Hypertensive pneumothorax with cystic lesions: Pleuropulmonary blastoma in an infant

被引:1
作者
Lamberti, Rossella [1 ]
Ghezzi, Michele [1 ]
Riccaboni, Francesca [1 ]
Farolfi, Andrea [1 ]
Zirpoli, Salvatore [3 ]
Zenga, Alessandra [1 ]
Riccipetitoni, Giovanna [4 ]
Zuccotti, Gianvincenzo [1 ,2 ]
D'Auria, Enza [1 ]
机构
[1] Vittore Buzzi Childrens Hosp, Pediat Dept, I-20154 Milan, Italy
[2] Univ Milan, Dept Biomed & Clin Sci L Sacco, I-20157 Milan, Italy
[3] Vittore Buzzi Childrens Hosp, Radiol Dept, I-20154 Milan, Italy
[4] Univ Pavia, Fdn IRCCS Policlin San Matteo, Pediat Surg Unit, Pavia, Italy
关键词
Children; Pleuropulmonary blastoma; Early surgical resection;
D O I
10.1016/j.rmcr.2022.101793
中图分类号
R56 [呼吸系及胸部疾病];
学科分类号
摘要
Pleuropulmonary blastoma (PPB) is a rare primitive malignant lung cancer that occurs in pedi-atric age. Its main differential diagnosis is congenital cystic pulmonary malformation (CPAM).A 30-day-old infant with respiratory failure obtained a chest x-ray and a computed tomogra-phy scan (CT) which revealed hypertensive pneumothorax with multifocal bilateral cysts. After thoracic drainage, the patient underwent multiple thoracoscopic pulmonary resections. The first histological diagnosis was of type 2 CPAM.During the radiological follow-up, an increase in the number and dimension of the lesions was detected. Thus, a histological revision was performed, leading to the diagnosis of type I PPB, at nine months. The patient subsequently underwent chemotherapy.At the five-year follow-up appointment, chest magnetic resonance (MR) and CT scans showed a dimensional increase in size of the lesions, with the risk of recurrent pneumothorax. An upper right lobectomy and wedge resection of the residual cysts were performed.Control MR scans showed normalization of the lung parenchyma and the patient showed sub-stantial clinical improvement.
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页数:5
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