Hamad Sara G, Al-Naimi Amal, Abu-Hasan Mutasim
Pediatric Pulmonology, Sidra Medicine, Doha, Qatar.
Case Rep Pulmonol. 2021 Oct 29;2021:1983864. doi: 10.1155/2021/1983864. eCollection 2021.
Pleuropulmonary blastoma (PPB) is a rare intrathoracic malignancy, which arises from the lung parenchyma and/or pleura. PPB has strong genetic association with mutations in gene. Despite being rare, PPB is the most common lung tumor in children below 6 years of age. International registry of the disease has a total of 350 cases worldwide. We report the first case of PPB in the state of Qatar, which presented as a large cystic lung lesion. The patient was first thought to have benign congenital pulmonary airway malformation (CPAM) based on chest X-ray findings. The diagnosis of PPB was suspected based on chest CT scan findings and was confirmed after surgical resection of the cystic mass. The case highlights the need to consider PPB in the differential diagnosis of cystic lung lesions in children and the need for further radiological imaging (i.e., CT scan), genetic testing, and/or excisional biopsy to confirm the diagnosis.
肺胚细胞瘤（PPB）是一种罕见的胸内恶性肿瘤，起源于肺实质和/或胸膜。PPB与该基因的突变有很强的遗传关联。尽管罕见，但PPB是6岁以下儿童最常见的肺部肿瘤。该疾病的国际登记处全球共有350例病例。我们报告卡塔尔首例PPB病例，表现为肺部巨大囊性病变。根据胸部X光检查结果，患者最初被认为患有良性先天性肺气道畸形（CPAM）。基于胸部CT扫描结果怀疑为PPB，并在手术切除囊性肿块后得到证实。该病例强调了在儿童囊性肺病变的鉴别诊断中需要考虑PPB，以及需要进一步的放射学成像（即CT扫描）、基因检测和/或切除活检来确诊。
