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肺母细胞瘤

Pulmonary blastomas.

作者信息

Koss M N, Hochholzer L, O'Leary T

机构信息

Department of Pathology, University of Southern California (USC), School of Medicine, Los Angeles.

出版信息

Cancer. 1991 May 1;67(9):2368-81. doi: 10.1002/1097-0142(19910501)67:9<2368::aid-cncr2820670926>3.0.co;2-g.

Abstract

Pulmonary blastoma is a rare lung tumor composed of immature mesenchyme and/or epithelium that morphologically mimics embryonal pulmonary structure. The prognosis of these tumors is poor, and the clinical course is not readily predicted from histologic appearance. In this report, the clinical, gross, microscopic, and immunopathologic features of 52 cases are described, and prognostically important correlates are determined. Twenty-eight patients were women, and 24 were men. There was a unimodal age peak in the fourth decade; only two patients were younger than 10 years old, and both had biphasic blastomas. Forty-one percent of patients were asymptomatic. Chest radiography typically showed a peripheral or midlung mass without predilection for any lobe. Microscopically, tumors could be divided into two classes: those composed solely of malignant glands of embryonal appearance (well-differentiated fetal adenocarcinomas [WDFA], 28 cases) and those with a biphasic appearance (24 cases). The malignant epithelium contained cytokeratin, carcinoembryonic antigen, milk fat globulin, and often chromogranin; vimentin, actin, and less frequently desmin and myoglobin were present in malignant stromal cells. More often WDFA was a smaller tumor (less than 5 cm) than biphasic tumors (P less than or equal to 0.001). It was more likely to be asymptomatic (P less than or equal to 0.001), and it was less likely to show pleural effusion by chest radiography (P less than or equal to 0.01) or giant or bizarre tumor cells (P less than or equal to 0.001) or frequent (greater than or equal to 30 mitoses/10 high-power fields) mitoses in the microscopic sections (P less than or equal to 0.01). Only 14% of patients with WDFA died of their tumor; 52% of patients with biphasic tumors died (mean follow-up, 97 months and 49 months, respectively). For patients with WDFA, the presence of thoracic adenopathy by chest radiography (P less than or equal to 0.001) and metastasis at initial presentation (P less than or equal to 0.001), followed by tumor recurrence (P less than or equal to 0.01), were the factors most highly correlated with poor prognosis. For patients with biphasic tumors, tumor recurrence (P less than or equal to 0.001) was the most significant indicator of poor prognosis, followed by metastasis at initial presentation (P less than or equal to 0.05) and gross size of the tumor (greater than or equal to 5 cm) (P less than or equal to 0.05). These findings support the idea that histologic class and gross and clinical findings can be of prognostic value in pulmonary blastoma.

摘要

肺母细胞瘤是一种罕见的肺肿瘤,由未成熟的间充质和/或上皮组成,形态上模拟胚胎肺结构。这些肿瘤的预后较差,临床病程难以从组织学表现进行预测。在本报告中,描述了52例病例的临床、大体、显微镜和免疫病理特征,并确定了具有预后意义的相关因素。28例患者为女性,24例为男性。发病年龄高峰在40岁;只有2例患者年龄小于10岁,且均为双相性母细胞瘤。41%的患者无症状。胸部X线检查通常显示外周或肺中部肿块,无任何肺叶偏好。显微镜下,肿瘤可分为两类:一类仅由胚胎样恶性腺体组成(高分化胎儿腺癌[WDFA],28例),另一类具有双相外观(24例)。恶性上皮细胞含有细胞角蛋白、癌胚抗原、乳脂肪球蛋白,且常含有嗜铬粒蛋白;波形蛋白、肌动蛋白,较少见的结蛋白和肌红蛋白存在于恶性基质细胞中。WDFA通常比双相性肿瘤小(小于5 cm)(P≤0.001)。它更可能无症状(P≤0.001),胸部X线检查显示胸腔积液的可能性较小(P≤0.01),显微镜切片中出现巨大或怪异肿瘤细胞的可能性较小(P≤0.001)或有频繁(≥30个有丝分裂/10个高倍视野)有丝分裂的可能性较小(P≤0.01)。WDFA患者中只有14%死于肿瘤;双相性肿瘤患者中有52%死亡(平均随访时间分别为97个月和49个月)。对于WDFA患者,胸部X线检查显示胸腺瘤(P≤0.001)和初诊时转移(P≤0.001),其次是肿瘤复发(P≤0.01),是与预后不良最密切相关的因素。对于双相性肿瘤患者,肿瘤复发(P≤0.001)是预后不良的最显著指标,其次是初诊时转移(P≤0.05)和肿瘤大体大小(≥5 cm)(P≤0.05)。这些发现支持组织学类型以及大体和临床发现对肺母细胞瘤具有预后价值这一观点。

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