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垂体大腺瘤卒中:12个月内8例患者发病。

Apoplexy in pituitary macroadenoma: eight patients presenting in 12 months.

作者信息

Elsässer Imboden Petra Nadja, De Tribolet Nicolas, Lobrinus Alexander, Gaillard Rolf C, Portmann Luc, Pralong François, Gomez Fulgencio

机构信息

From Service of Endocrinology, Diabetology and Metabolism, Department of Internal Medicine (PNEI, RCG, LP, FP, FG), Department of Neurosurgery (NDT), and Department of Pathology (AL), University Hospital, Lausanne, Switzerland.

出版信息

Medicine (Baltimore). 2005 May;84(3):188-196. doi: 10.1097/01.md.0000164205.77287.9e.

Abstract

Pituitary apoplexy is an ill-defined clinical entity. Some authors include hypoxic pituitary infarction, even in the absence of tumor after hemorrhagic delivery, whereas others apply this term strictly to hemorrhage within a pituitary adenoma. We conducted the present study to establish the prevalence, clinical characteristics, and outcome of pituitary apoplexy, defined as an endocrine crisis characterized by acute intense headache, with or without altered consciousness, rapid development of visual or motor ocular disorders, and pituitary failure, associated with a large pituitary adenoma. We describe 8 consecutive patients (1 woman and 7 men, aged 29-66 yr) presenting over 12 months with pituitary apoplexy. We reviewed patient charts for symptoms, imaging characteristics, hormonal data, management, pathologic findings, and outcome. We examined our pituitary tumors database for cases of macroadenoma without apoplexy occurring during the same period. In 5 patients, potential precipitating factors were present. In 6 patients (3 nonsecreting tumors, 1 free-alpha-subunit-secreting tumor, 1 growth hormone and prolactin-secreting tumor with acromegaly, and 1 prolactinoma), no pituitary disease was suspected before the acute event, representing 19% of newly diagnosed pituitary macroadenomas during the same period of time, a higher proportion than expected from our previously published series. The 2 other patients had known pituitary macroadenomas, a nonsecreting tumor and a prolactinoma on dopamine agonist therapy. Pituitary insufficiency at diagnosis included adrenal failure in 4 patients. Transsphenoidal tumor removal was performed 3-9 days after the onset of symptoms (mean, 5.3 d) in 7 of the 8 patients. Pathologic analysis disclosed tumor hemorrhage in 4 cases, ischemic necrosis in 2, and ischemia after intrasellar hemorrhage in 1. Preoperative magnetic resonance imaging was more sensitive than computed tomography for identifying hemorrhage. The newly diagnosed prolactinoma was treated with dopamine agonist. Complete neuro-ophthalmic recovery was observed in all cases, but only 2 patients displayed normal pituitary function on follow-up. The other 6 patients required long-term hormone replacement therapy. These data show that early surgical decompression prevents persistent neuro-ophthalmic deficit, but does not prevent persistent pituitary insufficiency. Moreover, published data indicate that the efficacy of surgery for the relief of neuro-ophthalmic symptoms decreases with increasing syndrome duration. Our data confirm that apoplexy occurs most often as the inaugural manifestation of pituitary macroadenoma, and suggest a recent increase of cases of apoplexy in our area.

摘要

垂体卒中是一种定义不明确的临床病症。一些作者将缺氧性垂体梗死包括在内,即使在产后出血且无肿瘤的情况下,而另一些作者则严格将该术语用于垂体腺瘤内的出血。我们开展本研究以确定垂体卒中的患病率、临床特征及转归,垂体卒中定义为一种内分泌危象,其特征为急性剧烈头痛,伴或不伴有意识改变、视力或动眼功能障碍迅速发展以及垂体功能减退,并与大型垂体腺瘤相关。我们描述了连续8例(1例女性和7例男性,年龄29 - 66岁)在12个月内出现垂体卒中的患者。我们查阅了患者病历以获取症状、影像学特征、激素数据、治疗、病理结果及转归信息。我们检查了我们的垂体肿瘤数据库,以查找同期未发生卒中的大腺瘤病例。5例患者存在潜在的诱发因素。6例患者(3例无功能性肿瘤、1例分泌游离α亚基的肿瘤、1例合并肢端肥大症的分泌生长激素和催乳素的肿瘤以及1例催乳素瘤)在急性事件发生前未被怀疑患有垂体疾病,占同期新诊断垂体大腺瘤的19%,这一比例高于我们之前发表系列中的预期。另外2例患者已知患有垂体大腺瘤,分别为1例无功能性肿瘤和1例接受多巴胺激动剂治疗的催乳素瘤。诊断时垂体功能减退包括4例患者出现肾上腺功能衰竭。8例患者中的7例在症状出现后3 - 9天(平均5.3天)进行了经蝶窦肿瘤切除术。病理分析显示4例肿瘤出血,2例缺血性坏死,1例鞍内出血后缺血。术前磁共振成像在识别出血方面比计算机断层扫描更敏感。新诊断的催乳素瘤用多巴胺激动剂治疗。所有病例均观察到神经眼科症状完全恢复,但随访时只有2例患者垂体功能正常。其他6例患者需要长期激素替代治疗。这些数据表明早期手术减压可预防持续性神经眼科缺陷,但不能预防持续性垂体功能减退。此外,已发表的数据表明,手术缓解神经眼科症状的疗效随综合征持续时间延长而降低。我们的数据证实卒中最常作为垂体大腺瘤的首发表现出现,并提示我们所在地区近期垂体卒中病例有所增加。

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