TSH secreting pituitary tumor — an experience of 20 years follow-up

D. Rebrova, I. Sleptsov, R. Chernikov, A. Uspenskaya, V. Rusakov, L. Krasnov, E. Fedorov, I. Sablin, M. Isheyskaya, Irina V. Olovyanishnikova, Yury N. Fedotov, A. Bubnov
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Abstract

Thyrotropinoma is a rare pituitary tumor that causes the development of thyrotoxicosis syndrome as a result of hyperproduction of thyroid stimulating hormone (TSH). In the Russian literature over the past 10 years, one case of thyrotropinoma in a child, four cases of TSH-producing pituitary adenoma in women and only one in a man have been described. The article presents a unique clinical case of a 20-years history of observation of a patient with TSH-oma. The rarity of this disease led to the fact that it took more than 10 years to make a correct diagnosis. The first operation of thyroid gland was performed before the diagnosis of pituitary adenoma and inappropriate TSH secretion syndrome. That right hemithyroidectomy was supposed to cure a toxic adenoma of thyroid gland. The diagnosis of thyrotropin-secreting piruitary tumor was established only after 6 years even after finding a combination of pituitary adenoma and thyrotoxicosis. After that, the patient steadfastly refuses neurosurgical treatment, despite the presence of macroadenoma with intrasellar growth. The therapy with somatostatin analogs led to patient’s intolerance with gastrointestinal side effects and hospitalization for acute pancreatitis. The absence of the therapy due to low compliance led to long-term persistence of thyrotoxicosis. The absence of signs and symptoms of expanding tumor mass (visual field defects, loss of vision, headache, partial or total hypopituitarism) demonstrates the slow growth of this kind of pituitary tumor. The long-term effect of elevated TSH levels led to diffuse goiter with compression of the neck organs, and the need of the surgical treatment of the thyroid. Stable euthyroidism after the operation led to stable normoglycemia in the patient with previously diagnosed diabetes mellitus type 2. This fact should keep an attention of physicians and endocrinologists to screen for the secondary reasons of hyperglycemia in a patient with diabetes mellitus manifestation. Long-term history of thyrotoxicosis led to the deleterious effects of thyroid hormone excess on the heart (atrial fibrillation, cardiomyopathy, cardiac failure). Those effects are still observed even after thyroidectomy and medical euthyroidism achievement. This fact demonstrates the importance of early diagnosis and treatment of TSH-omas.
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垂体TSH分泌瘤- 20年随访的经验
甲状腺腺瘤是一种罕见的垂体肿瘤,由于促甲状腺激素(TSH)分泌过多而导致甲状腺毒症的发展。在过去10年的俄罗斯文献中,有1例儿童甲状腺腺瘤,4例女性产生tsh的垂体腺瘤,只有1例男性被描述。文章提出了一个独特的临床病例,20年的历史观察患者的tsh -瘤。这种疾病的罕见性导致人们花了10多年的时间才做出正确的诊断。在诊断为垂体腺瘤及TSH分泌不当综合征前行首次甲状腺手术。右半甲状腺切除术是为了治疗甲状腺的毒性腺瘤。促甲状腺激素分泌垂体瘤的诊断是在发现垂体腺瘤合并甲状腺毒症6年后才确定的。此后,患者坚决拒绝神经外科治疗,尽管存在鞍内生长的大腺瘤。生长抑素类似物治疗导致患者不耐受并伴有胃肠道副作用,并因急性胰腺炎住院。由于依从性低而缺乏治疗导致甲状腺毒症长期持续存在。没有肿瘤肿物扩大的体征和症状(视野缺损、视力丧失、头痛、部分或全部垂体功能减退)表明这类垂体瘤生长缓慢。TSH水平升高的长期影响导致弥漫性甲状腺肿压迫颈部器官,需要手术治疗甲状腺。术前诊断为2型糖尿病的患者术后稳定的甲状腺功能亢进导致血糖稳定正常。这一事实应引起内科医生和内分泌学家的注意,以便在有糖尿病表现的患者中筛查高血糖的继发原因。长期甲状腺毒症史导致甲状腺激素过量对心脏的有害影响(心房颤动、心肌病、心力衰竭)。即使在甲状腺切除术和医疗促甲状腺功能实现后,这些影响仍然存在。这一事实表明早期诊断和治疗tsh瘤的重要性。
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