Cemeroglu A P, Blaivas M, Muraszko K M, Robertson P L, Vázquez D M
Eur J Pediatr. 1997 Sep;156(9):684-8. doi: 10.1007/s004310050690.
Lymphocytic hypophysitis is a rare disorder predominantly affecting females during the antepartum or postpartum period. It is characterized by destruction and lymphocytic infiltration of the pituitary gland, probably by an autoimmune process, leading to a pituitary mass lesion and/or various degrees of hypopituitarism. The lesion is usually confined to the adenohypophysis. Posterior pituitary gland or stalk involvement is rare, although patients presenting with diabetes insipidus have been reported. We describe a girl aged 13 years 9 months with lymphocytic hypophysitis who presented with diabetes insipidus and secondary amenorrhea. MRI of the brain revealed a 1 cm enhancing mass in the pituitary stalk. A biopsy of the mass by right pterional craniotomy showed lymphocytic infiltration without neoplastic cells or granuloma formation. To our knowledge, this is the youngest reported patient with a diagnosis of lymphocytic hypophysitis. In this case report, her clinical presentation is discussed along with a review of the literature.
We present the first childhood case of lymphocytic hypophysitis which is an autoimmune inflammatory disorder of the pituitary gland. Although this is a rare condition in adults, it also needs to be considered in the pediatric population. Conservative management is preferred unless there are signs of increased intracranial pressure. Most importantly, close monitoring for multiple hormone deficiencies is indicated in this condition.
淋巴细胞性垂体炎是一种罕见疾病，主要在产前或产后影响女性。其特征是垂体的破坏和淋巴细胞浸润，可能由自身免疫过程引起，导致垂体肿块病变和/或不同程度的垂体功能减退。病变通常局限于腺垂体。虽然有报道称患者出现尿崩症，但垂体后叶或垂体柄受累罕见。我们描述了一名13岁9个月患有淋巴细胞性垂体炎的女孩，她出现了尿崩症和继发性闭经。脑部MRI显示垂体柄有一个1厘米的强化肿块。通过右翼点开颅对肿块进行活检，显示有淋巴细胞浸润，无肿瘤细胞或肉芽肿形成。据我们所知，这是报道中最年轻的诊断为淋巴细胞性垂体炎的患者。在本病例报告中，我们讨论了她的临床表现并回顾了相关文献。
我们报告了首例儿童淋巴细胞性垂体炎病例，这是一种垂体的自身免疫性炎症性疾病。虽然在成人中这是一种罕见疾病，但在儿科人群中也需要考虑。除非有颅内压升高的迹象，否则首选保守治疗。最重要的是，对于这种疾病需要密切监测多种激素缺乏情况。
