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内胚窦瘤(卵黄囊瘤)、精原细胞瘤和血管外皮细胞瘤。罕见的原发性纵隔肿瘤。9例患者的疾病病程报告。

Endodermal sinus tumor (yolk sac tumor), seminoma and hemangiopericytoma. Rare primary mediastinal tumors. A report on the course of the disease in nine patients.

作者信息

Dittler H J, Mack D, Steuer G

出版信息

Thorac Cardiovasc Surg. 1980 Aug;28(4):259-64. doi: 10.1055/s-2007-1022091.

DOI:10.1055/s-2007-1022091
PMID:6158129
Abstract

The pathological and clinical features of primary mediastinal hemangiopericytoma, seminoma, and endodermal sinus tumor are presented and discussed. These tumors were found in 3 patients each out of 898 cases of mediastinal tumor. Primary thoracic hemagiopericytomas are rare. The diagnosis can be determined by histology only, but nothing can be said about the malignancy of the tumor. Tumor recurrence is relatively frequent. Two patients were radically operated, and they are living today 4 and 4 1/2 years later, without evidence of recurrence. The third patient with clinically clearly malignant hemangiopericytoma was inoperable. This patient died 6 months after diagnosis. The anterior mediastinum is a predilection site for extragonadal germ cell tumors. Histologically there is no difference between this tumor and the gonadal seminoma. This tumor is well known to be radiosensitive. All 3 male patients were inoperable and received radiotherapy. One patient died after 4 months, the other 2 are living one and 3 years later, respectively, without complaints or evidence of recurrence. Only 12 cases of primary mediastinal endodermal sinus tumors have been reported in the literature, we observed 3 more such tumors. The highly malignant alpha-fetoprotein producing tumor occurs exclusively in young males. There is, as yet, no clear-cut form of treatment. One of our patients died after 2 weeks, another received chemotherapy and died a few months after diagnosis, the third received chemotherapy and irradiation and is living today, 20 months after discovery of the tumor.

摘要

本文介绍并讨论了原发性纵隔血管外皮细胞瘤、精原细胞瘤和内胚窦瘤的病理及临床特征。在898例纵隔肿瘤患者中,每种肿瘤各发现3例。原发性胸段血管外皮细胞瘤较为罕见。其诊断只能通过组织学确定,但无法判断肿瘤的恶性程度。肿瘤复发相对常见。2例患者接受了根治性手术,术后4年和4年半,至今仍存活,无复发迹象。第3例临床诊断为恶性血管外皮细胞瘤的患者无法手术,确诊后6个月死亡。前纵隔是性腺外生殖细胞肿瘤的好发部位。从组织学上看,该肿瘤与性腺精原细胞瘤并无差异。众所周知,这种肿瘤对放疗敏感。所有3例男性患者均无法手术,接受了放疗。1例患者4个月后死亡,另外2例分别在1年和3年后存活,无不适症状或复发迹象。文献中仅报道了12例原发性纵隔内胚窦瘤,我们又观察到3例。这种高度恶性的产生甲胎蛋白的肿瘤仅发生于年轻男性。目前尚无明确的治疗方法。我们的1例患者在2周后死亡,另1例接受化疗,确诊后几个月死亡,第3例接受化疗和放疗,在肿瘤发现后20个月,至今仍存活。

相似文献

1
Endodermal sinus tumor (yolk sac tumor), seminoma and hemangiopericytoma. Rare primary mediastinal tumors. A report on the course of the disease in nine patients.内胚窦瘤(卵黄囊瘤)、精原细胞瘤和血管外皮细胞瘤。罕见的原发性纵隔肿瘤。9例患者的疾病病程报告。
Thorac Cardiovasc Surg. 1980 Aug;28(4):259-64. doi: 10.1055/s-2007-1022091.
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