7岁甲状腺促性腺激素分泌垂体腺瘤患儿管理的挑战及文献综述。
Challenges in the management of a 7 years old child with thyrotropin-secreting pituitary adenoma and the review of the literature.
发表日期:2023 Jan 11
作者:
Tarık Kirkgöz, Saygin Abali, Askin Seker, Busra Gurpinar Tosun, Mehmet Eltan, Didem Helvacioglu, Belma Haliloglu, Sare Betul Kaygusuz, Zehra Yavas Abali, Tuba Seven Menevse, Suheyla Bozkurt, Tunc Ones, Tulay Guran, Adnan Dagcinar, Abdullah Bereket, Serap Turan
来源:
Hormone Research in Paediatrics
摘要:
甲状腺刺激素瘤(TSHoma)是一种非常罕见的疾病,占垂体肿瘤的不到1%,表现为甲状腺激素升高和正常/高TSH浓度。一名7岁的男孩因神经紧张而被他的精神科医生转诊,血液检查显示游离T4,T3和TSH水平升高。初步评估显示有α-亚单位升高。垂体MRI显示有一个巨型腺瘤。患者接受了经蝶鞍切除肿瘤的手术,肿瘤组织内显示TSH,生长激素和催乳素的免疫组化染色呈阳性反应。手术后一年,达到了甲状腺功能正常状态。之后,由于肿瘤复发,TSH和甲状腺激素水平升高,患者接受了第二次经蝶鞍切除肿瘤手术,并随后接受了伽马刀射线手术。这次,患者达到了甲状腺功能正常状态,维持了2.5年。但由于再次发作,他开始接受卡贝哌醇和奥曲肽的药物治疗,可持续维持正常甲状腺功能4年。复查MRI未发现垂体肿瘤,但在蝶窦发现了一个肿瘤。手术切除该肿瘤并未达到正常甲状腺功能状态。68Ga-DOTA-TATE PET / CT显示仍有残留组织从垂体区域延伸到蝶窦。患者在诊断时骨龄已经提前2年,并在诊断后的一年内增加了4年,随访期间仍保持不变,导致他在16岁时的最终身高比目标身高低3.3 SDS。TSHoma的诊断,治疗和随访具有挑战性,加速骨骼成熟导致的矮小是儿科TSHoma的并发症。 S. Karger AG,巴塞尔。
Thyrotropin-producing pituitary adenoma (TSHoma) is a very rare disease, representing less than 1% of the pituitary tumours, present with elevated thyroid hormones and normal/high TSH concentrations.A 7-year-old boy with nervousness was referred by his psychiatrist for elevated free T4, T3 and TSH levels. Initial evaluation revealed an elevated -subunit.Pituitary MRI demonstrated a macroadenoma. The patient underwent a trans-sphenoidal tumour resection (TSS) which showed positive immunohistochemical staining for TSH, growth hormone, and prolactin in tumoral tissue. Euthyroidism was achieved for one year after TSS, then, recurrence of tumour with elevated TSH and thyroid hormone levels necessitated a re-operation with TSS followed by gamma-knife radiosurgery. The euthyroid state was achieved and lasted for 2.5 years this time, but, due to the recurrence, medical treatment had been commenced with cabergoline and octreotide. Euthyroidism was maintained for the last 4 years on monthly octreotide treatment. A repeat MRI demonstrated no pituitary mass but a mass in the sphenoidal sinus had been detected. Removal of this mass by surgery did not achieve euthyroidism. 68Ga-DOTA-TATE PET/CT showed residual tissue extending from the pituitary region to the sphenoid sinus. The patient's bone age was advanced 2 years at diagnosis which became 4 years in one year after the diagnosis and remained so throughout follow-up, leading to a final height of -3.3 SDS below his target height at the age of 16 years.The diagnosis, treatment, and follow-up of TSHomas are challenging and short stature due to accelerated bone maturation is a complication of paediatric TSHomas.S. Karger AG, Basel.