胸膜肺母细胞瘤1例报告

目的：探讨胸膜肺母细胞瘤的早期诊断及治疗。方法回顾性分析1例由1型胸膜肺母细胞瘤逐渐进展为3型患儿的诊治过程。结果患儿，男，生后18 d胸片未见明显异常；1岁时胸片示左肺近肺门处囊性占位，约5 cm×4 cm×4 cm，考虑为先天性肺囊性腺瘤样畸形，家长拒绝手术；3岁2个月，胸片及胸部CT提示左肺囊性实性占位，体积较前明显增大，背侧为实性，上部位巨大含气空腔；2个月后患儿出现呼吸喘憋，复查胸部CT示肿瘤完全实变，考虑胸膜肺母细胞瘤3型。手术切除后常规化疗，随访1年尚未复发。结论1型胸膜肺母细胞瘤与先天性肺囊性腺瘤样畸形不宜鉴别，早期诊断及治疗可改善1型胸膜肺母细胞瘤的预后。     

胸膜肺母细胞瘤1例

1　临床资料　　患儿,男,4月,因低热,气促4月加重并咳嗽2月住院,患儿系第一胎足月顺产,自出生后每于啼哭时出现气促,经常有不规则低热,近2月安静时也出现气促,并逐渐加重,无发绀,有阵发性干咳,曾在当地医院行胸片、Ｂ超检查,诊断为“包裹性胸腔积液”,治疗无效,转来我院。体查:Ｔ36.5℃,Ｒ60次/ｍｉｎ,Ｐ132次/ｍｉｎ。发育正常,体质消瘦,反应正常,呼吸急促,无发绀,气管偏左,胸廓无隆起,右肺语颤减弱,叩诊实音,右中下肺呼吸音低,肝脾未扪及。实验室检查:血象ＷＢＣ12.7×109/Ｌ,Ｌ0.43%,Ｎ0.50%,Ｍ0.07%,ＲＢＣ34.5×1012/Ｌ,Ｈｂ11ｇ/Ｌ。…     

小儿胸膜肺母细胞瘤1例报告并文献复习

<正>胸膜肺母细胞瘤(pleuropulmonary blastoma,PPB)属于临床上罕见的与发育不良有关的恶性肿瘤,好发于12岁以下的儿童和婴幼儿,偶尔也可发生于成人。常累及胸膜和肺,出现症状较晚,当瘤体迅速膨大时患儿可出现呼吸困难、咳嗽、咯血或胸痛     

儿童胸膜肺母细胞瘤38例诊治分析

目的探讨儿童胸膜肺母细胞瘤的发病年龄、临床表现、病理分型及特点、治疗原则及预后情况。方法回顾性研究自2002年3月至2018年3月期间首都医科大学附属北京儿童医院收治的38例胸膜肺母细胞瘤患儿临床资料,并进行统计描述。结果38例中男18例,女20例;首诊诊断为非肿瘤性病变者占比47.4%(18/38),从出现症状到手术或化疗干预的时间范围为5~210 d;Ⅰ型4例(10.5%),中位诊断年龄22个月;Ⅱ型12例(31.6%),中位诊断年龄37个月;Ⅲ型22例(57.9%),中位诊断年龄39.5个月。治疗方式包括手术及化疗。术中见肿瘤最大直径为2~18 cm,手术时间25~210 min,术中出血量2~500 mL(1例出血500 mL病例于术中行胸膜剥脱术);化疗方案多采用IVADo(异环磷酰胺+阿霉素+长春新碱+放线菌素D)和IVA(异环磷酰胺+长春新碱+放线菌素)方案,5年无瘤生存率为69.2%。结论胸膜肺母细胞瘤是一种较为罕见的儿童恶性肿瘤,临床表现缺乏特异性,误诊率高,预后较差,需要手术与化疗联合进行治疗。儿科医生应该充分认识到PPB的严重性,对于出现气胸、多发部位肺囊性病变、肺囊肿家族史、PPB家族史、DICER1家族性综合征的胸腔占位性病变患儿应警惕本病的可能,并做到严密监测及随访。     

儿童胸膜肺母细胞瘤临床分析

目的 通过1例儿童胸膜肺母细胞瘤(PPB)的诊治经过,结合相关文献复习,加强对该病的认识。方法 对1例诊断为PPB I型的患儿临床资料进行分析,并结合文献报道的19例儿童PPB I型进行分析。结果 患儿,女婴,9个月,首发表现为咳嗽伴喘息,胸部CT示右肺囊性占位病变,手术完整切除肿瘤,病理报告为胸膜肺母细胞瘤(Ⅰ型)。术后化疗4疗程后获得完全缓解,随访12个月,患儿无病生存。近10年国内文献报道PPB I型患儿19例,左右肺均有发病,临床症状基本为咳嗽、气促、气喘、发热、胸痛等,其中1例有家族史,4例手术切除后接受化疗。结论 PPB是儿童时期罕见的发生于肺部的恶性肿瘤,一般症状出现较晚,以呼吸道症状为主,预后与病理类型、肿瘤能否完全切除、是否转移有关。     

儿童胸膜肺母细胞瘤三例并文献复习

目的：探讨儿童胸膜肺母细胞瘤( pleuropulmonary blastoma,PPB)的误诊原因及早期诊断。方法回顾性分析我院2007年6月至2011年12月收治的3例儿童PPB病例,对发病原因、临床表现、胸部CT及增强CT特点、病理分型、治疗效果等进行分析,总结漏诊原因并评价其治疗效果。结果2例患儿术前误诊,例1误诊为胸腔积液,例2误诊为右侧膈膨升。术后依靠病理诊断PPB,其中例1术后2周即出现胸腔内复发,术后1年半死亡。例2化疗后1年出现对侧胸壁转移瘤,后放弃治疗,术后2年死亡。例3术前即诊断,术前术后化疗,术后2年至今尚无瘤生存。结论 PPB是一种临床少见的极易误诊的高度恶性肿瘤,提高Ⅰ型PPB的诊断率是提高该病治疗效果的关键,术前进行增强CT检查有助于早期诊断,晚期配合术前化疗能提高患儿的治疗效果。     

儿童胸膜肺母细胞瘤的诊治研究进展

胸膜肺母细胞瘤(PPB)是一种罕见且侵袭性极强的恶性间叶性肿瘤,来源于胸膜或肺。早期PPB无特异性临床及影像学表现,易与肺部占位性病变相混淆,给PPB早期诊治带来很大困难。因此,本文就PPB的诊治研究进展展开综述。     

中国儿童胸膜肺母细胞瘤诊疗建议

正一、前言胸膜肺母细胞瘤(pleuropulmonary blastoma,PPB)是一种罕见的、发生于儿童期肺部的恶性肿瘤。临床和病理均有其独特表现,肿瘤主要位于胸腔,多位于肺的周边,甚至胸膜,常伴有胸腔积液。目前认为儿童PPB是家族癌征综合症的表现之一,约20%患儿具有遗传性肿瘤易感倾向,部分患儿存     

异戊酸血症1例报告

1 临床资料 患儿,女,4 d.因反应差伴拒乳1 d入院.患儿系G_3P_3,孕39周足月自然娩出.生后哭声响亮,Apgar评分1 min、5min、10 min均为10分.无胎膜早破,脐带、胎盘正常.生后24 h开奶,母乳喂养,吸吮有力.生后第3天患儿出现反应差,拒乳,少哭少动,门诊以拒乳原因待查收入院.     

Neonatal pulmonary blastoma

A thoracic mass was identified on a routine ultrasound (US) examination in the 30th week of gestation. A postnatal chest radiograph, US, and computed tomography demonstrated a well-delineated, solid, cystic tumor in the left lower lobe. The mass was excised and a diagnosis of pulmonary blastoma (PB) was made on pathologic examination. The patient is alive and free of disease 8 years after the operation. PB is a rare primary neoplasm of the lung, adults being more frequently affected than children. This case appears to be the third neonatal PB reported and one of the few long-term survivors.     

Biphasic pulmonary blastoma in a child

Pulmonary blastoma (PB) is a rare malignant pulmonary tumor composed of immature mesenchyme and/or epithelium that resembles an embryonic lung at 10-16 weeks gestation. PBs constitute only 0.25 to 0.5 percent of all primary malignant lung tumors. Approximately 20 percent of the reported cases have occurred in pediatric patients. A seven-year-old girl presented with fever, cough, respiratory distress and chest pain on the left side. An x-ray, ultrasonography and a computed tomographic scan of the chest showed a large mass consisting of solid and cystic components almost completely occupying the left hemithorax associated with pleural effusion. The diagnosis of biphasic PB was established by histological examination of thoracotomy material. The patient was considered inoperable due to tumor involvement of the mediastinum, and she died two days after the initiation of chemotherapy. We report this case of PB to raise attention to the clinical, radiological and pathological features of PB in childhood because of its rarity.     

Bilateral reexpansion pulmonary edema associated with pleural empyema: a case report

Reexpansion pulmonary edema is an uncommon complication following rapid reexpansion of the lungs. The risk increases with a prolonged duration of pulmonary collapse, the amount of drained liquid or air, and with decreased time of draining. Treatment is supportive. In general, the prognosis is favorable. A nine-year-old boy was presented with fever, cough, and respiratory distress. Pneumonia and left-sided pleural empyema were determined and a chest tube was emplaced. Clinical deterioration occurred in just a few minutes following chest tube insertion. His chest radiography revealed a pulmonary edema in the left lung. Despite mechanical ventilation, antibiotics, and diuretic treatment, no significant improvement occurred. Acute respiratory distress syndrome and multiple organ dysfunctions developed in the follow-up. The patient died on day 5 of hospitalization. In this report, a complicated reexpansion pulmonary edema with a lathal outcome in a 9-year-old child is presented.     

Case of extra pulmonary,pleuro-pulmonary blastoma in a child: Pathological and cytogenetic findings

We report the cytogenetic findings in a case of Pleuro-Pulmonary Blastoma of Childhood Type II. This is a rare intrathoracic tumour that can occur in the lungs with up to 25% of cases being extra pulmonary. Med. Pediatr. Oncol. 29:61–64, 1997. © 1997 Wiley-Liss, Inc.     

Pulmonary blastoma in a 13-year-old boy. Case report and review of the literature

On examining a 13-year-old boy, a skull tumor and multiple pulmonary tumors were discovered. He was given three cycles of multidrug chemotherapy in accordance with Rosen's T-11 protocol. The skull tumor disappeared after the first cycle, but the lung masses remained unchanged. The diagnosis of pulmonary blastoma was made on the surgically removed specimen. Local recurrence occurred in the skull and widespread metastases developed later, despite high dose melphalan treatment with autologous bone-marrow rescue. The patient died 9 months after the debut. The case is presented to show the diagnostic difficulties in this rare tumor and to illustrate its varying biological behavior in the published cases. Child, high dose melphalan, pulmonary blastoma     

Chemotherapy of pulmonary blastoma: A case report and review of the literature

Pulmonary blastoma is a rare lung tumor of uncertain histogenesis. Although surgery may be curative therapy for localized lesions, recurrent tumor has been resistant to various treatment modalities. We report a case of metastatic pulmonary blastoma that had a partial remission with combination chemotherapy. Suggestions are made for the treatment of localized and metastatic pulmonary blastoma.     

Case report: Pulmonary blastoma in children—response to chemotherapy

Pulmonary blastoma (PB) is a rare primary malignancy of the lung, with about 54 cases reported in children. The tumor consists of mesenchymal and epithelial components resembling the fetal lung. It has been treated primarily with surgery and the effect of combination chemotherapy has not been systematically investigated. A 15-year-old girl with PB with metastases to bone and regional lymph node, and high levels of alphafetoprotein, is reported. A preoperative combination chemotherapy consisting of cisplatinum, etoposide alternating with iphosphamide with mesna, vincristine and epirubicine resulted in an objective response that permitted subsequent safe surgical excision of the primary tumor. This intensive combination chemotherapy should be tested in the management of advanced PB in children, as initial therapy as well as an adjuvant to surgery. © 1996 Wiley-Liss, Inc.     

Role of chemotherapy in pediatric pulmonary blastoma

Pulmonary blastoma is a rare malignant tumor of the lung that has been treated primarily with surgery. The effect of combination chemotherapy has not been systematically investigated. Two pediatric cases are reported in whom combination chemotherapy consisting of vincristine, actinomycin-D, cyclophosphamide, cis-platinum, and adriamycin was successfully used. The first case, a 5-year-old boy, underwent incomplete surgical excision of the tumor followed by a 104-week course of combination chemotherapy. The second case is a 3-year-old boy who was initially treated with combination chemotherapy that resulted in an objective response; he subsequently underwent surgical excision. This intensive combination chemotherapy is effective both in inoperable tumors as initial therapy as well as a surgical adjuvant.