Thomas C, Kalifa C, Couannet D, Bayle C, Pierre-Kahn A
Services de Pédiatrie, de Radiologie et d'Anatomo-pathologie, Institut Gustave-Roussy, Hôpital Necker-Enfants malades, Paris.
Arch Fr Pediatr. 1993 Dec;50(10):879-81.
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.
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.
Germ cell tumors may appear in unusual sites. They can be identified by measuring biological markers, and this avoid primary surgery.
生殖细胞肿瘤通常发生于性腺,但也可能出现在其他部位,从骶尾部到中枢神经系统。本报告描述了一例后纵隔此类肿瘤以哑铃状方式向椎管内发展的病例。
一名2岁半女孩因腹部压痛、步态障碍和发热入院。临床检查显示痉挛性截瘫和膀胱功能障碍。胸部X线显示左后上纵隔肿块伴肋骨侵蚀。MRI显示该肿块在椎间孔之间向椎管内发展,并在T4、T5、T6水平导致脊髓受压。肿瘤未钙化。急诊行椎板切除术进行手术切除,但不得不切除T5神经根。病理检查显示为卵黄囊癌的组织学特征;血清甲胎蛋白升高(12400IU/ml)。患者接受了6个月的化疗,2年后情况良好。
生殖细胞肿瘤可能出现在不寻常的部位。可通过检测生物标志物来识别,从而避免初次手术。