Franke A, Ströbel P, Fackeldey V, Schäfer R, Göller T, Becker H P, Schöneich R, Müller-Hermelink H K, Marx A
Department of Visceral and Thoracic Surgery, Central Army Hospital, Koblenz, Germany.
Am J Surg Pathol. 2004 Feb;28(2):250-6. doi: 10.1097/00000478-200402000-00014.
We describe the clinicopathologic findings in a so far unrecognized thymic tumor. The tumor occurred in a 70-year-old woman with respiratory distress but neither myasthenia gravis nor other symptoms. Metastases or another primary tumor were absent. The well-circumscribed neoplasm was located in the thymic region, measured 18 x 12 x 8 cm, and showed a homogeneous, tan-colored, soft cut surface. By histology, the tumor lacked a true capsule and a lobular growth pattern, was almost devoid of stroma, and infiltrated among remnant thymus lobules. The polygonal tumor cells formed solid sheets, trabeculae, or occurred as single cells that resembled hepatocytes. Proliferative activity was low. Portal structures, sinuses, and bile were absent as were areas of conventional thymoma, adenocarcinoma, or germ cell tumor. The tumor expressed cytokeratins 7 and 19, alpha1-antitrypsin, alpha1-antichymotrypsin, and hep-Par-1. Alpha-fetoprotein (AFP), human beta-chorionic gonadotropin (beta-HCG), placental alkaline phosphatase, CD5, CD30, CD31, CD34, CD45, CD68, CD99, S-100, HMB45, desmin, actin, or neuroendocrine markers were not expressed, and intratumorous CD1a+ or TdT+ immature T cells were absent. AFP was repeatedly undetectable in the blood. Mediastinal tumor recurrence was detected 6 months after surgery. Following radiochemotherapy, the patient has remained free of disease for 26 months. We conclude that this tumor is a thymic carcinoma (WHO type C thymoma). A diagnosis of hepatoid yolk sack tumor appears unlikely considering absence of a bona fide germ cell component, lack of AFP expression, and the patient's female gender. Because of its morphologic and immunohistochemical features, we propose the term "hepatoid thymic carcinoma" for this new type of thymic carcinoma.
我们描述了一种迄今为止未被认识的胸腺肿瘤的临床病理特征。该肿瘤发生在一名70岁女性身上,患者有呼吸窘迫症状,但无重症肌无力或其他症状。未发现转移灶或其他原发性肿瘤。该肿瘤边界清晰,位于胸腺区域,大小为18×12×8cm,切面呈均匀的黄褐色,质地柔软。组织学检查显示,肿瘤无真正的包膜和小叶状生长模式,几乎没有间质,浸润于残留的胸腺小叶之间。多边形肿瘤细胞形成实性片状、小梁状,或单个细胞存在,类似肝细胞。增殖活性较低。无门管结构、血窦和胆汁,也无传统胸腺瘤、腺癌或生殖细胞肿瘤区域。肿瘤表达细胞角蛋白7和19、α1-抗胰蛋白酶、α1-抗糜蛋白酶和肝型脂肪酸结合蛋白-1。未表达甲胎蛋白(AFP)、人绒毛膜促性腺激素β亚基(β-HCG)、胎盘碱性磷酸酶、CD5、CD30、CD31、CD34、CD45、CD68、CD99、S-100、HMB45、结蛋白、肌动蛋白或神经内分泌标志物,肿瘤内也无CD1a+或TdT+未成熟T细胞。血液中AFP多次检测不到。术后6个月检测到纵隔肿瘤复发。放化疗后,患者已无病生存26个月。我们得出结论,该肿瘤为胸腺癌(世界卫生组织C型胸腺瘤)。考虑到缺乏真正的生殖细胞成分、AFP不表达以及患者为女性,肝样卵黄囊瘤的诊断似乎不太可能。由于其形态学和免疫组化特征,我们建议将这种新型胸腺癌称为“肝样胸腺癌”。