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纵隔生殖细胞肿瘤合并脾脏组织细胞肉瘤:一例罕见关联的病例报告

Mediastinal germ cell tumor associated with histiocytic sarcoma of spleen: case report of an unusual association.

作者信息

Song Sang Yong, Ko Young Hyeh, Ahn Geunghwan

机构信息

Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50 Irwon-dong, Gangnam-gu, Seoul, Korea.

出版信息

Int J Surg Pathol. 2005 Jul;13(3):299-303. doi: 10.1177/106689690501300314.

Abstract

We present an unusual association of mediastinal germ cell tumor containing seminoma and angiosarcoma components and splenic histiocytic sarcoma. A 15-year-old boy presented with chest pain. Histopathologically, an anterior mediastinal mass contained typical seminoma, immature teratoma, embryonal carcinoma, angiosarcoma, yolk sac tumor, and polyembryoma. An abdominal ultrasonogram revealed a huge splenomegaly with multiple ill-defined low echogenic nodules, 1 month after the second cycle of chemotherapy. Histopathologically, large, round-to-oval tumor cells with abundant eosinophilic cytoplasm often contained eccentrically placed nuclei with vesicular chromatin and an irregular nuclear membrane. The tumor cells were immunoreactive for CD68, CD31, and CD4. The cytogenetic results showed deletion of the long arm of chromosome 5 and trisomy 8. This lesion might have been on the pathway of multistep tumorigenesis toward a final leukemia.

摘要

我们报告了一例罕见的纵隔生殖细胞肿瘤,其包含精原细胞瘤和血管肉瘤成分,同时伴有脾脏组织细胞肉瘤。一名15岁男孩因胸痛就诊。组织病理学检查显示,前纵隔肿块包含典型的精原细胞瘤、未成熟畸胎瘤、胚胎癌、血管肉瘤、卵黄囊瘤和多胚瘤。在第二轮化疗1个月后,腹部超声检查发现脾脏巨大,伴有多个边界不清的低回声结节。组织病理学检查显示,大的圆形至椭圆形肿瘤细胞,胞质丰富嗜酸性,细胞核常偏位,染色质呈泡状,核膜不规则。肿瘤细胞对CD68、CD31和CD4呈免疫反应阳性。细胞遗传学结果显示5号染色体长臂缺失和8号染色体三体。该病变可能处于多步骤肿瘤发生过程中,最终发展为白血病。

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