Lymphocytic hypophysitis with central diabetes insipidus and subsequent hypopituitarism masking a suprasellar germinoma in a 13-year-old girl

Lymphocytic hypophysitis with central diabetes insipidus and subsequent hypopituitarism masking a suprasellar germinoma in a 13-year-old girl
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DOI:
10.1007/s00381-006-0078-7
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发表时间:
2006-10-01
影响因子:
1.4
通讯作者:
Eto, Yoshikatsu
Eto, Yoshikatsu
中科院分区:
医学4区
文献类型:
--
作者:
Mikami-Terao, Yoko;Akiyama, Masaharu;Eto, Yoshikatsu

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病例报告:我们报告一例13岁青春期女孩因淋巴细胞性垂体炎掩盖生殖细胞瘤而导致的中枢性尿崩症、甲状腺功能减退和随后的垂体功能减退。核磁共振显示垂体增大,垂体柄和下丘脑下部有肿块病变。脑垂体前叶开颅手术显示为活动性垂体炎,伴有淋巴细胞浸润物,但无坏死。尽管接受了强的松龙治疗,一年后还是出现了一个增大的、不规则的囊性肿块;在垂体柄和下丘脑下部,内窥镜活检发现生殖细胞瘤。结论:儿童淋巴细胞性垂体炎可能是宿主对隐匿性生殖细胞瘤的首发症状。中枢性尿崩症合并脑垂体柄增厚的诊断需要长期随访以确定潜在原因。
Case report: We report a case of central diabetes insipidus, hypothyroidism, and subsequent hypopituitarism due to lymphocytic hypophysitis masking a germinoma in a 13-year-old pubertal girl. Magnetic resonance revealed an enlarged pituitary gland and a mass lesion in the pituitary stalk and inferior hypothalamus. Open cranial surgery of the anterior pituitary showed active hypophysitis with lymphocytic infiltrates but without necrosis. Despite prednisolone therapy, 1 year later an enlarged, irregular cystic mass lesion had developed; in the pituitary stalk and inferior hypothalamus, a endoscopic biopsy revealed germinoma. Conclusion: Lymphocytic hypophysitis in children may be the first sign of a host reaction to an occult germinoma. The diagnosis of central diabetes insipidus with a thickened pituitary stalk requires long-term follow-up to establish the underlying cause.