Familial medullary thyroid carcinoma and C cell hyperplasia.
Familial medullary thyroid carcinoma and C cell hyperplasia.
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家族性甲状腺髓样癌和C细胞增生。
DOI：
10.1016/s0300-595x(81)80027-8
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发表时间：
1981
期刊：
影响因子：
--
通讯作者：
Delellis,RA
中科院分区：
文献类型：
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作者：
Wolfe,HJ;Delellis,RA
虽然甲状腺髓样癌作为一种临床病理学实体已被明确鉴定超过20年，但这种肿瘤及其祖细胞C细胞仍然是当前研究的主要课题（Hazard，Hawke和Crile，1959;威廉姆斯，1966）。认识到C细胞是不断增长的神经内分泌程序化细胞系统的原型，已知这些系统在结构和功能上弥合了经典神经和内分泌调节机制之间的鸿沟，这是这种持续关注的一个重要因素。此外，发现这些C细胞来源的肿瘤中的一些是遗传决定的，并且可能与其他神经内分泌程序化细胞系统中的瘤形成相关，如多发性内分泌瘤形成II型综合征，提供了该领域大量进展所基于的人类模型（Steiner，Goodman和Powers，1968; DeLellis和Wolfe，1981）。家族性髓样癌不仅为研究高危人群的内分泌肿瘤提供了机会，而且也为检测和表征疾病的早期阶段提供了机会。肿瘤的C细胞起源和高风险的激酶的鉴定为研究人员提供了研究正常和肿瘤性C细胞的激素和非激素产物作为潜在肿瘤标志物的理由和机会。这种方法已被证明是如此成功，以至于今天，使用选定的标记物，不仅可以在家族性甲状腺髓样癌中检测临床和组织病理学上的癌前病变和早期瘤形成，而且还可以通过组织病理学标记物区分甲状腺髓样癌的家族性和散发性变体（Wolfe等，1973，1980）。
Although medullary thyroid carcinoma has been clearly identified as a clinicopathological entity for over 20 years, this tumour and its progenitor, the C cell, remain major topics of current investigation (Hazard, Hawke and Crile, 1959; Williams, 1966). The recognition that the C cell is a prototype of the ever-growing list of neuroendocrine-programmed cell systems that are known to bridge both structurally and functionally the chasm between classical neural and endocrine regulatory mechanisms has been an important factor in this continued interest. Furthermore, the discovery that some of these tumours of C cell origin are genetically determined and may be associated with neoplasia in other neuroendocrine-programmed cell systems, as in the syndrome of multiple endocrine neoplasia type II, provides a human model upon which a great deal of the progress in this field has been based (Steiner, Goodman and Powers, 1968; DeLellis and Wolfe, 1981). Familial medullary carcinoma has offered the opportunity not only to study endocrine neoplasia in a high-risk population but also to detect and characterize the earliest stages of the disease. The tumour's C cell origin and the identification of kindreds at high risk for the disease have offered investigators the rationale and opportunity for studying both hormonal and non-hormonal products of the normal and neoplastic C cell as potential tumour markers. This approach has proved so successful that today, with selected markers, not only is it possible in familial medullary thyroid carcinoma to detect preneoplasia and early neoplasia both clinically and histopathologically, but it is also possible to distinguish the familial from the sporadic variant of medullary thyroid carcinoma by a histopathological marker (Wolfe et al, 1973, 1980).