小儿腹部炎性肌纤维母细胞瘤诊治探讨

目的探讨小儿腹部炎性肌纤维母细胞瘤(inflammatory myofibroblastic tumor,IMT)的诊断与治疗方法,以提高其诊治水平。方法分析首都医科大学附属北京儿童医院肿瘤外科2006—2018年收治的18例经手术病理确诊为腹部IMT患儿的临床资料,包括临床表现、实验室检查、影像学检查、病理学诊断、外科治疗方法及随访情况等。18例患儿术前均行超声检查、CT或MRI检查。1例行穿刺及术前化疗,1例仅行开腹活检及术后化疗,其余均接受肉眼下肿瘤全切术,其中2例弥漫性腹部病变行多次手术并接受全身规律化疗。结果术后病理提示肿瘤细胞均呈梭形伴有炎症浸润,部分镜下可见玻璃样变或局灶出血。1例随访中失访,1例肿瘤位于腹膜后间隙者于术后3个月死亡,其余16例随访4~29个月,未见术后严重并发症或死亡发生,1例弥漫腹部IMT病灶患儿带瘤存活。结论IMT可来源于腹部各区域并引发不同症状。影像学检查利于定位和诊断,但缺乏特异性。大部分单发病变可以单纯经手术治愈,对患有弥漫性腹部病变的难治性病例的治疗亟待经验积累及进一步研究。     

小儿胸膜肺母细胞瘤临床特点及病理分析

目的探讨小儿胸膜肺母细胞瘤（pleuro．pulmonaryblastoma,PPB）的临床特点、病理学特征及早期诊治方法。方法对4例反复咳嗽,x线及CT检查发现胸腔内巨大占位性病变患儿的临床特点进行分析,l例放弃治疗,3例手术切除,病理学检查及免疫组化染色确诊为PPB,结合文献进行分析。结果肿瘤组织由原始胚胎小圆细胞及梭形细胞组成,核圆形或卵圆形,部分细胞呈巢状分布,原始细胞胞质空泡状,核深染,可见明显核分裂现象。免疫组化Vimentin（＋）,Desmin（＋）。结论 小儿胸膜肺母细胞瘤是罕见的肺间质恶性肿瘤,临床表现不典型,预后差,病理学检查和免疫组织化学检查有助于诊断,应及早完全手术切除,必要时辅以术后化疗。     

儿童输尿管肿瘤的诊治分析

目的 总结儿童输尿管肿瘤的病理类型、临床表现、诊断、治疗和预后,提高对该病的认识.方法 回顾性分析2011年1月至2015年10月我院收治的5例输尿管肿瘤患儿的临床资料,结合文献总结儿童输尿管肿瘤的病理类型、临床表现、诊断、治疗及预后.5例患儿均为男性,输尿管炎性肌纤维母细胞瘤3例,2例以腹痛就诊,分别为6岁和10岁,肿瘤位于输尿管下段,均行输尿管下段肿物切除+输尿管端端(输尿管膀胱)吻合,1例以尿痛并血尿就诊,年龄1岁9月龄,肿物来源于下段输尿管,经输尿管膀胱连接部长入膀胱,继发膀胱输尿管积水,经输尿管和膀胱联合切除肿物+输尿管膀胱再植术.结果 术后随访5~48个月,未见肿瘤复发及转移.恶性横纹肌样瘤1例6岁,以左下腹痛2周就诊,输尿管肿瘤边界不清与腰大肌肉和后腹膜粘连,切除肿物行输尿管端端吻合,术后ICE方案化疗一疗程后局部复发,放弃治疗,术后233d死亡.尤文/原始神经外胚层瘤1例12岁,以右侧腰痛10d就诊,肿瘤位于输尿管髂血管水平,行输尿管肿瘤切除术+输尿管端端吻合术,术后予CAV+IE化疗12个月,随访56个月,未见转移及复发.结论 输尿管肿瘤在儿童发病率极低,文献曾报道的病理类型包括炎性肌纤维母细胞瘤、恶性横纹肌样瘤、尤文/原始神经外胚层瘤、横纹肌肉瘤,术前无特异方法诊断,手术完整切除肿瘤并重建输尿管是治疗的主要方法,确诊需要结合病理检查,根据病理类型决定是否化疗,治疗后均需要长期随访.     

小儿原发性肠道恶性淋巴瘤13例临床分析

目的探讨小儿小肠恶性淋巴瘤的临床特点、诊断及治疗方案。方法对1996年12月至2009年6月收治的13例小肠恶性淋巴瘤的临床资料进行回顾性分析。结果13例患儿术前均误诊，10例诊断为肠套叠，1例为不全性肠梗阻，1例为腹部包块并严重的腹水，1例为小肠套叠并梗阻性黄疸。均急诊手术治疗，术后病理检查证实12例为B细胞性恶性淋巴瘤，1例为T细胞性恶性淋巴瘤，发病部位：12例在回盲部，1例在空肠及十二指肠和胃部。术后4例拒绝化疗，其中2例失访，2例分别于术后8个月和11个月死亡。9例术后辅以CHOP方案化疗，均获随访，1例大量腹水并腹膜转移患儿在化疗过程中死亡，1例化疗2个疗程后因经济原因放弃治疗，7例患儿能坚持化疗，元复发及转移，随访最短1年，最长6年。结论小儿原发性恶性淋巴瘤以回盲部多见，以B细胞性恶性淋巴瘤为主，临床表现缺乏特异性，给诊断带来困难，早期诊断尤其困难，常因诱发其它疾病而急诊手术。手术中疑为肠道肿瘤时应尽可能完整切除病变肠管，术后辅化疗及综合治疗可提高患儿的生存率。     

婴儿恶性横纹肌样瘤病理特征及临床预后探讨

目的 探讨婴儿恶性横纹肌样瘤(MRT)临床病理特征、诊断及治疗预后.方法 收集我院2009年1月至2014年12月间病理诊断为MRT 5例患儿的临床资料、常规组织切片及免疫组化切片,显微镜下观察及追踪随访.结果 5例患儿就诊年龄1～330 d,平均70 d;男∶女为3∶2;肿瘤发生于肾1例、皮肤软组织4例;临床肿瘤分期Ⅰ期1例,Ⅳ期4例.5例患儿均接受了局部扩大切除及周围淋巴结清扫术,其中发生于颈部和大腿的3例手术时肿瘤细胞已侵及周围组织,脉管内查见转移瘤栓,临床肿瘤分期均处于Ⅳ期,放弃治疗术后5～7 d复发,存活14～28 d,平均21 d;发生于肾的1例手术时肾皮质静脉内见瘤栓,CT提示双肺多发转移结节,临床肿瘤分期为Ⅳ期,化疗5个疗程后CT提示肺转移灶扩大,术后存活180 d;发生于会阴皮肤的患儿1例手术时肿瘤细胞未侵及周围扩大切除的组织,淋巴结内未见转移,脉管内未见瘤栓,临床肿瘤分期为Ⅰ期,术后未化疗,随访3年无复发和转移,生存良好.4例Ⅳ期MRT患儿术后半年内病死率为75％(3/4),1年内病死率为100％.免疫组化肿瘤细胞胞浆强表达Vimentin,灶状表达CK、EMA,不表达INI1.结论 MRT具有进展快、侵袭性强、易复发和转移、预后差的临床特点,具有独特的病理组织学特征,患儿病死率与肿瘤分期直接相关,肿瘤分期越高,术后1年内病死率越高.肿瘤处于Ⅰ期的患儿完整切除有较长的生存期,处于Ⅳ期的患儿即使完整切除后辅以强力的化疗生存期仍很短.     

神经母细胞瘤97例诊断治疗分析

目的讨论小儿神经母细胞瘤(NB)诊断、治疗与预后特点。方法分析1987～1999年间收治小儿NB97例,以尿中3-甲氧基-4-羟基杏仁酸(VMA)、B超、磁共振、骨穿及病理检查确诊。术前确诊患儿化疗12～18天。全部病例肿瘤切除。结果97例NB术后15个月内先后复发来院再次治疗50例,仅2例再次手术切除肿瘤,病理检查已趋向神经节细胞瘤。结论NB已经确定诊断者首次化疗效果明显,其后是手术的良好时机。骨穿和尿VMA检查是诊断NB的较好方法。     

小儿腹部肿瘤的诊治经验的探讨

目的对小儿腹部实体肿块常见类型及肿瘤的早期诊断、外科治疗适应证及手术技巧进行深入讨论。方法总结了1993～2003年十年间我院共收治小儿腹部肿瘤患儿29例，其中22例接受手术，除1例术后死亡，其他21例预后良好。回顾性研究其相关因素。结果22例腹部肿瘤患儿，其中16例肝母细胞瘤，1例术后因凝血功能障碍死亡，2例肾母细胞瘤，3例畸胎瘤，1例神经母细胞瘤。术后恢复良好。肾母细胞瘤术后均行化疗。术后患儿未发生腹腔大出血及邻近器官损伤。结论小儿腹部肿瘤要早期诊断、及时治疗，术前要认真检查及分析，根据病理分型及患儿耐受能力进行合理的个体治疗方案。     

小儿胰腺肿瘤的诊断与治疗(附15例报告)

目的 探讨小儿胰腺肿瘤的诊断和治疗.方法 回顾性分析2002年1月至2010年1月收治的15例胰腺肿瘤,男8例,女7例;年龄45 d至17岁(平均11.3岁).临床表现:腹痛9例,2例合并黄疸;腹部包块6例.B超和CT检查均为胰腺占位性病变.手术方式:腹腔镜胰腺肿瘤切除术6例;胰腺体尾部切除术4例;肿瘤剔除术4例;Whipple术1例.结果 本组患儿无围手术期死亡.病理:胰腺实性乳头状瘤8例,胰腺母细胞瘤4例,胰腺血管瘤1例,胰腺无功能性内分泌癌1例,胰腺癌1例.术后随访1年至8年,患儿全部存活.14例未见复发和转移,1例胰腺癌化疗后2年肝转移,行转移瘤局部切除术后治愈.结论 小儿胰腺肿瘤罕见,临床多见胰腺实性乳头状瘤和胰腺母细胞瘤.腹腔镜和开腹根治性手术均可获得良好的治疗效果.胰腺癌根治性手术后易复发,需长期随访.     

儿童胰母细胞瘤诊治分析

目的 儿童胰母细胞瘤是一种罕见的小儿恶性肿瘤,本文拟通过分析本院近年来收治的儿童胰母细胞瘤病例,探讨其诊断与治疗要点.方法 回顾性分析本院2002～2006年收治的3例儿童胰母细胞瘤患儿临床资料.结果 3例中,除1例因瘤体巨大出现腹痛、呕吐等非特异性症状,就诊发现包块外,其余两例均因偶然原因体检发现腹部包块.人院体查均可扪及左腹部包块,质硬,不活动.CT检查均提示为左腹膜后巨大实性占位性病变,血清AFP值均显著增高.术中发现,3例患儿肿瘤均位于胰尾部,边界清楚,包膜完整,手术完整切除肿瘤.术后病理检查证实为胰母细胞瘤.本组均手术完整切除肿瘤且未查见远处转移,3例患儿术后均未行化疗.所有患儿均生存,随访期间无复发,复查AFP均无异常升高.结论 儿童胰母细胞瘤多无特异性症状、体征及影像学表现,早期发现和诊断非常困难.血清AFP值可作为瘤标用于诊断与随访.儿童胰母细胞瘤往往呈膨胀性生长,有完整包膜,预后相对较好,应积极治疗.在肿瘤发生远处转移之前,早期诊断并手术切除肿瘤是治愈该病的唯一途径.若能一期彻底切除肿瘤,术后可无需化疗.     

儿童脊柱肿瘤

目的 探讨儿童脊柱肿瘤的临床特点、诊断、治疗方法及预后。方法回顾性分析我院1991年1月-2003年10月治疗的16岁以下脊柱肿瘤及瘤样病变44例，男27例，女17例；平均9．6岁。良性肿瘤（包括瘤样病变）38例，恶性肿瘤3例，转移瘤3例。疼痛39例，肿块9例，侧弯或斜颈8例，不同程度神经损害14例。18例初诊不确定或与最后诊断不符。发病至确诊时间平均8．5个月。手术治疗17例，化疗10例，放疗8例，手术与化疗1例，手术与放疗2例，放弃治疗3例，观察3例。结果围手术并发症4例。35例获平均5．5年随访。良性肿瘤及瘤样病变患儿手术或放疗或化疗后疗效较满意，所有患儿的局部疼痛消失，除1例外，所有患儿神经功能均有改善，3例病变复发再次手术。4例出现后弯畸形。6例恶性肿瘤和转移瘤患儿全部死亡。2例嗜酸性肉芽肿患儿症状消失，病变得到控制。结论对于儿童脊柱肿瘤，根据肿瘤的性质选择恰当的治疗方法，良性肿瘤与肿瘤样病变预后良好，恶性肿瘤及转移瘤治疗效果欠佳。     

Successful treatment of inflammatory myofibroblastic tumor with malignant transformation by surgical resection and chemotherapy

Inflammatory myofibroblastic tumor (IMT) is a tumor composed of myofibroblasts and a mixed inflammatory infiltrate that rarely undergoes malignant transformation. The authors present the case of a 7-year-old boy with an abdominal mass diagnosed as IMT with malignant transformation. The tumor recurred twice after attempts at resection and was initially treated with vincristine and etoposide. After a third recurrence and incomplete resection, he was treated with cisplatin, Adriamycin, and methotrexate. He is disease-free after 2 years, representing successful combined surgery and chemotherapy in the treatment of malignant IMT. The use of chemotherapy for aggressive myofibroblastic tumors is reviewed.     

Pediatric inflammatory myofibroblastic tumor of the bladder with ALK–FN1 fusion successfully treated by alectinib

An inflammatory myofibroblastic tumor (IMT) is a mesenchymal neoplasm characterized by the proliferation of myofibroblasts and inflammatory cell infiltration. Although radical resection is the only established treatment strategy for IMT, it can cause functional disorders when vital organs are affected. We describe a case of pediatric IMT of the bladder with FN1–ALK (fibronectin 1–anaplastic lymphoma kinase) fusion. Radical resection might lead to urinary disturbance due to the large tumor size at diagnosis. However, the tumor was successfully treated with alectinib, a second-generation ALK inhibitor, followed by transurethral resection of the bladder tumor without any complications.     

Pulmonary inflammatory myofibroblastic tumor after Hodgkin’s lymphoma and application of PET imaging

Inflammatory myofibroblastic tumor (IMT) is a rare tumor with a particular histological pattern of myofibroblasts and mixed inflammatory infiltrate. IMT has been rarely described in association with malignancy. This case report is of a 16-year-old male who had Hodgkin's disease (stage IVA) and who after chemotherapy and radiotherapy developed IMT, 16 months post completion of therapy. The IMT was in the lung in an area which was previously involved with HD and had undergone radiotherapy. PET imaging with F(18)FDG was used in the initial diagnosis and has been used in follow-up after full surgical resection of the lesion.     

Pediatric inflammatory myofibroblastic tumor: anaplastic lymphoma kinase (ALK) expression and prognosis

BACKGROUND: Pediatric inflammatory myofibroblastic tumor (IMT) is rare, with unpredictable clinical behavior. Recently, it has been associated with anaplastic lymphoma kinase (ALK) expression. METHODS: Patients under age 16, treated for IMT between 1976 and 2000 were reviewed. Mean follow-up was 8 years (range 1 month-22 years). RESULTS: Eight children had IMT, with a mean age of 6 years (range, 11 months-14 years) and female to male ratio of 3:1. Tumor location was lung (four patients), abdomen (two patients), lung and abdomen (one patient), and abdomen, head, and neck (one patient). Presenting symptoms included anemia (seven patients), fever (six patients), and dyspnea (four patients). Laboratory results included thrombocytosis (six patients), hypergammaglobulinemia (four patients), elevated sedimentation rate (four patients), and leukocytosis (three patients). Immunohistochemistry revealed ALK expression in four of eight tumors. Four children had complete resection and are alive. Two of these children had ALK-positive tumors. Four patients had incomplete resection, and two had recurrences treated successfully with resection and radiotherapy; the other two died of disease. For the incomplete resection patients, those that were ALK-positive lived, and those that were ALK-negative did not. CONCLUSIONS: Eight children were treated for IMT over a 15-year period. ALK expression was found in half the tumors. Prognosis was improved with ALK expression and complete surgical resection.     

Inflammatory myofibroblastic tumor of the lung: a rare cause of atelectasis in children

Although rare, inflammatory myofibroblastic tumor is the most common primary lung mass in children. We report the case of an 11-year-old boy investigated for persistent cough and dyspnea with complete left lung atelectasis mimicking pneumonia. CT and MRI showed an endobronchial mass of the left main bronchus. The boy underwent endoscopic resection of the tumor and histology was in favor of an inflammatory myofibroblastic tumor of the lung. This diagnosis should be suspected in children with recurrent pneumonia. The prognosis is good after complete resection.     

经导管动脉化疗栓塞在肝母细胞瘤综合治疗中的应用评价及文献回顾

目的：探讨经导管动脉化疗栓塞术（TACE ）在肝母细胞瘤综合治疗中的作用及疗效。方法回顾性总结2005年-2013年收治的10例经TACE 治疗的肝母细胞瘤患儿的临床资料。随访治疗后患儿的全身情况、血AFP值及瘤体体积大小的变化及生存情况。结果患儿10例,男女比例4∶1,发病中位年龄9.5个月（1个月～10岁）,10例患儿首诊后均予以1～4次TACE 治疗,其中6例TACE＋手术＋化疗,1例TACE＋化疗,3例在TACE 治疗后放弃继续治疗。介入治疗后肿瘤体积较前均有明显缩减（26.2％～10.00％,平均70.0％）,血清AFP值下降明显（28.5％～99.7％,平均83.6％）。所有患儿在接受TACE 治疗后均出现不同程度的发热、呕吐、一过性肝功能损害（Ⅰ度3例,Ⅱ度2例,Ⅲ度1例）、轻度骨髓抑制、贫血等不适,未见明显心脏毒性及肾毒性损害。随访时间2～114个月,平均随访时间为36.1个月,1年存活率100％（7／7）,2年存活率86％（6／7）,3年存活率71％（5／7）。6例患儿在接受TACE治疗后成功行手术切除,术后均接受全身化疗,1例仅经过4次TACE治疗及化疗后肿瘤消失,未行手术治疗,均无瘤存活至今。3例放弃治疗患儿分别于2～8个月后死亡。结论 TACE治疗可作为肝母细胞瘤术前重要的辅助治疗方式,能够使肿瘤体积明显缩小,血供减少,促进肿瘤包膜增厚,为尽可能完整切除创造了条件,能够改善肝母细胞瘤患儿的生活质量,提高长期存活率。     

Combined liver transplant and pancreaticoduodenectomy for inflammatory hilar myofibroblastic tumor: Case report and review of the literature

IMT, previously known as IPT, is a relatively rare tumor that was originally described in the lungs, but case reports have reported the tumor in almost every organ system. Surgical resection is typically the mainstay of therapy; however, tumors have also been shown to respond to chemotherapy or anti‐inflammatory therapy and some have spontaneously regressed. We present a literature review and case report representing the first documentation to date of liver transplant combined with PD for surgical resection of a myofibroblastic tumor non‐responsive to medical therapy.     

Inflammatory myofibroblastic tumor of the bladder in children: what can be expected?

Inflammatory myofibroblastic tumor of the bladder is an uncommon condition of unknown neoplastic potential. In adults the tumor is seen in association with instrumentation of the lower genitourinary tract, while in children it appears to run an idiopathic course. Its clinical and radiological presentation in children resembles sarcoma. The case of a 10-year-old girl with inflammatory myofibroblastic tumor is presented, outlining the histological and immunhistochemical features to allow differentiation between sarcomas, the most important differential diagnosis. An outcome meta-analysis of the literature identified 35 cases of inflammatory myofibroblastic tumor in the bladder of children. Conservative surgery is the strategy of choice. There is no evidence of recurrence or metastasis at a median follow up of 1.5 years.