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两例因 Rathke 裂隙囊肿引起的症状性垂体炎患者接受糖皮质激素治疗:长期随访。

Two cases of symptomatic secondary hypophysitis due to Rathke's cleft cysts treated with glucocorticoids: long-term follow-up.

机构信息

Department of Endocrinology, Hematology and Gerontology, Chiba University Graduate School of Medicine, Chiba 260-8670, Japan.

Department of Molecular Diagnosis, Chiba University Graduate School of Medicine, Chiba 260-8670, Japan.

出版信息

Endocr J. 2021 Mar 28;68(3):269-279. doi: 10.1507/endocrj.EJ20-0361. Epub 2020 Oct 21.

Abstract

Rathke's cleft cyst (RCC) is a common incidental tumor in the hypothalamic-pituitary region. Some reports have shown that the clinical symptoms and endocrine functions of symptomatic RCCs are temporarily improved by glucocorticoid administration. However, it is still unknown whether glucocorticoid treatment is effective for symptomatic RCCs according to long-term observations. In this study, we describe the long-term clinical outcomes of two cases of glucocorticoid-treated biopsy-proven secondary hypophysitis caused by RCCs. We summarize the symptoms, imaging findings, and endocrine evaluations of two symptomatic RCC patients with concomitant hypophysitis before and after prednisolone treatment. In both evaluated cases, visual impairments and altered endocrine parameters were present due to chiasm and stalk compression; these outcomes improved after shrinkage of RCCs in response to prednisolone administration, and partial recovery of anterior pituitary hormone secretion was observed. However, in both cases, the deficits in anterior pituitary hormone secretion recurred, possibly due to persistent inflammatory infiltration in the RCCs and pituitary glands. After relapse of hypophysitis, anterior hormone secretion did not fully recover. In our cases of secondary hypophysitis caused by RCCs, prednisolone administration had an early effect of cyst shrinkage, followed by partial improvements in clinical symptoms and pituitary functions. However, long-term observation showed that prednisolone treatment did not contribute to complete improvement in anterior pituitary hormone dysfunction.

摘要

拉克氏囊肿(RCC)是下丘脑-垂体区域常见的偶发肿瘤。一些报道表明,在给予糖皮质激素后,有症状的 RCC 的临床症状和内分泌功能会暂时改善。然而,根据长期观察,尚不清楚糖皮质激素治疗对有症状的 RCC 是否有效。在这项研究中,我们描述了两例经活检证实的由 RCC 引起的糖皮质激素治疗的继发性垂体炎的长期临床结果。我们总结了两例伴有垂体炎的有症状 RCC 患者在泼尼松龙治疗前后的症状、影像学发现和内分泌评估。在这两个评估病例中,由于视交叉和柄受压,存在视力障碍和内分泌参数改变;这些结果在 RCC 对泼尼松龙治疗的反应缩小后得到改善,并且观察到前垂体激素分泌部分恢复。然而,在这两种情况下,前垂体激素分泌的缺陷都再次出现,可能是由于 RCC 和垂体中的持续炎症浸润。在垂体炎复发后,前激素分泌并未完全恢复。在我们的 RCC 引起的继发性垂体炎病例中,泼尼松龙给药具有囊肿缩小的早期作用,随后临床症状和垂体功能部分改善。然而,长期观察表明,泼尼松龙治疗并不能促进前垂体激素功能障碍的完全改善。

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