Madaan Prateek K, Sidhu Harsumeet S, Girdhar Smriti, Mann Khushpreet K
Department of Radiodiagnosis, VMMC and Safdarjung Hospital, New Delhi, India.
Radiodiagnosis, Resident Doctor, Department of Radiodiagnosis, ABVIMS and Dr RML Hospital, New Delhi, India.
Radiol Case Rep. 2021 Aug 1;16(10):2862-2868. doi: 10.1016/j.radcr.2021.06.046. eCollection 2021 Oct.
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.
肺胚细胞瘤是一种罕见且侵袭性很强的儿童肺部恶性肿瘤。临床上，这种恶性肿瘤常被误诊为呼吸道感染或气胸的症状。该肿瘤在组织学上的特征是原始胚基和具有多向分化能力的恶性间充质基质。肺胚细胞瘤患者采用多模式治疗。我们报告3例经组织病理学诊断为肺胚细胞瘤的病例。这些患者的主要症状为呼吸困难、咳嗽、发热和胸痛。患者的X线片显示半侧胸腔部分或完全致密影。增强计算机断层扫描显示胸腔内有边界清晰的、不均匀强化的大实性肿块。了解其类型、影像学表现、分期以及与其他肿瘤的关联对于正确诊断肺胚细胞瘤及后续的适当治疗至关重要。
