Yu Lu, Cheng Hong, Yang Shou Jing
Department of Pathology, Xijing Hospital, Fourth Military Medical University , Xi'an , China.
Fetal Pediatr Pathol. 2014 Feb;33(1):1-8. doi: 10.3109/15513815.2013.839011. Epub 2013 Sep 30.
Pleuropulmonary blastoma (PPB) is a rare malignant dysontogenetic neoplasm primarily affecting younger children, even in newborns with an unfavorable outcome. PPB is histologically composed of a primitive, variably mixed blastematous and sarcomatous components, and exclusively subclassified as type I (purely cystic), type II (both cystic and solid elements) and type III (completely solid) by increasing histological evidence of malignancy. At present, well-documented cases or cases of truly precise presentation of either pathological or immunohistochemical findings in PPB are rare. The authors report one case of PPB in a 44-month-old child presenting as a solid and cystic mass with special emphasis on its radiological, histopathological and immunohistochemical aspects. The histological diagnosis was PPB, which would belong to the type II category.
肺胚细胞瘤(PPB)是一种罕见的恶性发育异常肿瘤,主要影响年幼患儿,甚至在新生儿中也有发生,预后不良。PPB在组织学上由原始的、不同程度混合的胚基和肉瘤成分组成,根据恶性程度的组织学证据增加,可分为I型(纯囊性)、II型(囊性和实性成分均有)和III型(完全实性)。目前,PPB中病理或免疫组化结果记录良好或呈现真正精确表现的病例很少。作者报告了一例44个月大儿童的PPB病例,该病例表现为实性和囊性肿块,并特别强调了其放射学、组织病理学和免疫组化方面。组织学诊断为PPB,属于II型。