胸膜肺母细胞瘤（儿童期肺母细胞瘤）：与其他胚胎性恶性肿瘤的遗传关联？

Pleuropulmonary blastoma (pulmonary blastoma of childhood): genetic link with other embryonal malignancies?

作者信息

Sciot R, Dal Cin P, Brock P, Moerman P, Van Damme B, De Wever I, Casteels-Van Daele M, Van den Berghe H, Desmet V

机构信息

Department of Pathology, University of Leuven, Belgium.

出版信息

Histopathology. 1994 Jun;24(6):559-63. doi: 10.1111/j.1365-2559.1994.tb00576.x.

DOI:10.1111/j.1365-2559.1994.tb00576.x

PMID:8063284

Abstract

A case of pleuropulmonary blastoma (childhood variant of pulmonary blastoma) was examined using histological, immunohistochemical, ultrastructural and cytogenetic methods. The tumour consisted of undifferentiated 'blastematous' areas admixed with zones of rhabdomyoblastic and chondroid differentiation and fascicular areas. Desmin and S-100 protein immunoreactivity confirmed the myogenic and cartilaginous differentiation. Ultrastructurally only undifferentiated mesenchymal cells were present. The cytogenetic analysis revealed abnormalities of 2q. Involvement of 2q has also been described in hepatoblastoma and embryonal rhabdomyosarcoma. Although further confirmation is needed, our cytogenetic findings in pleuropulmonary blastoma suggest common genetic mechanisms in some paediatric embryonal malignancies.

摘要

采用组织学、免疫组织化学、超微结构和细胞遗传学方法对1例胸膜肺母细胞瘤（肺母细胞瘤的儿童型）进行了检查。肿瘤由未分化的“胚基样”区域组成，混有横纹肌母细胞和软骨样分化区域以及束状区域。结蛋白和S-100蛋白免疫反应证实了肌源性和软骨性分化。超微结构上仅见未分化的间充质细胞。细胞遗传学分析显示2号染色体长臂异常。在肝母细胞瘤和胚胎性横纹肌肉瘤中也有2号染色体长臂受累的报道。虽然需要进一步证实，但我们在胸膜肺母细胞瘤中的细胞遗传学发现提示某些儿童胚胎性恶性肿瘤存在共同的遗传机制。