JCEM case reports Pub Date : 2024-12-12 eCollection Date: 2025-01-01 DOI:10.1210/jcemcr/luae225
Amissabah M Kanley, Whitney L Bossert, Salaheddin H Elrokhsi
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摘要

垂体巨人症(PG)是一种罕见的内分泌疾病,儿童患者可能会出现多种垂体激素异常。其特征性表现是生长激素(GH)过多导致生长加速。目前的治疗方法包括手术、放射治疗和药物治疗。我们描述了一名 14 岁女孩的病例,她患有复发性股骨头骺滑脱,并伴有 GH 过多和其他多种激素异常。脑部磁共振成像检查发现了一个髓质肿块,并进行了手术切除。病理报告显示其为垂体腺瘤伴乳腺增生。术后,连续化验结果显示生长因子和 GH 水平持续升高,随访造影报告显示肿瘤残留。尽管我们发现有关长效体泌素类似物兰瑞奥肽治疗PG的疗效和安全性的数据有限,但兰瑞奥肽在我们的患者身上总共使用了4个剂量,成功地将生长因子和GH水平降至正常范围。停用兰瑞奥肽 5 周后的复查显示,残余肿瘤体积缩小。本病例表明,对于手术治疗后有残余病灶的儿科PG患者,短期服用兰瑞奥肽可作为一种有效的药物治疗方法。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Pituitary Gigantism in an Adolescent Girl With Postsurgical Residual Disease Treated With Lanreotide.

Pituitary gigantism (PG) is a rare endocrine disorder that may present with multiple pituitary hormone abnormalities in pediatric patients. A hallmark presentation is accelerated growth due to growth hormone (GH) excess. Current treatment modalities include surgery, radiation, and medical therapy. We describe a 14-year-old girl who presented with recurrent slipped capital femoral epiphysis with GH excess and multiple other hormonal abnormalities. A sellar mass was identified on magnetic resonance imaging of the brain and was surgically resected. The pathology report was consistent with pituitary gland adenoma with mammosomatotrophs hyperplasia. Post surgery, serial laboratory results showed persistently elevated growth factor and GH levels, and residual tumor was reported on follow-up imaging. Even though we found limited data on the efficacy and safety of a long-acting somatostatin analogue, lanreotide, in the treatment of PG, a total of 4 doses of lanreotide successfully reduced growth factor and GH levels to normal ranges in our patient. Repeat imaging 5 weeks post discontinuation of lanreotide showed reduction of residual tumor volume. This case reveals that a short course of lanreotide may be used as an effective medical treatment in pediatric patients with PG who have residual disease after surgical intervention.

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