Vilar Helena, Nobre E Lacerda, Jorge Zulmira, Filipe J Sousa, Castro J Jácome de
Serviço de Endocrinologia, Diabetes e Metabolismo e de Neurocirurgia, Hospital Militar Principal, Lisboa.
Acta Med Port. 2005 Jul-Aug;18(4):309-13. Epub 2005 Sep 16.
Sarcoidosis is a granulomatous disease of unknown etiology that can affect all organs and systems, including the central nervous system in approximately 5% of patients. Although the hypothalamo-hypophyseal system may be one of the areas affected by neurosarcoidosis, pseudotumoral lesions are very rare. This paper reports the case of a 42-year-old male Caucasian patient followed by the Pneumology and Dermatology services for pulmonary and cutaneous sarcoidosis. Corticotherapy was initiated five months from diagnosis and the patient showed clear clinical and imagiological signs of improvement. In the following two months, the patient experienced visual disturbances and a slight decrease in libido, but denied having other symptoms associated with hypothalamo-hypophyseal hypofunction. Laboratorial evaluation (basal determinations and stimulation tests) revealed panhypopituitarism. The EC-NMR showed a bulky intrasellar lesion extending into the suprasellar and right parasellar regions, with evidence of optic chiasm compression, erosion of the floor of the sella and cavernous sinus invasion. Campimetry showed a bitemporal hemianopsia. For the purposes of chiasmatic decompression and histological study of the lesion the patient underwent a right frontal craniotomy, with a subtotal resection of the hypophyseal lesion. The histological diagnosis revealed a pituitary adenoma with no immunohistochemical expression. The patient began radiotherapy six months after surgery. Although today's diagnostic tools allow for a high degree of accuracy, hypophyseal lesions still present problems to the endocrinologist as their identification is essential for determining the appropriate treatment. At times the final diagnosis is only achieved after a histological identification of the lesion (biopsy/surgery). It should be noted that the described cases of association between sarcoidosis and hypophyseal adenoma are very rare, making the differential diagnosis with neurosarcoidosis difficult--a condition which has a preferential medical approach and a different prognosis.
结节病是一种病因不明的肉芽肿性疾病,可累及所有器官和系统,约5%的患者会影响中枢神经系统。尽管下丘脑 - 垂体系统可能是神经结节病受累的区域之一,但假瘤性病变非常罕见。本文报告了一名42岁的白人男性患者,因肺部和皮肤结节病在呼吸科和皮肤科就诊。确诊后五个月开始进行皮质激素治疗,患者出现了明显的临床和影像学改善迹象。在接下来的两个月里,患者出现视觉障碍和性欲略有下降,但否认有其他与下丘脑 - 垂体功能减退相关的症状。实验室评估(基础测定和刺激试验)显示全垂体功能减退。增强磁共振成像显示鞍内有一个巨大病变,延伸至鞍上和右侧鞍旁区域,有视交叉受压、蝶鞍底部侵蚀和海绵窦侵犯的迹象。视野检查显示双颞侧偏盲。为了进行视交叉减压和病变的组织学研究,患者接受了右额开颅手术,垂体病变次全切除。组织学诊断为无免疫组化表达的垂体腺瘤。患者术后六个月开始放疗。尽管如今的诊断工具具有很高的准确性,但垂体病变对内分泌学家来说仍然存在问题,因为其识别对于确定适当的治疗至关重要。有时只有在对病变进行组织学鉴定(活检/手术)后才能做出最终诊断。需要注意的是,结节病与垂体腺瘤相关的病例非常罕见,这使得与神经结节病的鉴别诊断变得困难——神经结节病有其优先的治疗方法和不同的预后。