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2B型多发性内分泌腺瘤病综合征的闪烁显像描绘

Scintigraphic portrayal of the syndrome of multiple endocrine neoplasia type-2B.

作者信息

Yobbagy J J, Levatter R, Sisson J C, Shulkin B L, Polley T

机构信息

University of Michigan Medical Center, Ann Arbor.

出版信息

Clin Nucl Med. 1988 Jun;13(6):433-7. doi: 10.1097/00003072-198806000-00011.

DOI:10.1097/00003072-198806000-00011
PMID:2900089
Abstract

The scintigraphic appearance of the neoplasms in multiple endocrine neoplasia type 2B (MEN-2B) and the interpretations of the image patterns are described. An 18-year-old male patient with the MEN-2B syndrome underwent TI-201 imaging that showed concentrations of TI-201 in the primary medullary thyroid carcinoma (MTC) tumor and in cervical lymph node metastases. After total thyroidectomy and lymph node dissection, the TI-201 image was normal. Catecholamine levels in the blood and urine were only borderline elevated. Yet, greater than normal concentrations of I-131 metaiodobenzylguanidine (I-131 MIBG) were present in both adrenal glands. Computed tomography of the abdomen showed normal adrenal glands. These results were consistent with the diagnosis of adrenal medullary hyperplasia, a precursor of pheochromocytoma. No operation was indicated to remove the adrenal glands. Imaging with TI-201 appears to be useful in identifying sites of MTC in patients with the MEN-2B syndrome. I-131 MIBG imaging, in conjunction with computed tomography of the adrenal glands and appropriate catecholamine measurements, should be performed in patients with the MEN-2B syndrome to determine the status of the adrenal medullae, which then may be classified as normal, hyperplastic, or tumorous with pheochromocytoma.

摘要

本文描述了2B型多发性内分泌腺瘤(MEN-2B)中肿瘤的闪烁显像表现及图像模式解读。一名患有MEN-2B综合征的18岁男性患者接受了铊-201(TI-201)显像,结果显示原发性甲状腺髓样癌(MTC)肿瘤及颈部淋巴结转移灶中有TI-201浓聚。甲状腺全切及淋巴结清扫术后,TI-201图像恢复正常。血液和尿液中的儿茶酚胺水平仅略高于正常范围。然而,双侧肾上腺均存在高于正常浓度的碘-131间碘苄胍(I-131 MIBG)。腹部计算机断层扫描显示肾上腺正常。这些结果与肾上腺髓质增生的诊断相符,肾上腺髓质增生是嗜铬细胞瘤的前驱病变。未建议进行肾上腺切除手术。TI-201显像似乎有助于识别MEN-2B综合征患者的MTC病灶部位。对于MEN-2B综合征患者,应结合肾上腺计算机断层扫描及适当的儿茶酚胺测量进行I-131 MIBG显像,以确定肾上腺髓质的状态,进而可将其分类为正常、增生或伴有嗜铬细胞瘤的肿瘤状态。

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引用本文的文献

1
Adrenal Medullary Hyperplasia: A Systematic Review and Meta-analysis.肾上腺髓质增生:系统评价和荟萃分析。
J Clin Endocrinol Metab. 2023 Aug 18;108(9):e885-e892. doi: 10.1210/clinem/dgad121.
2
Accumulation of thallium-201 in medullary thyroid cancer with negative serum calcitonin and carcinoembryonic antigens: a case report.
Ann Nucl Med. 1993 Feb;7(1):53-6. doi: 10.1007/BF03164793.
3
Diagnostic problems in pheochromocytoma.嗜铬细胞瘤的诊断问题。
J Endocrinol Invest. 1989 Nov;12(10):739-57. doi: 10.1007/BF03350050.