Primary malignant germ cell tumours

M. Mlika, A. Ayadi-Kaddour, L. Kochbati, S. Hantous-Zannad, F. Mezni
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Abstract

Study objectives : the aim of this enquiry was to perform a retrospective review on patients treated for primary malignant mediastinal germ cell tumours (PMMGCT) between 1992-2008, to specify their clinical characteristics, their prognostic factors and to evaluate the current therapeutic strategies.Patients: we report a study about 8 patients with seminoma in 3 cases, yolk sac tumour (YST) in 2 cases, embryonal carcinoma (EC) in 1 case, choriocarcinoma in 1 case and teratocarcinoma in 1 case. Pathologic diagnoses were confirmed in all cases by surgical biopsy and a resection of the residual mass in 2 cases.Results: all the patients were male with a mean age of 32 years (range, 16 to 63 years). Only one patient was asymptomatic. The most frequent symptoms were cough (87.5%), chest pain (50%) and dyspnea (50%). The 8 tumours were located in the anterior mediastinum. One patient with EC didn’t received any treatment because of the rapid evolution of the disease. The 7 others received pre-operative chemotherapy and 3 patients received a second-line chemotherapy because of the persistence of viable tumours in one case (YST) and a resistance to the first chemotherapy in 2 cases (seminoma and teratocarcinoma). The overall 2-year survival of patients with seminoma was 100% towards 25% for those with nonseminomatous tumours. The patients with satge I disease survived for a mean period of 3 years.Conclusion: The PMMGCT cause generally a diagnostic challenge because of the difficulty of their resection and the little size of the biopsy specimen. Besides, the absence of a primary gonadal localization may be difficult to establish. These tumours have a worse prognosis than their gonadal counterparts. Prognostic factors are multiple and not accurate. The most relevant ones seem to be the histological type and the disease stage.
原发性恶性生殖细胞肿瘤
研究目的:本研究的目的是对1992-2008年间接受原发性恶性纵隔生殖细胞肿瘤(PMMGCT)治疗的患者进行回顾性研究,以明确其临床特征、预后因素并评估当前的治疗策略。患者:我们报告了8例精原细胞瘤3例,卵黄囊瘤2例,胚胎癌1例,绒毛膜癌1例,畸胎瘤1例。所有病例均经手术活检证实病理诊断,2例行残留肿块切除。结果:所有患者均为男性,平均年龄32岁(16 ~ 63岁)。只有一名患者无症状。最常见的症状是咳嗽(87.5%)、胸痛(50%)和呼吸困难(50%)。8例肿瘤位于前纵隔。一名患有EC的患者没有接受任何治疗,因为疾病的快速演变。其他7例患者接受术前化疗,3例患者接受二线化疗,因为1例(YST)存活肿瘤持续存在,2例(精原细胞瘤和畸胎瘤)对第一次化疗有耐药性。精原细胞瘤患者的总体2年生存率为100%,而非精原细胞瘤患者的总体2年生存率为25%。I期患者平均存活时间为3年。结论:PMMGCT由于切除困难和活检标本体积小,通常给诊断带来挑战。此外,缺乏原发性性腺定位可能难以确定。这些肿瘤的预后比性腺肿瘤差。预后因素多种多样,并不准确。最相关的似乎是组织学类型和疾病分期。
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