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青少年中表现为全垂体功能减退的拉克氏裂囊肿

Rathke Cleft Cyst Presenting as Panhypopituitarism in an Adolescent.

作者信息

Bano Tarannum, Alghamdi Najat Nasser, Altwijri Abdulrahman Jalal

机构信息

Adult Endocrinology Department, King Saud Medical City, Riyadh 12746, Kingdom of Saudi Arabia.

出版信息

JCEM Case Rep. 2025 Jun 10;3(7):luaf129. doi: 10.1210/jcemcr/luaf129. eCollection 2025 Jul.

DOI:10.1210/jcemcr/luaf129
PMID:40496472
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12150398/
Abstract

Rathke cleft cyst (RCC) is a benign lesion of the pituitary region, more commonly diagnosed in adults, in whom it typically presents with headaches, visual disturbances, or endocrine dysfunction. In pediatric patients, RCC is rare and even less frequently associated with panhypopituitarism. We report a case of a 16-year-old male patient who presented with short stature and delayed puberty. Hormonal evaluation revealed deficiencies in cortisol, thyroid hormone, gonadotropins, testosterone, and growth hormone. Magnetic resonance imaging (MRI) of the pituitary showed a 10 × 12.5 × 11.3-mm RCC displacing the pituitary stalk. Hormone replacement therapy with hydrocortisone, levothyroxine, and growth hormone was initiated. After 12 months, the patient showed a 6-cm increase in height and mild regression of cyst size. This case highlights the importance of considering RCC in the differential diagnosis of adolescents with growth failure or delayed puberty, even in the absence of typical symptoms such as headache or visual impairment. Hormone replacement therapy can be a safe and effective first-line treatment for RCC-associated hypopituitarism in the absence of severe neurological symptoms. Surgery should be reserved for cases with substantial headache or vision-related complications. Regular clinical and radiological follow-up is essential to monitor hormonal function and cyst progression.

摘要

拉克氏囊肿(RCC)是一种垂体区域的良性病变,在成人中更常被诊断出来,成人患者通常表现为头痛、视觉障碍或内分泌功能障碍。在儿科患者中,RCC很少见,与全垂体功能减退的关联甚至更少。我们报告一例16岁男性患者,其表现为身材矮小和青春期延迟。激素评估显示皮质醇、甲状腺激素、促性腺激素、睾酮和生长激素缺乏。垂体磁共振成像(MRI)显示一个10×12.5×11.3毫米的RCC,使垂体柄移位。开始使用氢化可的松、左甲状腺素和生长激素进行激素替代治疗。12个月后,患者身高增加了6厘米,囊肿大小略有缩小。该病例强调了在鉴别诊断生长发育迟缓或青春期延迟的青少年时考虑RCC的重要性,即使没有头痛或视力损害等典型症状。在没有严重神经症状的情况下,激素替代治疗可以作为RCC相关垂体功能减退的一种安全有效的一线治疗方法。对于有严重头痛或视力相关并发症的病例,应考虑手术治疗。定期进行临床和影像学随访对于监测激素功能和囊肿进展至关重要。

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本文引用的文献

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Symptomatic Rathke cleft cyst in paediatric patients - clinical presentations, surgical treatment and postoperative outcomes - an analysis of 38 cases.小儿有症状的拉克氏裂囊肿——临床表现、手术治疗及术后结果——38例分析
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Headache Resolution After Rathke Cleft Cyst Resection: A Meta-Analysis.Rathke裂囊肿切除术后头痛缓解情况:一项荟萃分析
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Presentation and outcomes in surgically and conservatively managed pediatric Rathke cleft cysts.手术治疗与保守治疗的小儿拉克氏裂囊肿的临床表现及预后
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A Case for Conservative Management: Characterizing the Natural History of Radiographically Diagnosed Rathke Cleft Cysts.保守治疗的案例:描述经影像学诊断的拉克氏裂囊肿的自然病程。
J Clin Endocrinol Metab. 2015 Oct;100(10):3943-8. doi: 10.1210/jc.2015-2604. Epub 2015 Aug 25.
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Rathke's cleft cyst.拉克氏裂囊肿
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