are required to avoid missing the diagnosis.     

先天性食管狭窄并食管炎1例

患儿,男,4个月,因咳嗽、呛奶8d入院。8d前无明显诱因出现咳嗽,伴发热,呛奶严重。当地医院予抗炎、止咳化痰、静脉营养液等综合治疗7d,效果欠佳,下胃管不能而转入本院。查体:神志清,发育营养欠佳,双肺可闻及大量中小水泡音,心、腹及神经系统检查未见异常。辅助检查:心脏及大血管     

Non-Hodgkin Lymphoma in a Child Presenting with Acute Paraplegia: A Case Report

Spinal cord compression is a rare presentation of non-Hodgkin lymphoma. Extradural location at onset is a rare but devastating event in pediatric oncology. The authors describe a girl with acute spinal cord compression due to epidural non-Hodgkin lymphoma, emphasizing the encouraging perspective for a complete recovery in children with this condition. A 5-year-old girl presented with pain followed by progressive hyposthenia and paraplegia after a trauma. CT scan and MRI showed homogeneous tissue extending from T2 to L4, occupying the entire vertebral canal and extending to the para- and peri-vertebral soft parts. Emergency surgical debulking was carried out through T6-L1 laminectomy. The patient began chemotherapy (LMB 89 Protocol) and the tumor quickly disappeared. The patient is maintaining a complete remission 42 months after diagnosis. Significant results may be obtained with the chemotherapy treatment of epidural non-Hodgkin lymphoma when the disease is promptly diagnosed. Considering the effectiveness of chemotherapy, the authors believe that a neuro-surgical approach should be employed only when rapid worsening of symptoms is observed or for diagnostic purpose.     

Squamous Cell Carcinoma of the Tongue in a Child with Fanconi Anemia: A Case Report and Review of the Literature

This report documents a case of squamous cell carcinoma (SCC) of the tongue in a child with Fanconi anemia (FA). FA is an autosomal recessive syndrome defined by chromosomal breakage in response to diepoxybutane or mitomycin C in which many patients present with pancytopenia, hypoplastic bone marrow, hyperpigmentation of the skin, skeletal malformations, small stature, hypogonadism, and chromosomal aberrations. Such patients are prone to the development of hematological malignancies and squamous cell carcinoma, especially of the head and neck. Although FA appears to be genetically heterogeneous, all cases display abnormalities of DNA repair. A gene defective in one of the four subsets of FA patients has been defined. Defects in this gene are thought to play a role in the development of neoplasia in FA patients. However, many other factors may also contribute to the development of malignancies, including immune deficiencies, therapeutic strategies, and bone marrow transplantation. This report reviews the association of FA and SCC and highlights the many factors involved in the development of neoplasia within a single patient, including FA, cyclophosphamide, immunosuppression, X-irradiation, and chronic oral graft-versus-host disease. In addition, the human papillomavirus status, although negative, is documented for the first time in such a case.