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首页> 外文期刊>Medical and Pediatric Oncology: The Official Journal of the American Association for Cancer Education >Adult primary extragonadal germ cell tumors: Treatment results and long-term follow-up.
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Adult primary extragonadal germ cell tumors: Treatment results and long-term follow-up.

机译：成人原发性腺外生殖细胞肿瘤：治疗结果和长期随访。

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BACKGROUND: Primary extragonadal germ cell tumors (PEGCT) are rare neoplasms. They have a poor prognosis, different behavior, and natural course compared to their gonadal counterparts. Both primary and salvage treatment of these tumors constitute a challenge. We retrospectively evaluated the clinicopathologic status, therapeutic implications, and outcome of our patients with PEGCT. PROCEDURE: Between 1991 and 2000, 18 patients with PEGCT (median age 31 years; range 17-63), diagnosed with tru-cut biopsy and treated with cisplatin-based chemotherapy, were evaluated in respect to treatment response and outcome. RESULTS: Cisplatin-based chemotherapy achieved a complete response rate of 27.8% and a partial response rate of 55.5%. Overall response rate was 83.3%. Only three patients were unresponsive to chemotherapy; an additional six patients with residual mass underwent surgical resection and were rendered disease-free by surgery. The 5-year actuarial event-free and overall survival were 63.4 and 71.3%, respectively. CONCLUSIONS: The outcomes of our patients with extragonadal primaries including mediastinal localization appear to be slightly better than those previously reported. Multimodality therapy is essential for these patients and given the relatively poor prognosis, prospective trials with large sample sizes, and new treatment approaches to improve outcome are required.
机译：背景：原发性腺外生殖细胞肿瘤（PEGCT）是罕见的肿瘤。与性腺疾病相比，他们的预后差，行为不同且病程自然。这些肿瘤的原发性和挽救性治疗均构成挑战。我们回顾性评估了PEGCT患者的临床病理状况，治疗意义和结果。程序：在1991年至2000年之间，对18例PEGCT患者（中位年龄31岁;范围17-63）确诊为切开活检并以顺铂为基础的化疗方案进行了治疗反应和结果评估。结果：基于顺铂的化学疗法的完全缓解率为27.8％，部分缓解率为55.5％。总体回应率为83.3％。只有三名患者对化疗无反应;另有6例残留肿物的患者接受了手术切除，并通过手术使其无病。 5年无精算事件和总生存率分别为63.4％和71.3％。结论：我们的性腺外原发患者包括纵隔定位的结果似乎比以前报道的要好。多模态治疗对于这些患者至关重要，鉴于预后相对较差，需要进行大样本量的前瞻性试验，并需要采用新的治疗方法以改善预后。

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