Poorly differentiated thyroid carcinoma An underdiagnosed entity
Poorly differentiated thyroid carcinoma An underdiagnosed entity
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DOI：
10.1007/s00292-019-0600-9
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发表时间：
2020-06-01
期刊：
影响因子：
--
通讯作者：
Perren, A.
中科院分区：
文献类型：
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作者：
Dettmer, M. S.;Schmitt, A.;Perren, A.
低分化甲状腺癌（PDTC）是一种罕见的甲状腺癌亚型，生物学上位于分化良好的乳头状/滤泡状甲状腺癌和未分化甲状腺癌（ATC）之间。传统型和嗜酸细胞型低分化甲状腺癌的诊断是困难的，在日常工作中经常被漏诊。目前世卫组织允许诊断PDTC的标准是基于2006年在都灵举行的共识会议的结果。即使是10%的微小低分化癌也会显著影响患者的预后，嗜酸细胞亚型甚至可能有更差的预后。免疫组化没有太大帮助，主要用于用降钙素排除甲状腺髓样癌，并通过甲状腺球蛋白染色确定起源于滤泡细胞。由于逐步去分化的概念，在不同的甲状腺癌亚型之间存在大量重叠的不同分子改变，如BRAF、RAS、CTNNB 1、TP 53等。因此，目前尚无法获得独特的分子肿瘤特征。PDTC具有独特的miRNA特征，将其与其他甲状腺癌区分开来。平均无复发生存期不到一年，约50%的患者死于该病。现代酪氨酸激酶抑制剂与强大的分子诊断相结合，为这些难以治疗的癌症提供了新的机会。
Poorly differentiated thyroid carcinomas (PDTCs) are a rare subtype of thyroid carcinomas that are biologically situated between well-differentiated papillary/follicular thyroid carcinomas and anaplastic thyroid carcinomas (ATCs). The diagnosis of conventional as well as oncocytic poorly differentiated thyroid carcinoma is difficult and often missed in daily routine. The current WHO criteria to allow the diagnosis of PDTCs are based on the results of a consensus meeting held in Turin in 2006. Even a minor poorly differentiated component of only 10%of a given carcinoma significantly affects patient prognosis and the oncocytic subtype may even have a worse outcome. Immunohistochemistry is not much help and is mostly used to exclude a medullary thyroid carcinoma with calcitonin and to establish a follicular cell of origin via thyroglobulin staining. Due to the concept of stepwise dedifferentiation, there is a vast overlap of different molecular alterations likeBRAF, RAS, CTNNB1, TP53and others between different thyroid carcinoma subtypes. A distinctive molecular tumor profile is therefore currently not available. PDTCs have a unique miRNA signature, which separates them from other thyroid carcinomas. The average relapse free survival is less than one year and about 50% of patients die of the disease. Modern tyrosine kinase inhibitors offer in conjunction with powerful molecular diagnostic new chances in these difficult to treat carcinomas.