Department of Neurosurgery, Clinica Medellin, Hospital Pablo Tobon Uribe, Medellin, Colombia.
Clinics (Sao Paulo). 2012;67 Suppl 1(Suppl 1):43-8. doi: 10.6061/clinics/2012(sup01)09.
We briefly review the characteristics of pituitary tumors associated with multiple endocrine neoplasia type 1. Multiple endocrine neoplasia type 1 is an autosomal-dominant disorder most commonly characterized by tumors of the pituitary, parathyroid, endocrine-gastrointestinal tract, and pancreas. A MEDLINE search for all available publications regarding multiple endocrine neoplasia type 1 and pituitary adenomas was undertaken. The prevalence of pituitary tumors in multiple endocrine neoplasia type 1 may vary from 10% to 60% depending on the studied series, and such tumors may occur as the first clinical manifestation of multiple endocrine neoplasia type 1 in 25% of sporadic and 10% of familial cases. Patients were younger and the time between initial and subsequent multiple endocrine neoplasia type 1 endocrine lesions was significantly longer when pituitary disease was the initial manifestation of multiple endocrine neoplasia type 1. Tumors were larger and more invasive and clinical manifestations related to the size of the pituitary adenoma were significantly more frequent in patients with multiple endocrine neoplasia type 1 than in subjects with non-multiple endocrine neoplasia type 1. Normalization of pituitary hypersecretion was much less frequent in patients with multiple endocrine neoplasia type 1 than in subjects with non-multiple endocrine neoplasia type 1. Pituitary tumors in patients with multiple endocrine neoplasia type 1 syndrome tend to be larger, invasive and more symptomatic, and they tend to occur in younger patients when they are the initial presentation of multiple endocrine neoplasia type 1.
我们简要回顾了与多发性内分泌肿瘤 1 型相关的垂体肿瘤的特征。多发性内分泌肿瘤 1 型是一种常染色体显性遗传疾病，最常见的特征是垂体、甲状旁腺、内分泌-胃肠道和胰腺的肿瘤。我们对所有关于多发性内分泌肿瘤 1 型和垂体腺瘤的可用出版物进行了 MEDLINE 搜索。多发性内分泌肿瘤 1 型中垂体肿瘤的患病率可能因研究系列而异，从 10%到 60%不等，此类肿瘤可能在 25%的散发性和 10%的家族性病例中作为多发性内分泌肿瘤 1 型的首发临床表现出现。当垂体疾病是多发性内分泌肿瘤 1 型的首发表现时，患者年龄更小，初始和随后的多发性内分泌肿瘤 1 型内分泌病变之间的时间间隔明显更长。肿瘤更大、更具侵袭性，与垂体腺瘤大小相关的临床表现在多发性内分泌肿瘤 1 型患者中更为常见。与非多发性内分泌肿瘤 1 型患者相比，多发性内分泌肿瘤 1 型患者的垂体激素过度分泌正常化的频率明显更低。多发性内分泌肿瘤 1 型患者的垂体肿瘤往往更大、侵袭性更强且症状更明显，当它们是多发性内分泌肿瘤 1 型的首发表现时，往往发生在更年轻的患者中。
