Tiedje V, Ting S, Dralle H, Schmid K W, Führer D
Klinik für Endokrinologie und Stoffwechselerkrankungen, Medizinisches Zentrum, Universitätsklinikum Essen (AöR), Hufelandstr. 55, 45147, Essen, Deutschland,
Internist (Berl). 2015 Sep;56(9):1019-31. doi: 10.1007/s00108-014-3638-z.
Medullary thyroid carcinoma (MTC) is a very rare malignancy, which arises from parafollicular C cells and accounts for 3-5% of all thyroid cancers. MTC represents a neuroendocrine tumor with a biology that differs considerably from differentiated thyroid cancer. Presence of a RET proto-oncogene germline mutation indicates hereditary C cell disease in the context of multiple endocrine neoplasia type 2 and hence a special treatment algorithm is required. Cure of MTC is only possible through surgery. Calcitonin screening is advocated for early MTC diagnosis and preoperative MTC management stratification. In case of surgically incurable persistent MTC, estimation of calcitonin and CEA doubling time is crucial to assess tumor biology and is complemented by multimodal imaging to assess tumor burden. Treatment decisions in incurable MTC must be carefully balanced with treatment-related morbidity, since MTC may take an indolent course over years.
甲状腺髓样癌（MTC）是一种非常罕见的恶性肿瘤，起源于滤泡旁C细胞，占所有甲状腺癌的3% - 5%。MTC是一种神经内分泌肿瘤，其生物学特性与分化型甲状腺癌有很大不同。RET原癌基因种系突变的存在表明在2型多发性内分泌肿瘤背景下存在遗传性C细胞疾病，因此需要一种特殊的治疗方案。MTC只有通过手术才能治愈。提倡进行降钙素筛查以早期诊断MTC并进行术前MTC管理分层。对于手术无法治愈的持续性MTC，降钙素和癌胚抗原倍增时间的评估对于评估肿瘤生物学特性至关重要，并辅以多模态成像来评估肿瘤负荷。对于无法治愈的MTC，治疗决策必须与治疗相关的发病率仔细权衡，因为MTC可能在数年内呈惰性病程。
