Campos Marcos Adriano Garcia, Vasques Lucas Fernandes, de Medeiros Rafael Goulart, Monteiro Cutrim Érico Murilo, Favarin Ana Júlia, Silva Sarah Rebecca Machado, Silva Gyl Eanes Barros, Moraes Marcelo Padovani de Toledo, Zanatta Mariana Lopes, Queiróz Diego Aparecido Rios
Department of Internal Medicine, Hospital of Medical School of São Paulo State University, Botucatu, Brazil.
Department of Medicine, University of Taubate Faculty of Medicine, Taubaté, Brazil.
Front Oncol. 2023 Sep 20;13:1260844. doi: 10.3389/fonc.2023.1260844. eCollection 2023.
Clear cell tumors of the lung (CCTL), or "sugar tumors" of lung, are very uncommon lesions and are mostly benign perivascular epithelioid cell (PEC) tumors with no specific morphologic features. Fewer than 100 cases have been reported; the aggressive nature demonstrated in sporadic reports has rarely been described in the literature. Although the course is generally described as benign, eight reported cases showed malignant behavior. We report a case of a PEC with a malignant presentation in a young man, correlating the main characteristics of the tumor with other cases reported in the literature to better elucidate this rare presentation. We also performed a literature review of reports on benign and malignant CCTL cases, with a focus on clinical, imaging, and immunohistochemical differentiation. CCTLs are rare tumors that require histopathological and immunohistochemical confirmation; to date, criteria that can predict malignant evolution are lacking.
肺透明细胞瘤（CCTL），即肺部的“糖瘤”，是非常罕见的病变，大多为良性血管周上皮样细胞（PEC）肿瘤，无特异性形态特征。报道的病例少于100例；散发性报道中显示的侵袭性在文献中很少被描述。虽然病程通常被描述为良性，但有8例报道显示为恶性行为。我们报告了1例年轻男性中表现为恶性的PEC病例，将该肿瘤的主要特征与文献中报道的其他病例相关联，以更好地阐明这种罕见表现。我们还对良性和恶性CCTL病例的报告进行了文献综述，重点关注临床、影像学和免疫组化鉴别。CCTL是罕见肿瘤，需要组织病理学和免疫组化确诊；迄今为止，缺乏能够预测恶性进展的标准。
