Vergès Bruno, Boureille Françoise, Goudet Pierre, Murat Arnaud, Beckers Albert, Sassolas Geneviève, Cougard Patrick, Chambe Béatrice, Montvernay Corinne, Calender Alain
Department of Endocrinology, University Hospital, 21000 Dijon, France.
J Clin Endocrinol Metab. 2002 Feb;87(2):457-65. doi: 10.1210/jcem.87.2.8145.
To date, data on pituitary adenomas in MEN type 1 (MEN1) still have to be evaluated. We analyzed the data of a large series of 324 MEN1 patients from a French and Belgian multicenter study. Data on pituitary disease were compared with those from 110 non-MEN1 patients with pituitary adenomas, matched for age, year of diagnosis, and follow-up period. Genetic analysis of the MEN1 gene was performed in 197 of the MEN1 patients. In our MEN1 series, pituitary disease occurred in 136 of 324 (42%), less frequently than hyperparathyroidism (95%, P < 0.001) and endocrine enteropancreatic tumors (54%, P < 0.01). Mean age of onset of pituitary tumors was 38.0+/-15.3 yr (range, 12-83 yr). Pituitary disease was associated with hyperparathyroidism in 90% of cases, with enteropancreatic tumors in 47%, with adrenal tumors in 16%, and with thoracic neuroendocrine tumors in 4%. Pituitary disease was the initial lesion of MEN1 in 17% of all MEN1 patients. MEN1 pituitary adenomas were significantly more frequent in women than in men (50% vs. 31%, P < 0.001). Among the 136 pituitary adenomas, there were 85 prolactinomas and 12 GH-secreting, 6 ACTH-secreting, 13 cosecreting, and 20 nonsecreting tumors. Eighty-five percent of MEN1-related pituitary lesions were macroadenomas (vs. 42% in non-MEN1 patients, P < 0.001), including 32% of invasive cases. Among secreting adenomas, hormonal hypersecretion was normalized, after treatment, in only 42% (vs. 90% in non-MEN1 patients, P < 0.001), with a median follow-up of 11.4 yr. No correlation was found between the type of MEN1 germ-line mutation and the presence or absence of pituitary adenoma. Our study, based on a large group of MEN1 patients, shows that pituitary adenomas occur in 42% of the cases and are characterized by a larger size and a more aggressive presentation than without MEN1.
迄今为止，1型多发性内分泌腺瘤病（MEN1）中垂体腺瘤的数据仍有待评估。我们分析了来自法国和比利时多中心研究的324例MEN1患者的大量数据。将垂体疾病的数据与110例非MEN1垂体腺瘤患者的数据进行比较，这些患者在年龄、诊断年份和随访期方面相匹配。对197例MEN1患者进行了MEN1基因的遗传分析。在我们的MEN1系列中，324例中有136例（42%）发生垂体疾病，其发生率低于甲状旁腺功能亢进（95%，P<0.001）和内分泌性肠胰腺肿瘤（54%，P<0.01）。垂体肿瘤的平均发病年龄为38.0±15.3岁（范围12 - 83岁）。90%的垂体疾病病例与甲状旁腺功能亢进相关，47%与肠胰腺肿瘤相关，16%与肾上腺肿瘤相关，4%与胸段神经内分泌肿瘤相关。垂体疾病是所有MEN1患者中17%的MEN1初始病变。MEN1垂体腺瘤在女性中比男性更常见（50%对31%，P<0.001）。在136例垂体腺瘤中，有85例泌乳素瘤、12例生长激素分泌型、6例促肾上腺皮质激素分泌型、13例分泌多种激素型和20例无分泌功能型肿瘤。85%的MEN1相关垂体病变为大腺瘤（非MEN1患者中为42%，P<0.001），其中32%为侵袭性病例。在分泌型腺瘤中，治疗后激素分泌过多仅在42%的病例中恢复正常（非MEN1患者中为90%，P<0.001），中位随访时间为11.4年。未发现MEN1种系突变类型与垂体腺瘤的有无之间存在相关性。我们基于一大组MEN1患者的研究表明，42%的病例发生垂体腺瘤，其特征是比无MEN1时肿瘤更大且表现更具侵袭性。
