Ng Hui Yi, Namboodiri Divya, Learoyd Diana, Davidson Andrew, Champion Bernard, Preda Veronica
Department of Clinical Medicine, Level 4, Macquarie University, 2 Technology Place, Macquarie University, New South Wales, Australia.
University of Sydney, Faculty of Medicine and Health, Northern Clinical School, Reserve Road St Leonards, New South Wales, Australia.
Endocrinol Diabetes Metab Case Rep. 2019 Sep 17;2019. doi: 10.1530/EDM-19-0068.
Co-secreting thyrotropin/growth hormone (GH) pituitary adenomas are rare; their clinical presentation and long-term management are challenging. There is also a paucity of long-term data. Due to the cell of origin, these can behave as aggressive tumours. We report a case of a pituitary plurihormonal pit-1-derived macroadenoma, with overt clinical hyperthyroidism and minimal GH excess symptoms. The diagnosis was confirmed by pathology showing elevated thyroid and GH axes with failure of physiological GH suppression, elevated pituitary glycoprotein hormone alpha subunit (αGSU) and macroadenoma on imaging. Pre-operatively the patient was rendered euthyroid with carbimazole and underwent successful transphenoidal adenomectomy (TSA) with surgical cure. Histopathology displayed an elevated Ki-67 of 5.2%, necessitating long-term follow-up.
Thyrotropinomas are rare and likely under-diagnosed due to under-recognition of secondary hyperthyroidism. Thyrotropinomas and other plurihormonal pit-1-derived adenomas are more aggressive adenomas according to WHO guidelines. Co-secretion occurs in 30% of thyrotropinomas, requiring diligent investigation and long-term follow-up of complications.
促甲状腺激素/生长激素(GH)共分泌型垂体腺瘤较为罕见;其临床表现及长期管理颇具挑战性。长期数据也较为匮乏。由于起源细胞的原因,这些肿瘤可能具有侵袭性。我们报告一例源自垂体多激素Pit-1的大腺瘤病例,该患者有明显的临床甲状腺功能亢进症状,而GH过量症状轻微。病理检查证实了诊断,显示甲状腺轴和GH轴升高,生理性GH抑制失败,垂体糖蛋白激素α亚基(αGSU)升高,影像学检查显示为大腺瘤。术前患者通过卡比马唑实现甲状腺功能正常,并成功接受经蝶窦腺瘤切除术(TSA),手术治愈。组织病理学显示Ki-67升高至5.2%,因此需要长期随访。
促甲状腺激素瘤较为罕见,可能因对继发性甲状腺功能亢进认识不足而诊断不足。根据世界卫生组织指南,促甲状腺激素瘤和其他源自垂体多激素Pit-1的腺瘤是更具侵袭性的腺瘤。30%的促甲状腺激素瘤会出现共分泌现象,需要进行仔细检查并对并发症进行长期随访。