The "sugar" clear cell tumor of the lung-clinical presentation and diagnostic difficulties of an unusual lung tumor in youth.

The "sugar" clear cell tumor of the lung-clinical presentation and diagnostic difficulties of an unusual lung tumor in youth.
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DOI:
10.1016/j.jpedsurg.2006.02.035
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发表时间:
2006-06-01
影响因子:
2.4
通讯作者:
Jaskiewicz, Kazimierz
Jaskiewicz, Kazimierz
中科院分区:
医学3区
文献类型:
--
作者:
Gora-Gebka, Magdalena;Liberek, Anna;Jaskiewicz, Kazimierz

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本文报告一例16岁男孩的肺部透明细胞瘤。该疾病的一般症状的过程中,从来没有报道过,突出了诊断的困难,一个非常罕见的肺肿瘤的青年。该男孩每天出现持续6周的不明原因高热,表现为低色素性小细胞性贫血、红细胞沉降率、C反应蛋白、α 2和β球蛋白升高以及血小板计数升高。肺部肿瘤是一个黄色的,局限性肿块局限于第六段的左肺。组织学检查显示肿瘤由细胞组成,细胞质透明,糖原含量高,无坏死迹象,HMB-45呈免疫反应性,但细胞角蛋白、LCA、CD 34和CD 68不呈免疫反应性。行开胸和肺段切除术均能确诊和治愈。
A case of the "sugar" clear cell tumor of the lung in a 16-year-old boy is presented. The course of the disease with general symptoms, never reported before, highlights diagnostic difficulties of an extremely rare lung tumor in youth. The boy presented with daily spikes of unexplained high fever of 6 weeks' duration with features of hypochromic microcytic anemia, elevated erythrocyte sedimentation rate, C-reactive protein, alpha(2)- and beta-globulins, and elevated platelet count. The lung tumor was a yellow, circumscribed mass confined to the sixth segment of the left lung. Histological examination revealed the tumor composed of cells with clear cytoplasm with large content of glycogen, with no signs of necrosis, and immunoreactive for HMB-45, but not for cytokeratin, LCA, CD34, and CD68. The performed thoracotomy and segmentectomy were both diagnostic and curative.