Tsuji S, Hisaoka M, Morimitsu Y, Hashimoto H, Jimi A, Watanabe J, Eguchi H, Kaneko Y
Department of Pathology and Oncology, School of Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan.
Histopathology. 1998 Oct;33(4):369-74. doi: 10.1046/j.1365-2559.1998.00485.x.
We report two unusual cases of intrapulmonary small round cell tumour with evidence that they are primitive neuroectodermal tumours.
The patients were a 25-year-old woman and a 15-year-old man, in whom mass shadows were found by routine chest radiography. Both tumours were located in the pulmonary parenchyma, and there was no evidence of an extrapulmonary involvement by the tumour. The lesions showed morphological and immunophenotypic features consistent with peripheral primitive neuroectodermal tumour (pPNET). These features included a hypercellular diffuse growth pattern of closely packed small round cells occasionally forming abortive Homer-Wright-type rosettes and intense immuroreactivities to neurone-specific enolase and MIC2 gene product (O13). Unusual chondroid foci were present in one of the cases. This diagnosis was further supported by the cytogenetic and reverse transcriptase-polymerase chain reaction (RT-PCR) findings of the characteristic t(11;22) chromosomal translocation and EWS/FLI-1 fusion transcripts, respectively, in one of the cases.
These morphological and cytogenetic findings substantiate pPNET as a subtype of intrapulmonary small round cell tumours.
我们报告两例罕见的肺内小圆形细胞肿瘤病例，有证据表明它们是原始神经外胚层肿瘤。
患者分别为一名25岁女性和一名15岁男性，通过常规胸部X线检查发现肺部有肿块阴影。两个肿瘤均位于肺实质内，无肿瘤肺外受累的证据。病变显示出与外周原始神经外胚层肿瘤（pPNET）一致的形态学和免疫表型特征。这些特征包括由紧密排列的小圆形细胞组成的高细胞密度弥漫性生长模式，偶尔形成不完整的霍纳-赖特（Homer-Wright）型菊形团，以及对神经元特异性烯醇化酶和MIC2基因产物（O13）的强烈免疫反应性。其中一例出现了异常的软骨样灶。在其中一例中，细胞遗传学和逆转录聚合酶链反应（RT-PCR）分别发现特征性的t(11;22)染色体易位和EWS/FLI-1融合转录本，进一步支持了这一诊断。
这些形态学和细胞遗传学发现证实pPNET是肺内小圆形细胞肿瘤的一种亚型。
