Bestepe N, Aydin C, Tam A A, Ercan K, Ersoy R, Cakir B
Ankara City Hospital, Dept. of Endocrinology and Metabolism, Ankara, Turkey.
"Yildirim Beyazit" University, Faculty of Medicine, Dept. of Endocrinology and Metabolism, Ankara, Turkey.
Acta Endocrinol (Buchar). 2022 Jan-Mar;18(1):97-101. doi: 10.4183/aeb.2022.97.
Acromegaly is an acquired disorder related to excessive production of growth hormone (GH) and insulin-like growth factor-1 (IGF-1). Empty sella (ES) is an anatomical condition of sella turcica that is partially or completely filled with cerebrospinal fluid mainly due to intrasellar herniation of subarachnoid space. Here, we describe a patient who presented with clinical and biochemical features of acromegaly and who had an ES on pituitary magnetic resonance imaging (MRI).
A 73-year-old male patient was consulted in our clinic because of the acromegalic phenotype while planning for colorectal adenocarcinoma surgery. The patient noticed gradual enlarging of his hands, feet and nose for 30 years, but never consulted to any clinician for this reason. Serum GH was 20.6 ng/mL (normal <3 ng/mL) and IGF-1 was 531 ng/mL (normal, 69-200 ng/ml). An oral glucose tolerance test showed no suppression of GH values. T1-weighted MRI revealed an ES. 18F-FDG PET/CT and Ga-DOTATADE PET/CT did not show any finding consistent with ectopic GH secretion. Growth hormone releasing hormone (GHRH) was within the normal range (<100mg/dL). He was treated with long-acting octreotide 20 mg per 28 days. At the 6 month of treatment, serum GH and IGF-1 levels were decreased to 5.45 ng/mL and 274 ng/mL, respectively.
The mechanism underlying the association of acromegaly and ES remains unclear. Apoplexy on existing pituitary adenoma and then formation of necrosis can proceed to ES. Since our patient did not have a history of pituitary apoplexy and we could not find any reason for secondary ES, we considered primary ES.
肢端肥大症是一种与生长激素（GH）和胰岛素样生长因子-1（IGF-1）过度分泌相关的后天性疾病。空蝶鞍（ES）是蝶鞍的一种解剖学状态，主要由于蛛网膜下腔向鞍内疝入，蝶鞍部分或完全被脑脊液填充。在此，我们描述一名患者，其具有肢端肥大症的临床和生化特征，且垂体磁共振成像（MRI）显示有空蝶鞍。
一名73岁男性患者在计划进行结肠直肠腺癌手术时，因肢端肥大症表型前来我院就诊。患者注意到其手部、足部和鼻子逐渐增大已有30年，但从未因此咨询过任何临床医生。血清GH为20.6 ng/mL（正常<3 ng/mL），IGF-1为531 ng/mL（正常，69 - 200 ng/ml）。口服葡萄糖耐量试验显示GH值未被抑制。T1加权MRI显示有空蝶鞍。18F-FDG PET/CT和Ga-DOTATADE PET/CT未显示任何与异位GH分泌一致的发现。生长激素释放激素（GHRH）在正常范围内（<100mg/dL）。他接受了每28天20 mg长效奥曲肽的治疗。在治疗6个月时，血清GH和IGF-1水平分别降至5.45 ng/mL和274 ng/mL。
肢端肥大症与空蝶鞍关联的潜在机制仍不清楚。现有的垂体腺瘤发生卒中，随后形成坏死，可发展为空蝶鞍。由于我们的患者没有垂体卒中病史，且我们找不到继发性空蝶鞍的任何原因，我们考虑为空蝶鞍综合征。
