促甲状腺激素分泌型垂体神经内分泌肿瘤(TSH PitNET)的临床特征、诊断和治疗:单中心经验。

IF 3.9 3区 医学 Q2 ENDOCRINOLOGY & METABOLISM Endocrinology and Metabolism Pub Date : 2024-04-01 Epub Date: 2024-02-05 DOI:10.3803/EnM.2023.1877
Jung Heo, Yeon-Lim Suh, Se Hoon Kim, Doo-Sik Kong, Do-Hyun Nam, Won-Jae Lee, Sung Tae Kim, Sang Duk Hong, Sujin Ryu, You-Bin Lee, Gyuri Kim, Sang-Man Jin, Jae Hyeon Kim, Kyu Yeon Hur
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引用次数: 0

摘要

背景:分泌促甲状腺激素(TSH)的垂体神经内分泌瘤(TSH PitNET)是垂体神经内分泌瘤的一种罕见亚型。我们调查了一家医疗中心的 TSH PitNET 病例的综合特征和预后。此外,我们还对诊断方法进行了比较,以确定哪种诊断方法具有更高的灵敏度:方法:我们对三星医疗中心 2002 年至 2022 年期间手术后确诊为 TSH PitNET 的 17 例患者进行了回顾性研究。收集了有关综合特征和治疗结果的数据。比较了各种诊断方法的敏感性:7名患者为男性(41%),诊断时的中位年龄为42岁(21至65岁);中位随访时间为37.4个月。最常见(59%)的初始症状是甲状腺功能亢进相关症状。有四名患者(23%)出现激素合并分泌。血清α-亚基(α-SU)升高的诊断敏感性最高(91%),其次是促甲状腺激素释放激素(TRH)刺激反应迟钝(80%)和性激素结合球蛋白升高(63%)。14名患者(82%)患有大腺瘤,其中一名患者的标本有严重钙化,但促甲状腺激素检测呈阴性。在随访超过 6 个月的 15 名患者中,有 10 人(67%)在术后 6 个月内实现了激素和结构缓解。此外,还发现了一例生长激素(GH)/促甲状腺激素(TSH)/泌乳素(PRL)共同分泌的混合性神经节细胞瘤-垂体腺瘤(MGPA):结论:大多数TSH PitNET病例为大腺瘤,23%显示激素联合分泌。发现了一例罕见的GH/TSH/PRL联合分泌的MGPA。血清α-SU和TRH刺激试验显示出极高的诊断敏感性。在诊断 TSH PitNET 时需要慎重考虑。要想获得缓解,必须彻底切除肿瘤。如果不能缓解,放射治疗或药物治疗可提高长期缓解率。
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Clinical Characteristics, Diagnosis, and Treatment of Thyroid Stimulating Hormone-Secreting Pituitary Neuroendocrine Tumor (TSH PitNET): A Single-Center Experience.

Backgruound: Thyroid-stimulating hormone (TSH)-secreting pituitary neuroendocrine tumor (TSH PitNET) is a rare subtype of PitNET. We investigated the comprehensive characteristics and outcomes of TSH PitNET cases from a single medical center. Also, we compared diagnostic methods to determine which showed superior sensitivity.

Methods: A total of 17 patients diagnosed with TSH PitNET after surgery between 2002 and 2022 in Samsung Medical Center was retrospectively reviewed. Data on comprehensive characteristics and treatment outcomes were collected. The sensitivities of diagnostic methods were compared.

Results: Seven were male (41%), and the median age at diagnosis was 42 years (range, 21 to 65); the median follow-up duration was 37.4 months. The most common (59%) initial presentation was hyperthyroidism-related symptoms. Hormonal co-secretion was present in four (23%) patients. Elevated serum alpha-subunit (α-SU) showed the greatest diagnostic sensitivity (91%), followed by blunted response at thyrotropin-releasing hormone (TRH) stimulation (80%) and elevated sex hormone binding globulin (63%). Fourteen (82%) patients had macroadenoma, and a specimen of one patient with heavy calcification was negative for TSH. Among 15 patients who were followed up for more than 6 months, 10 (67%) achieved hormonal and structural remission within 6 months postoperatively. A case of growth hormone (GH)/TSH/prolactin (PRL) co-secreting mixed gangliocytoma-pituitary adenoma (MGPA) was discovered.

Conclusion: The majority of the TSH PitNET cases was macroadenoma, and 23% showed hormone co-secretion. A rare case of GH/TSH/PRL co-secreting MGPA was discovered. Serum α-SU and TRH stimulation tests showed great diagnostic sensitivity. Careful consideration is needed in diagnosing TSH PitNET. Achieving remission requires complete tumor resection. In case of nonremission, radiotherapy or medical therapy can improve the long-term remission rate.

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来源期刊
Endocrinology and Metabolism
Endocrinology and Metabolism Medicine-Endocrinology, Diabetes and Metabolism
CiteScore
6.60
自引率
5.90%
发文量
145
审稿时长
24 weeks
期刊介绍: The aim of this journal is to set high standards of medical care by providing a forum for discussion for basic, clinical, and translational researchers and clinicians on new findings in the fields of endocrinology and metabolism. Endocrinology and Metabolism reports new findings and developments in all aspects of endocrinology and metabolism. The topics covered by this journal include bone and mineral metabolism, cytokines, developmental endocrinology, diagnostic endocrinology, endocrine research, dyslipidemia, endocrine regulation, genetic endocrinology, growth factors, hormone receptors, hormone action and regulation, management of endocrine diseases, clinical trials, epidemiology, molecular endocrinology, neuroendocrinology, neuropeptides, neurotransmitters, obesity, pediatric endocrinology, reproductive endocrinology, signal transduction, the anatomy and physiology of endocrine organs (i.e., the pituitary, thyroid, parathyroid, and adrenal glands, and the gonads), and endocrine diseases (diabetes, nutrition, osteoporosis, etc.).
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