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Prolactinoma

内分泌

关键词内分泌 疾病 催乳素分泌过度

词汇介绍

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解析

Prolactinoma   英/prəu'læktinəmə/

释    义   n. 泌乳素瘤

例    句   Objective To investigate the clinical characteristics, therapies and prognosis of male adolescent patients with prolactinoma. 目的探讨男性青少年催乳素瘤的临床特点、治疗与预后。

概述

概述


催乳素瘤是大脑脑垂体的非癌性肿瘤(腺瘤)过度分泌激素催乳素的疾病。主要作用是降低某些性激素的水平,即女性的雌激素和男性的睾丸激素。尽管泌乳素瘤不会危及生命,但会损害视力,引起不育并产生其他影响。催乳素瘤是最常见的激素分泌型肿瘤,可在垂体中发展。医生通常可以用药物治疗催乳素,使催乳素水平恢复正常,手术切除垂体瘤也是一种选择。


临床表现


绝经前妇女的催乳素瘤症状:①催乳素的少量增加会导致月经不调或月经完全消失,由于这种明显的症状,绝经前妇女的泌乳素瘤通常在肿瘤较小的情况下早期诊断出来;②乳房产生乳汁的证据(溢乳);③性欲减少。


男性和绝经后妇女的泌乳素瘤症状:男性和绝经后的女性没有月经期,因此催乳素升高的影响常常被忽略。特别是在男性中,泌乳素瘤可能多年未诊断。


男性的症状包括:①睾丸激素水平降低,导致性欲低下;②男性乳房发育,男性的泌乳素瘤通常非常大,可能会侵犯垂体正常腺体附近的结构。


诊断


通过身体检查并结合症状和病史。其他诊断方式包括:激素测试;影像检查;垂体功能检查等。验血会测量催乳素的水平,这对于确定诊断至关重要。


治疗


泌乳素瘤的最佳治疗取决于多种因素,包括:视力丧失的突然性和严重性、对药物治疗的反应;治疗选择包括:药物治疗、手术治疗、放射治疗。

Giant Prolactinoma Causing Hydrocephalus and Intracranial Hypertension as First Manifestations of Multiple Endocrine Neoplasia Type 1复制标题

巨大泌乳素瘤引起的脑积水和颅内高压是多发性内分泌腺瘤1型的首发表现

发表时间:2019-08-01

影响指数:3.6

作者: Naiara C B Dantas

期刊:Pituitary Endocrinology

We reported the peculiar case of a young man with MEN1 syndrome presenting with a combination of rare or exceptionally rare events, such as presence of giant prolactinoma larger than 6cm; pituitary tumor as the first clinical manifestation of MEN1 and occurrence of hydrocephalus and intra cranial hypertension, as initial symptoms of giant prolactinoma. Sporadic (non-familial) giant prolactinomas are rare tumors representing, respectively, 0.5% of all pituitary tumors and 4% of all prolactinomas, as reviewed by Maiter and Delgrange. Most of them are characterized by tumors smaller than 6 cm. In fact, only few cases whose dimensions exceed this diameter have been described either as part of several small series of giant prolactinomas or as case reports. So far, only one study focused on this specific tumor subset and compiled 18 cases with giant prolactinomas larger than 60 mm. The latter cases accounted for no more than 1% of all prolactinoma patients from five tertiary reference centers reported in a period of 20 years, highlighting the marked rareness of this condition. To our knowledge, we reported the first patient with MEN1 syndrome presenting with a giant prolactinoma larger than 6cm. Despite the small number of MEN1-related macroprolactinoma cases in patients younger than 21 years (14, including the present case), the frequency of giant prolactinomas at this age group is much higher (4/11; 36%) than the overall estimated prevalence (2–4%) of age-independent giant prolactinomas. These data may suggest that in younger MEN1 patients the frequency of giant prolactinomas is higher than in age-matched sporadic cases. Overall, giant prolactinomas are frequently diagnosed at the fourth to fifth decades being very rare in children and adolescents. Thus, most of the 140 giant prolactinoma cases compiled from a meta-analysis were diagnosed around 40 years of age. Furthermore, only 16 cases with giant prolactinoma were identified during infancy and adolescence, highlighting the rarity of the present case report. Three large series including apparently sporadic pituitary tumors diagnosed at younger ages have recommended routine genetic screening for MEN1 and AIP genes. Despite the occurrence of MEN1-related macroprolactinoma in 5% of patients younger than 20 y (3/59) and 10% (3/30) in those younger than 18 years, there was no case with MEN1-related giant prolactinoma. Several other prolactinoma series at young ages have been reported either with no MEN1 genetic analysis or MEN1 cases eventually diagnosed due to familial history and/or molecular diagnosis, suggesting potential underdiagnosis of this condition in this age group.

译文

我们报道了一名患有MEN1综合征的年轻人的特殊病例,伴有罕见或极为罕见的事件,例如大于6cm的巨大泌乳素瘤的存在;垂体瘤是MEN1的首例临床表现,脑积水和颅内高压的发生是巨大泌乳素瘤的最初症状。根据Maiter和Delgrange的评论,偶发性(非家族性)巨大的泌乳素瘤是罕见的肿瘤,分别占所有垂体瘤的0.5%和所有泌乳素瘤的4%。它们中的大多数以小于6cm的肿瘤为特征。实际上,只有少数几个尺寸超过该直径的病例被描述为几个小系列的巨大泌乳素瘤的一部分或作为病例报告。到目前为止,只有一项研究针对这一特定的肿瘤亚群,并汇编了18例大于60毫米的巨大泌乳素瘤。在过去的20年中,来自五个三级参考中心的泌乳素瘤患者中,后一种病例的发病率不超过1%,这突出表明了这种情况的罕见性。据我们所知,我们报道了第一例MEN1综合征患者,其表现为大于6cm的巨大泌乳素瘤。尽管在21岁以下的患者中与MEN1相关的大泌乳素瘤病例数量很少(14例,包括本病例),但该年龄组的巨大泌乳素瘤的发生率(4/11; 36%)比总体估计患病率高得多(2-4%)与年龄无关的巨大泌乳素瘤。这些数据可能表明,在年轻的MEN1患者中,巨乳催乳瘤的发生率高于年龄相匹配的散发性乳癌。总体而言,巨乳催乳素瘤通常在第四至第五十年被诊断出,在儿童和青少年中非常罕见。因此,根据荟萃分析汇编的140例巨大泌乳素瘤病例中,大多数被诊断为40岁左右。此外,在婴儿期和青春期仅发现了16例巨大的催乳素瘤病例,这突出了本病例报告的罕见性。建议对包括MEN1和AIP基因的常规基因筛查进行三个大系列研究,包括在年轻时诊断出的明显散发性垂体瘤。尽管在20岁以下(3/59)的患者中有5%发生了MEN1相关的大泌乳素瘤,而在18岁以下的人群中发生了MEN1相关的大泌乳素瘤,但没有MEN1相关的巨大泌乳素瘤的病例。据报道,其他几个年轻的泌乳素瘤系列患者均未进行MEN1遗传学分析或由于家族史和/或分子诊断而最终被确诊为MEN1病例,提示该年龄组对该病的潜在诊断不足。

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