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甲状腺髓样癌鞍内转移导致的尿崩症和全垂体功能减退症。

Diabetes insipidus and panhypopituitarism due to intrasellar metastasis from medullary thyroid cancer.

作者信息

Santarpia Libero, Gagel Robert F, Sherman Steven I, Sarlis Nicholas J, Evans Douglas B, Hoff Ana O

机构信息

Department of Endocrine Neoplasia and Hormonal Disorders, The University of Texas M. D. Anderson Cancer Center, Houston, Texas, USA.

出版信息

Head Neck. 2009 Mar;31(3):419-23. doi: 10.1002/hed.20911.

DOI:10.1002/hed.20911
PMID:18798312
Abstract

BACKGROUND

Medullary thyroid cancer (MTC) commonly metastasizes to lymph nodes in the central and lateral neck, but spread to distant organs also occurs, typically involving lung, liver, and bone. Metastases to pituitary gland are rare for this tumor.

METHODS

We describe an unusual case and peculiar presentation of pituitary metastasis from MTC. We report clinical, genetic, and laboratory data of this patient.

RESULTS

A young woman with multiple endocrine neoplasia type 2B was seen with recent onset of classic symptoms and signs of panhypopituitarism, mild diabetes insipidus, and optic chiasmatic compression. Transphenoidal resection of an intrapituitary mass confirmed the presence of metastatic MTC.

CONCLUSIONS

MTC recurrence may present solely with subacute pituitary symptomatology, even in the context of a very lengthy interval after initial surgery, atypically low calcitonin plasma levels, carcinoembryonic antigen doubling times, and the concomitant absence of other tell-tale signs of disseminated metastatic disease.

摘要

背景

甲状腺髓样癌(MTC)通常转移至颈部中央和侧方的淋巴结,但也会发生远处器官转移,典型的转移部位包括肺、肝和骨。该肿瘤转移至垂体较为罕见。

方法

我们描述了一例MTC垂体转移的罕见病例及特殊表现。我们报告了该患者的临床、基因和实验室数据。

结果

一名患有2B型多发性内分泌腺瘤病的年轻女性出现了全垂体功能减退、轻度尿崩症和视交叉受压的典型症状和体征。经蝶窦切除垂体肿块证实为转移性MTC。

结论

MTC复发可能仅表现为亚急性垂体症状,即使是在初次手术后很长一段时间内,降钙素血浆水平异常低、癌胚抗原倍增时间异常,且同时没有其他播散性转移性疾病的明显迹象。

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