Lymphocytic hypophysitis masking a suprasellar germinoma in a 12-year-old girl--a case report.
Lymphocytic hypophysitis masking a suprasellar germinoma in a 12-year-old girl--a case report.
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DOI：
10.1023/a:1009923029942
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发表时间：
1999-05-01
期刊：
影响因子：
3.8
通讯作者：
Saeger, W
中科院分区：
文献类型：
--
作者：
Fehn, M;Bettendorf, M;Saeger, W
本文报告1例12岁女孩合并鞍上性生殖细胞瘤的淋巴细胞性垂体炎的病史、光镜和电子显微镜表现。女孩表现为尿崩症和随后的全垂体功能减退症的长期病史。在诊断为尿崩症两年后，磁共振成像(MRI)显示鞍区内容物和垂体柄肿瘤性增大。最初经鼻腔探查发现淋巴细胞性垂体炎。光镜下可见成熟淋巴细胞和浆细胞密集分布于垂体前叶间质。不能暴露柄区域以排除生殖细胞瘤。一年后，尽管地塞米松治疗，病变复发，另一位神经外科医生不得不进行第二次手术。光镜下可见淋巴细胞渗出、纤维化、坏死。诊断结果又是淋巴细胞性垂体炎。虽然经颅骨暴露，但仅切除了脑下垂体组织。MRI显示手术中未见漏斗状肿块。五个月后，女孩出现了多发性脑部病变，显示为生殖细胞瘤。淋巴细胞性垂体炎在儿童中非常罕见，到目前为止，还没有从组织病理学的角度描述与生殖细胞瘤重合的情况。并对垂体浸润的来源进行了讨论。
Case history, light and electron microscopic findings of a case of a lymphocytic hypophysitis in coincidence with a suprasellar germinoma in a 12-year-old girl are reported. The girl presented with a long time case history of diabetes insipidus and subsequent panhypopituitarism. Two years after the diagnosis of diabetes insipidus magnetic resonance imaging (MRI) showed a tumorous enlargement of the sellar content and pituitary stalk. A transnasal exploration was initially performed and revealed a lymphocytic hypophysitis. Light microscopy showed a dense infiltration of mature lymphocytes and plasma cells in the interstitium of the anterior pituitary gland. The stalk area could not be exposed to exclude a germinoma. One year later the lesion relapsed despite dexamethason therapy and a second operation by another neurosurgeon had to be performed. Light microscopy showed lymphocytic infiltrates, fibrosis and necrosis. The diagnosis was a lymphocytic hypophysitis again. Though transcranially exposed only pituitary tissue was removed. No infundibular mass became visible at surgery as shown by MRI. The girl developed five months later multiple cerebral lesions, which revealed to be a germinoma. Lymphocytic hypophysitis in children is very rare and a coincidence with a germinoma has not been described from histopathological aspect until now. The origin of the pituitary infiltration is discussed.