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颅内生殖细胞瘤是一名23岁男性全垂体功能减退症的极为罕见病因。

Intracranial germinoma as a very rare cause of panhypopituitarism in a 23-year old man.

作者信息

Reisch N, Kühne-Eversmann L, Franke D, Beuschlein F, Mueller-Lisse U G, Reincke M, Seissler J

机构信息

Medizinische Klinik Innenstadt, Endocrinology/Diabetology, University Hospital Munich, Ziemssenstrasse 1, Munich, Germany.

出版信息

Exp Clin Endocrinol Diabetes. 2009 Jul;117(7):320-3. doi: 10.1055/s-0028-1100418. Epub 2008 Dec 3.

Abstract

A 23 year-old patient was referred to the endocrine outpatient clinic with the suspicion of diabetes insipidus as he complained of nycturia and polydipsia since 2-3 months. Further he presented with nausea, vomiting, loss of appetite, rapid weight loss, diffuse body pain and fatigue. No headache, blurred vision, or fever were reported. The clinical examination showed sexual infantilism (poor beard, pubic and axillary hair growth, small testis). The patient's skin was strikingly pale as well as dry and scaly. Lymph node palpation was unremarkable. Endocrine evaluation revealed diabetes insipidus as well as complete anterior pituitary insufficiency. MR imaging demonstrated contrast-enhancing mass lesions at the pineal gland, hypothalamus, and anterior horn of lateral ventricles bilaterally. The localization pattern deemed to be highly suspicious for intracranial germinoma. As beta-HCG and AFP were negative in serum and cerebrospinal fluid the diagnosis of germinoma was confirmed histologically. After radiotherapy with cranio-spinal radiation therapy with 24 Gy followed by two weeks of local tumor boost with 16 Gy, the posttherapy MRI scan indicated complete tumor removal. This case demonstrates a very rare and potentially curable tumor as the cause of panhypopituitarism in adults.

摘要

一名23岁患者因怀疑患有尿崩症被转诊至内分泌门诊,他自述2至3个月以来一直夜尿增多且烦渴。此外,他还出现恶心、呕吐、食欲不振、体重迅速减轻、全身弥漫性疼痛和疲劳。未报告有头痛、视力模糊或发热症状。临床检查发现患者有性幼稚症(胡须、阴毛和腋毛生长不良,睾丸较小)。患者的皮肤异常苍白,且干燥、有鳞屑。触诊淋巴结无异常。内分泌评估显示患者患有尿崩症以及完全性垂体前叶功能减退。磁共振成像显示松果体、下丘脑以及双侧侧脑室前角有强化的肿块病变。这种定位模式高度怀疑为颅内生殖细胞瘤。由于血清和脑脊液中的β-HCG和AFP均为阴性,因此通过组织学检查确诊为生殖细胞瘤。在进行了24 Gy的全脑全脊髓放射治疗,随后对局部肿瘤进行两周16 Gy的加量照射后,治疗后的磁共振成像扫描显示肿瘤已完全切除。该病例表明,一种非常罕见但可能治愈的肿瘤是成人全垂体功能减退的病因。

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