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MEN1 相关垂体瘤:勿被轻度升高的催乳素水平误导。

Pituitary tumors in MEN1: do not be misled by borderline elevated prolactin levels.

机构信息

Hadassah Medical School, Hebrew University of Jerusalem, Jerusalem, Israel.

Division of Endocrinology, Mercy Hospital, Chicago, IL, USA.

出版信息

Pituitary. 2016 Dec;19(6):601-604. doi: 10.1007/s11102-016-0752-z.

DOI:10.1007/s11102-016-0752-z
PMID:27600151
Abstract

PURPOSE

The objective of this case report is to demonstrate that the simple expedient of measuring periodic prolactin levels in patients with MEN1 who have modest hyperprolactinemia and normal pituitary MRI scans is insufficient to monitor for the development of pituitary adenomas.

METHODS

Review of relevant literature and chart review.

RESULTS

A 25 year old man with known MEN1 manifested by hyperparathyroidism and a gastrin-producing neuroendocrine tumor was found to have a prolactin [PRL] level of 20.0 ng/mL [1.6-16 ng/mL] but a normal pituitary MRI scan. The impression then was that he had prolactinoma too small to be visualized on the MRI. Over the next 3.5 years his PRL levels remained in this mildly elevated range but he then presented with severe headaches and visual field defects. An MRI showed a 3.1 × 1.7 × 1.9 cm pituitary adenoma with compression of the optic chiasm and invasion of the left cavernous sinus. Surgery revealed a gonadotroph adenoma and he subsequently required gamma knife radiotherapy for residual tumor. Postoperative PRL levels were normal.

CONCLUSIONS

Small, intrasellar microadenomas may be associated with elevated PRL levels due to possible direct hormone production [prolactinoma] or possibly to interference with portal vessel blood flow. In monitoring hyperprolactinemic MEN1 patients for the development of pituitary adenomas, measurement of PRL levels is insufficient and periodic MRI scans are necessary at a more frequent interval than every 3-5 years. This may also pertain to patients with "idiopathic" hyperprolactinemia.

摘要

目的

本病例报告的目的是证明,对于患有轻度高催乳素血症和正常垂体 MRI 扫描的 MEN1 患者,仅测量催乳素(PRL)水平来监测垂体腺瘤的发展是不够的。

方法

复习相关文献和病历。

结果

一名 25 岁男性,患有已知的 MEN1,表现为甲状旁腺功能亢进和胃泌素分泌神经内分泌肿瘤,发现催乳素(PRL)水平为 20.0ng/mL[1.6-16ng/mL],但垂体 MRI 扫描正常。当时的印象是他患有太小而无法在 MRI 上看到的催乳素瘤。在接下来的 3.5 年中,他的 PRL 水平仍处于轻度升高的范围内,但随后出现严重头痛和视野缺陷。MRI 显示一个 3.1×1.7×1.9cm 的垂体腺瘤,压迫视交叉并侵犯左侧海绵窦。手术显示为促性腺激素腺瘤,随后他需要伽玛刀放疗以治疗残留肿瘤。术后 PRL 水平正常。

结论

较小的鞍内微腺瘤可能与升高的 PRL 水平有关,可能是由于直接激素产生(催乳素瘤)或可能干扰门脉血流。在监测患有高催乳素血症的 MEN1 患者的垂体腺瘤发展时,仅测量 PRL 水平是不够的,需要更频繁地进行 MRI 扫描(每 3-5 年一次)。这也可能适用于“特发性”高催乳素血症患者。

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本文引用的文献

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Long-Term Natural Course of Pituitary Tumors in Patients With MEN1: Results From the DutchMEN1 Study Group (DMSG).MEN1患者垂体肿瘤的长期自然病程:荷兰MEN1研究组(DMSG)的结果
J Clin Endocrinol Metab. 2015 Sep;100(9):3288-96. doi: 10.1210/JC.2015-2015. Epub 2015 Jun 30.
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Pituitary tumors in patients with MEN1 syndrome.
MEN1 综合征患者的垂体瘤。
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Diagnosis and treatment of hyperprolactinemia: an Endocrine Society clinical practice guideline.高泌乳素血症的诊断和治疗:内分泌学会临床实践指南。
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Multiple endocrine neoplasia type 1 (MEN1).多发性内分泌腺瘤病 1 型(MEN1)。
Best Pract Res Clin Endocrinol Metab. 2010 Jun;24(3):355-70. doi: 10.1016/j.beem.2010.07.003.
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Multiple endocrine neoplasia type 1 in Northern Finland; clinical features and genotype phenotype correlation.芬兰北部的1型多发性内分泌肿瘤;临床特征与基因型-表型相关性
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Do the limits of serum prolactin in disconnection hyperprolactinaemia need re-definition? A study of 226 patients with histologically verified non-functioning pituitary macroadenoma.垂体柄离断性高催乳素血症中血清催乳素的界限是否需要重新定义?一项对226例经组织学证实的无功能垂体大腺瘤患者的研究。
Clin Endocrinol (Oxf). 2006 Oct;65(4):524-9. doi: 10.1111/j.1365-2265.2006.02627.x.
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A non-functioning pituitary adenoma initially mimicking a microprolactinoma: The case for long-term follow-up of patients with mild hyperprolactinemia?
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Multiple endocrine neoplasia type 1 variant with frequent prolactinoma and rare gastrinoma.1型多发性内分泌腺瘤病变异型,常伴泌乳素瘤,罕见胃泌素瘤。
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