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[成人颅咽管瘤。三例]

[Craniopharyngioma of the adults. Three cases].

作者信息

Ben Amor A, Ach K, Harzallah L, Zaouali M, Maaroufi A, Chaieb Chadli M, Chaieb L

机构信息

Service d'Endocrinologie-Diabétologie, CHU Farhat Hached, Sousse, Tunisia.

出版信息

Ann Endocrinol (Paris). 2003 Dec;64(6):428-33.

Abstract

Craniopharyngioma, an intra- and suprasellar tumor generally observed in pediatric patients, can also occur in adults. We report three cases of histologically confirmed craniopharyngioma in three men aged 34 to 53 Years. Clinical manifestations were headache with visual and gonadic disorders associating impotence, infertiligy, and gynecomastia. Computed tomographic and magnetic resonance imaging revealed an intra- and suprasellar tumor with solid, hydric, and calcified components. Third ventricle compression was observed in two patients. Hormone test revealed gonadotrope insufficiency in two patients, associated with hyperprolactinemia in one of them, and pituitary insufficiency in the third patient. None of the patients had diabetes insipidus. The frontopterional approach was used for surgery. One patient died and the two others experienced persistent visual impairment with worsening pituitary insufficiency. Our observations suggest that these craniopharyngiomas were probably clinically latent congienital forms since endocrine features were lacking during childhood. Early diagnosis is required to achieve good outcome.

摘要

颅咽管瘤是一种通常在儿科患者中观察到的鞍内和鞍上肿瘤,也可发生于成人。我们报告了3例年龄在34至53岁男性中经组织学确诊的颅咽管瘤病例。临床表现为头痛伴视觉和性腺功能障碍,伴有阳痿、不育和男子女性型乳房。计算机断层扫描和磁共振成像显示鞍内和鞍上肿瘤,伴有实性、囊性和钙化成分。两名患者观察到第三脑室受压。激素检测显示两名患者存在促性腺激素不足,其中一名患者伴有高催乳素血症,第三名患者存在垂体功能不全。所有患者均无尿崩症。手术采用额颞叶入路。一名患者死亡,另外两名患者出现持续性视力损害,垂体功能不全加重。我们的观察结果表明,这些颅咽管瘤可能是临床上潜伏的先天性形式,因为儿童期缺乏内分泌特征。需要早期诊断以取得良好预后。

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