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一名7个月大、即将出现呼吸衰竭的女婴患III型胸膜肺母细胞瘤:病例报告

Type III pleuropulmonary blastoma in a 7-month-old female baby with impending respiratory failure: a case report.

作者信息

Castro Agnese, Franzonello Chiara, Leonardi Salvatore, Di Cataldo Andrea, Potenza Enrico, Magro Gaetano, Rossi Giovanni A, La Rosa Mario

机构信息

Department of Pediatrics, University of Catania, Catania, Italy.

出版信息

J Med Case Rep. 2014 Jun 23;8:221. doi: 10.1186/1752-1947-8-221.

Abstract

INTRODUCTION

Pleuropulmonary blastoma is a very rare, aggressive, embryonal pulmonary neoplasm which mostly affects children under the age of 5. According to the histopathological features, three subtypes of pleuropulmonary blastoma have been recognized: type I (purely cystic), type II (grossly visible cystic and solid elements) and type III (purely solid). Characteristics of type I and type II blastoma allow an earlier diagnosis compared with type III. Here we present a case report of an unusual presentation of type III pleuropulmonary blastoma.

CASE PRESENTATION

We describe the case of a 7-month-old female baby of Italian mother and Kurdish father who was diagnosed with type III pleuropulmonary blastoma, which entirely occupied her right hemithorax.

CONCLUSIONS

The reported case is an unusual presentation because type III pleuropulmonary blastoma typically occurs in older children. The complete re-expansion of her residual, previously totally compressed, right lung observed immediately after the resection of the lesion suggests an atypical rapid growth of this embryonal tumor in the late phase of gestation or after delivery. This case report suggests that, in addition to other childhood tumors, type III pleuropulmonary blastoma should be included in the differential diagnosis of solid nonhomogeneous thoracic large masses, compressing the mediastinal and chest wall structures in infants. This is an original case report of interest for several specialities such us pediatrics, radiology, surgery and oncology.

摘要

引言

胸膜肺母细胞瘤是一种非常罕见的、侵袭性的胚胎性肺肿瘤,主要影响5岁以下儿童。根据组织病理学特征,胸膜肺母细胞瘤可分为三种亚型:I型(纯囊性)、II型(肉眼可见囊性和实性成分)和III型(纯实性)。与III型相比,I型和II型母细胞瘤的特征有助于更早诊断。在此,我们报告一例III型胸膜肺母细胞瘤的不寻常表现病例。

病例介绍

我们描述了一名7个月大女婴的病例,其母亲为意大利人,父亲为库尔德人,被诊断为III型胸膜肺母细胞瘤,肿瘤完全占据了她的右半胸。

结论

报告的该病例表现不寻常,因为III型胸膜肺母细胞瘤通常发生于年龄较大的儿童。在切除病变后立即观察到其残留的、先前完全受压的右肺完全复张,提示这种胚胎性肿瘤在妊娠晚期或出生后非典型快速生长。本病例报告表明,除其他儿童肿瘤外,III型胸膜肺母细胞瘤应纳入婴儿实性不均匀性胸部大肿块的鉴别诊断,这些肿块压迫纵隔和胸壁结构。这是一篇对儿科、放射科、外科和肿瘤科等多个专业有参考价值的原创病例报告。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8fd3/4131777/8c3703f6c97e/1752-1947-8-221-1.jpg

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