Shin B K, Kim M K, Park S H, Kim C Y, Cho S J, Lee K C, Kim K T, Oh Y H, Kim I, Kim H K
Department of Anatomical Pathology, Korea University Anam Hospital, Seoul, Korea.
Diagn Cytopathol. 2001 Dec;25(6):397-402. doi: 10.1002/dc.10006.
Pleuropulmonary blastoma (PPB) is a rare and aggressive intrathoracic neoplasm of childhood, typically presenting as a pulmonary and/or pleural-based mass with cystic, solid, or combined features. Histologically, the tumor is well characterized with a mixture of primitive mesenchymal and variably differentiated sarcomatous components, and the cytologic features described in the three previous reports are also compatible to the histologic ones. Now, we present another case of PPB showing unusual features. A 3-yr-old boy presented with a pleural- or chest wall-based tumor. The fine-needle aspiration cytologic smears were highly cellular with poorly differentiated cells showing variable size and shape rather than those of typical blastemal cells. The histologic findings were also distinctive, exhibiting diffuse sheets of poorly differentiated cells without typical blastemal cell component. Diagnosis was confirmed by the support of immunohistochemical and ultrastructural features. The patient underwent a typical aggressive clinical course to death within 8 mo after diagnosis.
肺胚细胞瘤(PPB)是一种罕见的、侵袭性的儿童胸腔内肿瘤,通常表现为以肺和/或胸膜为基础的肿块,具有囊性、实性或混合特征。在组织学上,该肿瘤具有原始间充质和不同程度分化的肉瘤成分混合的典型特征,并且之前三篇报告中描述的细胞学特征也与组织学特征相符。现在,我们报告另一例具有不寻常特征的PPB病例。一名3岁男孩出现以胸膜或胸壁为基础的肿瘤。细针穿刺细胞学涂片细胞丰富,细胞分化差,大小和形状各异,而非典型的胚基细胞。组织学表现也很独特,显示弥漫性的分化差的细胞片,无典型的胚基细胞成分。免疫组化和超微结构特征支持诊断。该患者在诊断后8个月内经历了典型的侵袭性临床病程并死亡。