Al-Backer Nouf, Puligandla Pramod S, Su Wendy, Anselmo Mark, Laberge Jean-Martin
Division of Pediatric Respirology, The Montreal Children's Hospital, Montreal, Quebec, Canada H3H 1P3.
J Pediatr Surg. 2006 Nov;41(11):e13-5. doi: 10.1016/j.jpedsurg.2006.07.015.
Pleuropulmonary blastoma (PPB) is a rare and aggressive intrathoracic malignant tumor that can be associated with cystic lung lesions in children. This neoplasm is histologically characterized by primitive blastoma and a malignant mesenchymal stroma. The authors describe a 3-year-old boy who presented with a history of fever and cough. Radiological imaging demonstrated a large cystic lesion replacing the left lower lobe. The patient underwent thoracoscopic resection of the lesion. Interestingly, the histopathology demonstrated a type 1 PPB. Type 1 lesions are usually observed in young infants, whereas older infants and children tend to present with type 2 or 3 PPB, which carry a poorer prognosis and higher risk of recurrence. Thus, the presence of large or peripherally based lung cysts should raise the suspicion of PPB. Resection is warranted for all such lesions.
肺胚细胞瘤（PPB）是一种罕见且侵袭性强的胸腔内恶性肿瘤，可与儿童肺部囊性病变相关。这种肿瘤在组织学上的特征是原始母细胞瘤和恶性间充质基质。作者描述了一名3岁男孩，有发热和咳嗽病史。影像学检查显示一个大的囊性病变取代了左下叶。患者接受了胸腔镜下病变切除术。有趣的是，组织病理学显示为1型PPB。1型病变通常见于幼儿，而较大婴儿和儿童往往表现为2型或3型PPB，其预后较差且复发风险较高。因此，出现大的或位于周边的肺囊肿应怀疑PPB。所有此类病变均需进行切除。
