Muhamad Tasnim, Amir Sayuti Khairil, Hasnita Ismail Mokhtar Noor, Hayati Yunus Nor, Syafawati Ismail Noor
Department of Radiology, School of Medical Sciences, Universiti Sains Malaysia, Jalan Raja Perempuan Zainab II, 16150 Kubang Kerian, Kelantan, Malaysia E-mail:
Hospital Universiti Sains Malaysia, Jalan Raja Perempuan Zainab II, 16150 Kubang Kerian, Kelantan, Malaysia E-mail:
Qatar Med J. 2022 Mar 1;2022(1):12. doi: 10.5339/qmj.2022.12. eCollection 2022.
Pleuropulmonary blastoma (PPB) is a rare malignant lung tumor in the pediatric population and occurs mainly in young children. Its clinical presentation is usually nonspecific. We report a rare occurrence of this tumor in a 15-year-old girl, who presented with symptoms mimicking respiratory tract infection and was nonresponsive to the initial treatment. Imaging investigations revealed a large solid lesion in the left hemithorax with a mass effect on the adjacent structures. Biopsy demonstrated primitive cells with blastematous appearances, and the stroma cells were positive for vimentin and desmin, consistent with PPB. Unfortunately, she died from neutropenic sepsis while undergoing chemotherapy. This report highlights the epidemiology of PPB, its imaging and histopathological features, overview of prognosis, and clinical management.
胸膜肺母细胞瘤（PPB）是儿科人群中一种罕见的恶性肺肿瘤，主要发生于幼儿。其临床表现通常不具有特异性。我们报告了1例15岁女孩罕见发生该肿瘤的病例，她表现出类似呼吸道感染的症状，对初始治疗无反应。影像学检查显示左半胸有一个大的实性病变，对邻近结构有肿块效应。活检显示具有母细胞样外观的原始细胞，间质细胞波形蛋白和结蛋白呈阳性，符合PPB。不幸的是，她在接受化疗时死于中性粒细胞减少性败血症。本报告强调了PPB的流行病学、其影像学和组织病理学特征、预后概述及临床管理。
