[An uncommon site of malignant germ cell tumor secreting alpha fetoprotein: the posterior mediastinum].

Archives francaises de pediatrie Pub Date : 1993-12-01
C Thomas, C Kalifa, D Couannet, C Bayle, A Pierre-Kahn
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Abstract

Background: While germ cell tumors generally occur in the gonads, they may also appear at other sites, from the sacrococcygeum area to the central nervous system. This report describes a case of such a tumor in the posterior mediastinum that developed intraspinally in a dumb-bell fashion.

Case report: A 2 1/2 year-old girl was admitted for abdominal tenderness, gait disturbance and fever. Clinical examination showed spastic paraparesis and bladder dysfunction. Thoracic X-rays showed a left postero-superior mediastinal mass with rib erosion. MRI showed that this mass had developed intraspinally between the intervertebral foramina and caused spinal cord compression at T4, T5, T6. The tumor was not calcified. Surgical resection via laminectomy was performed in emergency, but the T5 root had to be excised. Pathologic examination showed histologic features of yolk sac carcinoma; the serum alpha-foetoprotein was elevated (12, 400 IU/ml). The patient was given chemotherapy for 6 months and is well 2 years later.

Conclusion: Germ cell tumors may appear in unusual sites. They can be identified by measuring biological markers, and this avoid primary surgery.

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[一种罕见的分泌甲胎蛋白的恶性生殖细胞肿瘤:后纵隔]。
背景:虽然生殖细胞肿瘤通常发生在性腺,但也可能出现在其他部位,从骶尾骨区到中枢神经系统。本报告描述一例后纵隔肿瘤以哑铃方式在棘内发展。病例报告:一名两岁半女童因腹部压痛、步态障碍及发热入院。临床检查表现为痉挛性截瘫及膀胱功能障碍。胸部x光片显示左侧后上纵隔肿块伴肋骨糜烂。MRI显示该肿块在椎间孔间棘内发展,导致T4、T5、T6处脊髓受压。肿瘤未钙化。在紧急情况下通过椎板切除术进行手术切除,但必须切除T5根。病理检查显示卵黄囊癌的组织学特征;血清甲胎蛋白升高(12,400 IU/ml)。患者化疗6个月,2年后恢复健康。结论:生殖细胞瘤可出现在不同寻常的部位。它们可以通过测量生物标记来识别,这就避免了初次手术。
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