Moran C A, Suster S
Department of Pulmonary and Mediastinal Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA.
Am J Surg Pathol. 1997 Sep;21(9):1007-12. doi: 10.1097/00000478-199709000-00004.
Primary choriocarcinoma of the anterior mediastinum is by far the rarest and most controversial form of extragonadal germ cell tumor. A clinicopathologic study of eight primary mediastinal neoplasms bearing the histopathologic and immunohistochemical features of choriocarcinoma is presented. The patients were all men between the ages of 21 and 63 years (mean, 42 years). Clinical symptoms included shortness of breath, chest pain, cough, and superior vena cava syndrome; one patient also had gynecomastia. All patients presented with large anterior mediastinal masses on chest radiographs that measured an average of 10 cm in greatest diameter. Grossly, the tumors were described as large, soft, extensively hemorrhagic, and with foci of necrosis. Histologically, they were characterized by a dual cell population composed of cytotrophoblastic cells with uniform, round nuclei, clear cytoplasm, and prominent nucleoli admixed with large, multinucleated syncytiotrophoblastic cells with bizarre nuclei, prominent nucleoli, and abundant eosinophilic cytoplasm. Immunohistochemically, the tumors were notable for strong keratin and beta-human chorionic gonadotropin (HCG) positivity. Seven patients presented at the time of diagnosis with thoracic and extrathoracic (liver, adrenal, kidney, and spleen) metastases. In one case, the tumor was entirely confined to the mediastinum. All patients died over a period of 1 to 2 months. Complete autopsies were performed in all cases; none of the patients showed evidence of a testicular tumor or scar after thorough examination of the testes on serial sectioning. The present cases demonstrate the widespread distribution of germ cells in the human body and lend further support to the existence of primary extragonadal choriocarcinoma arising in the thymic region.
前纵隔原发性绒毛膜癌是目前最罕见且最具争议的性腺外生殖细胞肿瘤形式。本文呈现了一项对8例具有绒毛膜癌组织病理学和免疫组织化学特征的原发性纵隔肿瘤的临床病理研究。患者均为男性,年龄在21至63岁之间(平均42岁)。临床症状包括呼吸急促、胸痛、咳嗽和上腔静脉综合征;1例患者还伴有男性乳房发育。所有患者胸部X线片均显示前纵隔有巨大肿块,最大直径平均为10 cm。大体上,肿瘤被描述为巨大、柔软、广泛出血并有坏死灶。组织学上,其特征为双细胞群,由细胞核均匀、圆形、胞质清晰且核仁明显的细胞滋养层细胞与细胞核怪异、核仁突出且嗜酸性胞质丰富的大的多核合体滋养层细胞混合组成。免疫组织化学检查显示,肿瘤以强烈的角蛋白和β-人绒毛膜促性腺激素(HCG)阳性为显著特征。7例患者在诊断时已出现胸内和胸外(肝脏、肾上腺、肾脏和脾脏)转移。1例患者肿瘤完全局限于纵隔。所有患者在1至2个月内死亡。所有病例均进行了完整尸检;经对睾丸进行系列切片彻底检查,无一例患者显示有睾丸肿瘤或瘢痕的证据。本病例证明了生殖细胞在人体内的广泛分布,并进一步支持了胸腺区域存在原发性性腺外绒毛膜癌的观点。