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一例罕见的甲状腺髓样癌病例及当前文献综述

An Unusual Case of Medullary Thyroid Carcinoma and A Revision of Current Literature.

作者信息

Cipri Claudia, Vescini Fabio, Torresan Francesca, Pennelli Gianmaria, Pelizzo Maria Rosa, Triggiani Vincenzo, Guastamacchia Edoardo, Grimaldi Franco

机构信息

Endocrinology and Metabolism Unit, University-Hospital "Santa Maria della Misericordia", Udine, Italy.

II General Surgery, Department of Surgery, Oncology and Gastroenterology, University of Padova, Padova, Italy.

出版信息

Endocr Metab Immune Disord Drug Targets. 2019;19(2):226-229. doi: 10.2174/1871530319666181220165350.

Abstract

BACKGROUND

Medullary thyroid cancer (MTC) accounts for 5% of all thyroid cancers and occurs either sporadically or in a hereditary pattern. Routine calcitonin (CT) measurement is suggested for MTC screening in patients with nodular thyroid disease.

PATIENT FINDINGS

A 45 years-old woman incidentally discovered, with neck ultrasound, the presence of thyroid micronodules. Fine-needle aspiration (FNA) on thyroid prevailing nodule did not demonstrate cellular atypia. During follow-up, FNA was repeated on the previously analyzed nodule suspicious for Hürthle cell nodule suspicious for follicular neoplasm and on another hypoechoic right nodule which showed cellular atypia. CT was <2 pg/ml (normal values <18.2 pg/ml), anti-thyroid antibodies were positive and the patient showed a normal thyroid function. The patient also was diagnosed with primary hyperparathyroidism with an enlarged parathyroid gland behind the right thyroid lobe. Therefore, she underwent total thyroidectomy and a selective parathyroidectomy was performed. Histology showed an encapsulated microMTC (pT1aNxMx) associated with diffuse C-cell hyperplasia and lymphocytic thyroiditis. The neoplasm was positive for calcitonin and chromogranin A and negative for thyroglobulin. A right parathyroid adenoma was also diagnosed. One month after surgery basal and stimulated CT were <2 ng/ml. Genetic analysis did not reveal mutation of RET proto-oncogene. Twelve months after surgery, neck ultrasonography, chest and abdomen computed tomography did not demonstrated residual/recurrent disease with undetectable serum CT.

CONCLUSION

In the literature, few MTC cases with normal serum CT have been reported. Although MTC without elevated plasma CT is extremely rare, normal or low CT levels, do not entirely exclude this diagnosis.

摘要

背景

髓样甲状腺癌(MTC)占所有甲状腺癌的5%,可散发或呈遗传模式发生。建议对结节性甲状腺疾病患者进行常规降钙素(CT)检测以筛查MTC。

患者发现情况

一名45岁女性经颈部超声偶然发现甲状腺存在微小结节。对甲状腺优势结节进行细针穿刺(FNA)未显示细胞异型性。在随访期间,对先前分析的疑似许特莱细胞结节(可疑滤泡性腺瘤)的结节以及另一个显示细胞异型性的右叶低回声结节再次进行FNA。CT<2 pg/ml(正常值<18.2 pg/ml),抗甲状腺抗体呈阳性,患者甲状腺功能正常。该患者还被诊断为原发性甲状旁腺功能亢进,右侧甲状腺叶后方甲状旁腺肿大。因此,她接受了全甲状腺切除术并进行了选择性甲状旁腺切除术。组织学检查显示为包膜内微小MTC(pT1aNxMx),伴有弥漫性C细胞增生和淋巴细胞性甲状腺炎。肿瘤降钙素和嗜铬粒蛋白A呈阳性,甲状腺球蛋白呈阴性。还诊断出右侧甲状旁腺腺瘤。术后1个月,基础和刺激后的CT<2 ng/ml。基因分析未发现RET原癌基因突变。术后12个月,颈部超声、胸部和腹部计算机断层扫描未显示残留/复发病变,血清CT检测不到。

结论

文献中报道的血清CT正常的MTC病例很少。虽然血浆CT未升高的MTC极为罕见,但CT水平正常或降低并不能完全排除该诊断。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/976d/7360904/459859ce86d6/EMIDDT-19-226_F1.jpg

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