Foppiani L, Del Monte P, Ruelle A, Bandelloni R, Quilici P, Bernasconi D
Division of Endocrinology, Galliera Hospital, 16128 Genoa, Italy.
J Endocrinol Invest. 2007 Jul-Aug;30(7):603-9. doi: 10.1007/BF03346356.
TSH-secreting pituitary adenomas (TSH-omas) are a rare cause of hyperthyroidism in clinical practice. As their diagnosis is often delayed, these tumors are mostly diagnosed as macroadenomas, preventing an effective and radical cure and leading to serious local and systemic comorbidities. In addition to neurosurgery, medical therapy with the effective and tolerable SS analogs is a fundamental tool for the treatment of TSHomas. We report 3 cases of TSH-macroadenomas which displayed different clinical presentations. All patients showed increased free-thyroid hormone levels with inappropriately normal (2 patients) or high (1 patient) TSH levels. Magnetic resonance imaging (MRI)/computed tomography (CT) evidenced a pituitary macroadenoma and octreoscan was positive in all patients. In the 2 patients who underwent neurosurgery, hormonal hypersecretion by the tumor normalized. Histology showed nuclear pleomorphism and fibrosis, whereas immunohistochemistry showed positivity for TSH and, in a lesser amount, for FSH. In one of these patient (case 1), however, the presence of a tumor remnant inside the left cavernous sinus prompted us, in accordance with the patient, to start therapy with octreotide- long-acting release. As the third patient had a cardiac comorbidity which contraindicated neurosurgery, he underwent satisfactory treatment with long-acting SS analogs alone which normalized thyroid hormone levels. In this case, previous treatment with amiodarone confused and delayed the correct diagnosis of TSH-oma. As a result of improved laboratory and morphological techniques, TSH-omas should currently be diagnosed in early stages, thus enabling most patients to be managed satisfactorily through a combined approach.
促甲状腺激素分泌型垂体腺瘤(TSH瘤)是临床实践中导致甲状腺功能亢进的罕见病因。由于其诊断常常延迟,这些肿瘤大多被诊断为大腺瘤,从而无法实现有效根治,并导致严重的局部和全身合并症。除神经外科手术外,使用有效且耐受性良好的生长抑素(SS)类似物进行药物治疗是TSH瘤治疗的基本手段。我们报告了3例表现出不同临床表现的TSH大腺瘤病例。所有患者均表现为游离甲状腺激素水平升高,促甲状腺激素水平正常(2例患者)或升高(1例患者)但不恰当。磁共振成像(MRI)/计算机断层扫描(CT)证实为垂体大腺瘤,所有患者的奥曲肽扫描均呈阳性。在接受神经外科手术的2例患者中,肿瘤引起的激素分泌过多恢复正常。组织学显示核异型性和纤维化,而免疫组织化学显示促甲状腺激素呈阳性,促卵泡生成素呈弱阳性。然而,在其中1例患者(病例1)中,左侧海绵窦内存在肿瘤残余,因此我们根据患者情况,开始使用长效奥曲肽进行治疗。由于第3例患者存在心脏合并症,禁忌进行神经外科手术,因此他仅接受长效SS类似物治疗,治疗效果良好,甲状腺激素水平恢复正常。在该病例中,先前使用胺碘酮的治疗使TSH瘤的正确诊断变得复杂并延迟。由于实验室和形态学技术的改进,目前TSH瘤应在早期阶段被诊断出来,从而使大多数患者能够通过联合治疗方法得到满意的治疗。