Kanley Amissabah M, Bossert Whitney L, Elrokhsi Salaheddin H
Department of Pediatrics, Children's Nebraska and University of Nebraska Medical Center, Omaha, NE 68104, USA.
JCEM Case Rep. 2024 Dec 12;3(1):luae225. doi: 10.1210/jcemcr/luae225. eCollection 2025 Jan.
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.
垂体巨人症(PG)是一种罕见的内分泌疾病,在儿科患者中可能表现为多种垂体激素异常。其典型表现是由于生长激素(GH)过量导致生长加速。目前的治疗方式包括手术、放疗和药物治疗。我们描述了一名14岁女孩,她因GH过量及多种其他激素异常而反复出现股骨头骨骺滑脱。脑部磁共振成像检查发现鞍区有肿物,并进行了手术切除。病理报告显示符合垂体腺瘤伴乳腺生长激素细胞增生。术后,系列实验室检查结果显示生长因子和GH水平持续升高,随访影像报告有残留肿瘤。尽管我们发现关于长效生长抑素类似物兰瑞肽治疗PG的疗效和安全性的数据有限,但在我们的患者中,总共4剂兰瑞肽成功将生长因子和GH水平降至正常范围。停用兰瑞肽5周后的重复影像显示残留肿瘤体积减小。该病例表明,对于手术干预后有残留疾病的儿科PG患者,短期使用兰瑞肽可能是一种有效的药物治疗方法。