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Syncope as First Presentation in an Old Man with Follicle Stimulating Hormone-secreting Non-functioning Pituitary Macroadenoma: A Case Report and Review of the Literature

以暈厥表現的分泌FSH之非功能性腦垂體腺瘤:病例報告及文獻回顧

摘要


非功能性腦下垂體腺瘤(NFPA)是最常見的腦下垂體瘤。然而因症狀不明顯,臨床上要早期診斷並不容易,往往腺瘤已經壓迫到周邊組織或導致泛腦下垂體功能低下時才被察覺。大多數的NFPA是促性腺激素細胞腺瘤。本篇文章將報告一名高齡84歲男性,其腦下垂體腺瘤以少見的暈厥為初次表現,臨床上無其他局部神經系統病徵及腦下垂體激素過高的症狀。臨床上腦下垂體功能過低的表徵可能因年老而不顯著,然而血清中的血鈉偏低仍暗示著腦下垂體功能不全的存在。視野檢查發現雙顳側半盲。腦部電腦斷層及磁振造影均顯示在腦下垂體有一3.3×2.0×1.9厘米的巨大腫瘤。腦下垂體前葉的荷爾蒙分析表示,皮質醇,甲狀腺素和睾固酮濃度均有降低情形,符合次發性腦下垂體機能低下。然而,血清中FSH值上升,而LH值則否,疑似分泌FSH之NFPA。個案以內視鏡經鼻經蝶鞍腺瘤切除術(Endoscopic transnasaltranssphenoidal adenomectomy)進行腫瘤切除,病理切片具腺瘤特徵並有局部出血情形,符合pituitary apoplexy的表現,且FSH免疫染色呈陽性。術後病患接受皮質醇與甲狀腺素的補充治療,並穩定於門診追蹤。

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並列摘要


Non-functioning pituitary adenoma (NFPA) is the most prevalent type of pituitary tumor. However, it could be a diagnostic challenge due to the lack of clinical signs and symptoms. The diagnosis has mainly made by incident and frequently been delayed until macroadenoma causing mass effect or the appearance of panhypopituitarism. Most of NFPA are gonadotrophinomas. Herein we reported a pituitary macroadenoma in an 84-year-old man with unusual presentation of syncope without focal neurological signs or symptoms of pituitary hormones excess. Hyponatremia, however, was a significant clue of panhypopituitarism even when the clinical appearance could be missed by the old age. Visual field examination revealed bitemporal hemianopia. Computed tomography and magnetic resonance imaging of brain discovered a huge tumor of 3.3×2.0×1.9 cm in the pituitary gland with suprasellar extension. Hormone profile showed reduced cortisol, free T4, and testosterone concentrations, compatible with panhypopituitarism. However, serum FSH level was increased unexpectedly while the LH level was normal, suggesting FSH-secreting NFPA. Endoscopic transnasal-transsphenoidal adenomectomy was performed for tumor removal. Microscopically, the tumor cells revealed immunoreactivity to FSH staining. Focal area revealed extensive hemorrhage without necrosis, suggesting pituitary apoplexy. After the surgery, he was given glucocorticoids and thyroxine for replacement and was followed regularly under stable condition.

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