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[纵隔原发性生殖细胞瘤:1例长达7年病程的病例研究]

[Primary germinal tumor of the mediastinum: study of a case evolving over 7 years].

作者信息

Uldry P A, Barrelet L

出版信息

Schweiz Med Wochenschr. 1986 Sep 27;116(39):1337-43.

PMID:3775340
Abstract

Mediastinal seminoma is a rare malignant germ cell neoplasm histologically identical to the more common gonadal germinoma. The case reported here illustrates typical findings for presentation and response to primary therapy in mediastinal seminoma. The tumor was located in the anterior mediastinum and appeared as a well-defined lobulated mass. However, the patient's subsequent course was unusual in the multiplicity of sites of disseminated disease, and in that all were apparently controlled by local irradiation and chemotherapy. Primary treatment used was surgery and radiotherapy of the mediastinum. The patient developed bony metastases 2 years after, and these lesions were treated with radiotherapy followed by chemotherapy. Four years after, a new mediastinal mass began to enlarge with metastases in the lungs and the bone. This recurrence was controlled by chemotherapy only. Mediastinal germ cell tumors is thought by some observers to represent related aberrations of embryologic development with subsequent malignant changes. These tumors usually occur in young men and most patients are symptomatic (dyspnea), but some others present without symptoms and are diagnosed from a routine chest X-ray. The relatively high proportion of lungs and bony metastases may reflect an increased tendency to vascular spread from the great vessels. Combination chemotherapy with cis-platinum, bleomycin and vinblastine will induce complete remission in 70% of patients with disseminated germ cell tumors. New active agents such as VP-16 and carboplatin appear to be the most active drugs next cis-platinum for these tumors.

摘要

纵隔精原细胞瘤是一种罕见的恶性生殖细胞肿瘤,在组织学上与较常见的性腺生殖细胞瘤相同。本文报道的病例说明了纵隔精原细胞瘤的典型表现及对初始治疗的反应。肿瘤位于前纵隔,表现为边界清晰的分叶状肿块。然而,该患者随后的病程并不寻常,出现了多处播散性疾病,而所有这些病变显然都通过局部放疗和化疗得到了控制。最初采用的治疗方法是纵隔手术和放疗。患者在2年后出现骨转移,这些病变先接受放疗,随后进行化疗。4年后,一个新的纵隔肿块开始增大,并伴有肺和骨转移。这次复发仅通过化疗得到了控制。一些观察者认为纵隔生殖细胞肿瘤代表胚胎发育的相关异常,随后发生恶性变化。这些肿瘤通常发生在年轻男性身上,大多数患者有症状(呼吸困难),但也有一些患者没有症状,是通过常规胸部X线检查诊断出来的。肺和骨转移的比例相对较高,可能反映了从大血管发生血管播散的倾向增加。顺铂、博来霉素和长春碱联合化疗可使70%的播散性生殖细胞肿瘤患者获得完全缓解。对于这些肿瘤,除顺铂外,诸如依托泊苷和卡铂等新型活性药物似乎是最有效的药物。

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