Pregnancy on vandetanib in metastatic medullary thyroid carcinoma associated with multiple endocrine neoplasia type 2B.
Pregnancy on vandetanib in metastatic medullary thyroid carcinoma associated with multiple endocrine neoplasia type 2B.
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凡德他尼治疗伴有多发性内分泌肿瘤 2B 型的转移性甲状腺髓样癌的妊娠。
DOI：
10.1111/cen.13577
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发表时间：
2018
影响因子：
3.2
通讯作者：
Brooke,Antonia
中科院分区：
文献类型：
--
作者：
Thomas,Nicholas;Glod,John;Derse-Anthony,Claudia;Baple,EmmaL;Osborne,Nigel;Sturley,Rachel;Vaidya,Bijay;Newbold,Kate;Brooke,Antonia
凡德他尼是一种酪氨酸激酶抑制剂（TKI），用于治疗甲状腺髓样癌，> 95%的多发性内分泌腺瘤2b型（MEN 2b）患者发生甲状腺髓样癌。既往未报告接受凡德他尼治疗的MEN 2b女性妊娠，这对母亲和发育中的胎儿都有多种潜在影响[1]。我们描述了一例22岁女性MEN 2b背景患者的病例，该患者在6岁时首次诊断为马凡样体型和口腔粘膜神经瘤。RET基因突变筛查显示错义突变（M918 T/N），甲状腺切除术后的组织学检查证实甲状腺髓样癌伴血管浸润。她有四个进一步的切除术残留疾病的淋巴结和肺转移。16岁时，她开始服用凡德他尼125 mg，每日一次（100 mg/m2），以治疗疾病进展（降钙素24950 ng/l，正常范围< 4.8）（图1）。
Vandetanib is a tyrosine kinase inhibitor (TKI) used in the treatment of medullary thyroid carcinoma occurring in> 95% of patients with multiple endocrine neoplasia type 2b (MEN 2b). Pregnancy in women with MEN 2b on vandetanib is previously unreported and has multiple potential implications for both the mother and developing fetus [1].We describe the case of a 22 year old woman with a background of MEN 2b who was first diagnosed aged 6 presenting with marfanoid habitus, and oral mucosal neuromas. Screening of mutations in the RET gene revealed a missense mutation (M918T/N) and histology from her resulting thyroidectomy confirmed medullary thyroid carcinoma with vascular invasion. She had four further resections for residual disease in lymph nodes and had lung metastases. Aged 16, she commenced on vandetanib 125mg once daily (100mg/m2), for disease progression (calcitonin 24950 ng/l, normal range< 4.8)(Figure 1).