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伴有性早熟和尿崩症的原发性鞍内混合性生殖细胞肿瘤。

Primary intrasellar mixed germ-cell tumor with precocious puberty and diabetes insipidus.

作者信息

Cho D Y, Wang Y C, Ho W L

机构信息

Department of Surgery, Taichung Veterans General Hospital, Taiwan, Republic of China.

出版信息

Childs Nerv Syst. 1997 Jan;13(1):42-6. doi: 10.1007/s003810050038.

DOI:10.1007/s003810050038
PMID:9083701
Abstract

We report an 8-year-old boy with a primary intrasellar mixed germ-cell tumor who underwent the trans-sphenoidal approach for tumor removal. Initially he suffered from diabetes insipidus. Precocious puberty and left abducens nerve palsy were also observed. Elevation of serum testosterone, beta-human chorionic gonadotropin (HCG), and alpha-fetoprotein (AFP) were found on admission. The histological study revealed mixed cellular types of tumor including germinoma, choriocarcinoma, embryonic cell carcinoma, and teratoma. Postoperative radiation to a total of 5000 cGy was performed. Adjuvant chemotherapy was administered before and after radiation. The boy was disease-free during a 6-month follow-up period. Follow-up magnetic resonance imaging showed no presence of tumor. The signs of precocious puberty disappeared, and the diabetes insipidus was easily controlled. The abducens nerve regained normal function. Serum HCG, AFP, and testosterone levels all returned to normal. Serum antidiuretic hormone increased to reach the lower limit of the normal range.

摘要

我们报告一名8岁男孩,患有原发性鞍内混合性生殖细胞肿瘤,接受了经蝶窦入路肿瘤切除术。最初,他患有尿崩症。还观察到性早熟和左侧展神经麻痹。入院时发现血清睾酮、β-人绒毛膜促性腺激素(HCG)和甲胎蛋白(AFP)升高。组织学研究显示肿瘤细胞类型混合,包括生殖细胞瘤、绒毛膜癌、胚胎细胞癌和畸胎瘤。术后进行了总量为5000厘戈瑞的放疗。放疗前后均给予辅助化疗。该男孩在6个月的随访期内无疾病复发。随访磁共振成像显示无肿瘤存在。性早熟体征消失,尿崩症易于控制。展神经恢复正常功能。血清HCG、AFP和睾酮水平均恢复正常。血清抗利尿激素升高至正常范围下限。

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