Uptake of technetium 99m MDP by hepatoblastoma

Focal uptake of ^99mTc-MDP was seen in a case of hepatoblastoma. The focal uptake corresponded to an area of calcification on CT, which was shown histologically to consist of osteoid with mineralization. The mechanism of uptake by the tumor in this case is likely to be the same as for skeletal uptake.     

P53 gene mutations in pleuropulmonary blastomas

Pleuropulmonary blastoma (PPB) is rarechildhoodtumororiginating fromeitherlung orpleura. Although several cytogenetic changes, such as trisomy 2, trisomy 8, and loss of 17p material, have been reported, evidence of gene mutations is still lacking. Pathologically, PPB shares similarities with rhabdomyosarcoma in which p53 mutations are frequently detected. Possible implication of p53 mutations in PPB was investigated. PPBs of 3 patients were analyzed for occurrence of p53 mutations by using polymerase chain reaction-single-strand conformation polymorphism (PCR-SSCP) method, and the nature of mutations was confirmed by direct sequencing. Two PPBs were confirmed to harbor p53 mutations. One was a Val to Leu substitution at codon 173, and another was a ArgArg to TrpCys substitution at codons 282 and 283. In each tumor, only the mutated allele was detected, suggesting inactivation of p53. Both patients with mutations had fatal outcome, while the remaining patient in whom no mutation was detected is disease free for 3 years after completion of treatment. The results raise the possibility that p53 inactivation can occur as a nonrandom genetic change involving the pathogenesis and outcome of PPB. Further studies in a larger series are necessary to clarify these matters.     

Pleuropulmonary blastoma: A report of three cases and review of literature

Pleuropulmonary blastoma is a rare and highly aggressive pulmonary malignancy in children. Clinically, the malignancy is often mistaken for symptoms of respiratory tract infection or pneumothorax. The neoplasm is histologically characterized by primitive blastema and a malignant mesenchymal stroma that demonstrates multidirectional differentiation. The patients with PPB are managed by multimodal therapy. We present a report of 3 cases of histopathologically diagnosed pleuropulmonary blastoma. The patients presented with chief complaints of difficulty in breathing, cough, fever and chest pain. Radiographs of the patients showed partial to complete opacification of hemithorax. Contrast enhanced computed tomography scans revealed large well defined heterogenously enhancing solid mass lesions in the hemithorax. Knowledge of types, imaging findings, staging and association with other tumors is crucial for correct diagnosis of pleuropulmonary blastoma and subsequent adequate management.     

小儿胸膜肺母细胞瘤的诊断与治疗分析

目的初步探讨小儿胸膜肺母细胞瘤的临床特征、诊断与治疗方法。方法回顾性分析上海市儿童医院2013年10月至2017年6月收治的6例胸膜肺母细胞瘤患儿临床资料,其中男3例,女3例;年龄范围1岁2个月至10岁。5例患儿行肿瘤大部分切除(其中部分肺叶切除术1例,肺叶修补术4例),1例行肿瘤完整切除。6例均获得有效随访,随访时间1~34个月。结果 6例患儿中5例首诊被误诊,其中3例首诊为肺炎,1例首诊为胸腔积液,1例首诊为先天性肺囊性腺瘤样畸形。6例患儿随访结果分别为:临床痊愈1例;带瘤生存1例(目前正规接受化疗中);术后1个月死亡3例;术后2个月死亡1例。结论小儿胸膜肺母细胞瘤临床表现无特异性,Ⅰ型胸膜肺母细胞瘤易误诊为先天性肺囊性腺瘤样畸形,术后预后相对理想;早期发现、早期手术、后续正规化疗是治疗的主要方法。     

Pleuropulmonary blastoma: an aggressive intrathoracic neoplasm of childhood

A case of a 4-year-old girl with pleuropulmonary blastoma is reported. Surgical resection of the tumor was performed and histologic examination revealed pleuropulmonary blastoma with rhabdomyosarcomatous differentiation. Postoperative chemotherapy was administered and 3 weeks after initiation of treatment protocol a second site of lesion in the retroperitoneum was revealed with extension to the mediastinum, which shared similar mesenchymal neoplastic characteristics to the previously diagnosed primary lesion. The girl died 4 1/2 months after initial evidence of disease because of brain metastasis, indicating a very aggressive neoplasm unresponsive to treatment.     

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。     

Pharyngeal teratocarcinosarcoma: review of the literature and report of two cases

Teratocarcinosarcomas are rare malignant neoplasms histologically characterized by the presence of benign and malignant epithelial and mesenchymal elements. They are seen almost exclusively in the sinonasal tract of men. We report two cases of teratocarcinosarcomas involving the posterior pharyngeal wall in a 55-year-old male and 60-year-old men. The tumors consisted of epithelial components including squamous, neuroendocrine, and glandular structures; neuroepithelium, and mesenchymal components with prominent rhabdomyoblastic, osteoblastic and chondroid differentiation. Immunohistochemical studies demonstrated markers characteristic of each component. The tumors were resected, and the patients received postoperative radiation therapy. One patient is alive with recurrent tumor 33 months after treatment and the other died 26 months after radiation therapy with distant metastasis.     

Pulmonary blastoma

Summary A primary pulmonary tumour in a four year old boy arising from the subpleural zone of the lung is described. It contains both stromal and epithelial elements at the primary site and in the bony metastases. The combination of the age of the patient, the site of the tumour, the stromal and epithelial elements present in the tumour suggest that this is a true pulmonary blastoma. Electronmicroscopy showed the presence of intranuclear viral particles.