McCook T A, Putman C E, Dale J K, Wells S A
AJR Am J Roentgenol. 1982 Jul;139(1):149-55. doi: 10.2214/ajr.139.1.149.
Medullary carcinoma of the thyroid is a unique neoplasm characterized by consistent production of a hormonal marker, calcitonin, calcification of both primary and metastatic foci, and association with other endocrine neoplasms. It accounts for 3.5%--10% of all thyroid malignancies. First described by Hazard et al. [1] in 1959, this neoplasm has become the focus of increasing clinical and experimental investigation. It occurs both sporadically and in families. When familial, it is seen as a component of the multiple endocrine neoplasia type II (MEN-II) syndromes. Interest in medullary carcinoma of the thyroid far exceeds that warranted by its infrequent occurrence. Over 200 articles dealing with this tumor have appeared in the medical literature in the last 10 years. This may be attributed to the number of distinctive properties of the tumor: the familial occurrence when associated with endocrinopathies, the propensity to produce amyloid, and the great synthetic capability for the biogenic amines calcitonin, serotonin, histaminase, and prostaglandins. Of greatest importance is the value of calcitonin radioimmunoassay for detecting patients with the tumor even though it may be evident clinically. Fifty-three patients with surgically and biochemically proven medullary carcinoma were evaluated and followed at Duke University Medical Center from 1968 to 1981. Ten patients with sporadic tumor, 37 with MEN type IIa, and six with MEN type IIb prompted this review. They will be used to illustrate the varied and sometimes characteristic radiographic manifestations of medullary carcinoma of the thyroid. This tumor has a spectrum of radiographic features in multiple organs that, when familial, facilitate its recognition and treatment.
甲状腺髓样癌是一种独特的肿瘤，其特征是持续产生一种激素标志物——降钙素，原发灶和转移灶均有钙化，并与其他内分泌肿瘤相关。它占所有甲状腺恶性肿瘤的3.5% - 10%。1959年由哈泽德等人首次描述，这种肿瘤已成为临床和实验研究日益关注的焦点。它既散发发生，也有家族性发病。家族性发病时，它被视为II型多发性内分泌肿瘤（MEN-II）综合征的一个组成部分。对甲状腺髓样癌的关注远远超过其罕见发病率所应有的程度。在过去10年中，医学文献中出现了200多篇关于这种肿瘤的文章。这可能归因于该肿瘤的一些独特特性：与内分泌病相关时的家族性发病、产生淀粉样物质的倾向以及对生物胺降钙素、血清素、组胺酶和前列腺素的强大合成能力。最重要的是降钙素放射免疫测定对于检测患有该肿瘤的患者的价值，即使在临床上可能尚不明显。1968年至1981年期间，杜克大学医学中心对53例经手术和生化证实的甲状腺髓样癌患者进行了评估和随访。10例散发性肿瘤患者、37例IIa型MEN患者和6例IIb型MEN患者促使了本次综述。它们将用于说明甲状腺髓样癌多样且有时具有特征性的影像学表现。这种肿瘤在多个器官具有一系列影像学特征，家族性发病时便于其识别和治疗。
