Hira M, Caron F, Goujon J M, Lambard D, Martigne C, Patte F
Service de Pneumologie, CHU Poitiers.
Rev Pneumol Clin. 1994;50(6):338-41.
A young patient presented with a rare tumour of the mediastinum. The first sign was liquid effusion in the right hemithorax. A major rise in alpha-foeto-protein level to 34,000 ng/ml rapidly led to diagnosis. Histological confirmation was obtained on a surgical biopsy specimen of the lung. Management included primary polychemotherapy followed by complete surgical exeresis. A second post-operative cycle of chemotherapy was given but only led to temporary normalization of alpha-foeto-protein levels and intensive chemotherapy followed by autograft to peripheral stem cells was performed. The vitellin tumour is a primary germ cell tumour usually observed in young men. It originates in extraembryonary primary mesenchymal tissue. Like other germ cell tumours, the diagnosis should be entertained for all tumours of the anterior mediastinum. Tumour markers useful for diagnosis and prognosis are beta-HCG, AFP and CEA. Levels above 500 ng/ml confirm vitellin tumour. Chemotherapy should be instituted rapidly using a BEP protocol (bleomycine, etoposide, cisplatinium) or a PVB protocol (cisplatinium, vinblastin, bleomycine). Using these new drugs and current therapeutic protocols, the rate of complete remission has risen from 13% to 40%.
一名年轻患者出现了罕见的纵隔肿瘤。首个症状是右半胸出现胸腔积液。甲胎蛋白水平大幅升至34,000 ng/ml，迅速促成了诊断。通过肺部手术活检标本获得了组织学确诊。治疗包括先行多药化疗，随后进行完整的手术切除。术后进行了第二个化疗周期，但仅使甲胎蛋白水平暂时恢复正常，之后进行了强化化疗并给予外周干细胞自体移植。卵黄囊瘤是一种原发性生殖细胞肿瘤，通常见于年轻男性。它起源于胚胎外原始间充质组织。与其他生殖细胞肿瘤一样，对于所有前纵隔肿瘤都应考虑该诊断。对诊断和预后有用的肿瘤标志物是β-HCG、AFP和CEA。水平高于500 ng/ml可确诊卵黄囊瘤。应迅速采用BEP方案（博来霉素、依托泊苷、顺铂）或PVB方案（顺铂、长春花碱、博来霉素）进行化疗。使用这些新药和当前的治疗方案，完全缓解率已从13%提高到40%。
