具有非典型病程的巨大催乳素瘤

文献信息

中文摘要

背景： 催乳素瘤是最常见的垂体腺瘤之一。与催乳素瘤相关的高催乳素血症的典型症状主要包括因下丘脑-垂体-性腺轴受抑制而导致的性腺功能减退症状。在男性和绝经后女性中，通常以与肿瘤占位效应相关的症状为主。然而，在某些病例中，催乳素瘤最初可能表现为非典型症状，这会使正确诊断的建立变得困难。方法：使用PubMed数据库进行了涵盖过去30年的大范围文献检索。应用了以下检索词：“巨大催乳素瘤”、“非典型催乳素瘤”和“巨型催乳素瘤表现”。仅纳入与实验室确诊的高催乳素血症（催乳素浓度>100 ng/mL）相关的垂体大腺瘤非典型病例。在本研究中，我们还纳入了3例在我们科室遇到的、诊断时表现为非典型表现的大催乳素瘤病例。结果：总体而言，有15篇文章符合纳入标准。包括本研究中描述的病例在内，共识别出28例诊断时表现为非典型症状的大催乳素瘤患者。大多数患者为男性，且多数年龄在40-60岁。最常见的非典型表现包括神经系统症状，如癫痫、脑神经麻痹、脑积水或行为障碍，以及耳鼻喉科症状，如反复鼻出血或鼻塞。大催乳素瘤最常被误诊为颅底肿瘤、鼻窦恶性肿瘤或原发性神经系统疾病。在大多数病例中，开始多巴胺激动剂治疗可带来快速的临床改善以及肿瘤部分或完全消退，即使在巨型大催乳素瘤病例中也是如此。一部分分化良好或中等分化的嗅神经母细胞瘤实际上可能是被误诊的大催乳素瘤。如果存在其他内分泌肿瘤，高催乳素血症的典型症状可能会被掩盖。在某些病例中，大催乳素瘤是在病情变得危及生命时才被诊断出来。结论：对于任何患有鞍区或鞍旁肿瘤的患者，都应检测血清催乳素水平。

英文摘要

Prolactinomas are among the most common pituitary adenomas. The typical symptoms of hyperprolactinemia associated with a prolactinomaprimarily include symptoms of hypogonadism resulting from the suppression of the hypothalamic-pituitary-gonadal axis.In men and postmenopausal women, symptoms related to the mass effect of the tumor usually predominate. In some cases, however,prolactinomas may initially present with atypical symptoms, which can make it difficult to establish the correct diagnosis. An extensive literature search covering the past 30 years was conducted using the PubMed database. The following search terms were applied: "macroprolactinoma", "atypical prolactinoma", and "giant prolactinoma manifestations". Only atypical cases of pituitary macroadenomasassociated with laboratory-confirmed hyperprolactinemia (prolactin concentrations > 100 ng/mL) were included. In ourstudy, we also included 3 cases of macroprolactinoma with an atypical presentation at the time of diagnosis, which we encountered inour department. Overall, 15 articles met the inclusion criteria. Including the cases described in this study, 28 patients with macroprolactinoma presenting with atypical symptoms at diagnosis were identified. Most patients were men, and the majority were aged 40-60 years. The most commonatypical manifestations included neurological symptoms such as epilepsy, cranial nerve palsy, hydrocephalus or behavioral disorders,as well as otolaryngological symptoms such as recurrent epistaxis or nasal obstruction. Macroprolactinomas were most commonlymisdiagnosed as skull base tumors, sinonasal malignancies, or primary neurological disorders. In most cases, the initiation of dopamineagonist therapy led to rapid clinical improvement and partial or complete tumor regression, even in cases of giant macroprolactinomas. A proportion of well- or moderately differentiated olfactory neuroblastomas may, in fact, be misdiagnosed macroprolactinomas. If other endocrine tumors are present, the typical symptoms of hyperprolactinemia may be masked. In some cases, macroprolactinomas are diagnosedwhen the condition becomes life-threatening. Serum prolactin levels should be measured in any patient with a sellar or parasellar tumor.