Genetics and Endocrinology Section, Metabolic Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland 20892, USA.
J Clin Endocrinol Metab. 2013 Aug;98(8):3139-48. doi: 10.1210/jc.2013-1511. Epub 2013 Jun 14.
Multiplicity of hormone-secreting tumors occurs in a substantial portion of hormone-excess states. Multiplicity increases the difficulty of management and drives the selection of special strategies.
This is a synthesis from publications about tumor development and expression, and also about types of clinical strategy for hormone-secreting tumors.
Comparisons were made between patient groups with solitary tumors vs those with multiple tumors. Major themes with clinical relevance emerged. Usually, tumor multiplicity develops from a genetic susceptibility in all cells of a tissue. This applies to hormone-secreting tumors that begin as either polyclonal (such as in the parathyroids of familial hypocalciuric hypercalcemia) or monoclonal tumors (such as in the parathyroids of multiple endocrine neoplasia type 1 [MEN1]). High penetrance of a hereditary tumor frequently results in bilaterality and in several other types of multiplicity. Managements are better for the hormone excess than for the associated cancers. Management strategies can be categorized broadly as ablation that is total, subtotal, or zero. Examples are discussed for each category, and 1 example of each category is named here: 1) total ablation of the entire tissue with effort to replace ablated functions (for example, in C-cell neoplasia of multiple endocrine neoplasia type 2); 2) subtotal ablation with increased likelihood of persistent disease or recurrent disease (for example, in the parathyroid tumors of MEN1); or 3) no ablation of tissue with or without the use of pharmacotherapy (for example, with blockers for secretion of stomach acid in gastrinomas of MEN1).
Tumor multiplicity usually arises from defects in all cells of the precursor tissue. Even the optimized managements involve compromises. Still, an understanding of pathophysiology and of therapeutic options should guide optimized management.
在大量激素过多的情况下，会出现多种激素分泌肿瘤。多发性肿瘤增加了管理的难度，并促使选择特殊的策略。
这是对肿瘤发生和表达以及激素分泌肿瘤的临床策略类型的出版物进行综合分析的结果。
对患有单发肿瘤的患者组与患有多发肿瘤的患者组进行了比较。出现了具有临床相关性的主要主题。通常，肿瘤多发性是由组织中所有细胞的遗传易感性引起的。这适用于激素分泌肿瘤，它们最初可以是多克隆的（如家族性低钙血症性高钙血症的甲状旁腺）或单克隆肿瘤（如多发性内分泌肿瘤 1 型[MEN1]的甲状旁腺）。遗传性肿瘤的高穿透性常导致双侧性和其他几种多发性肿瘤。激素过多的管理比相关癌症的管理要好。管理策略可以大致分为完全、部分或零消融。对每个类别都进行了讨论，并在此处命名了每个类别的一个示例：1）努力替代被消融功能的整个组织的完全消融（例如，多发性内分泌肿瘤 2 型中的 C 细胞肿瘤）；2）具有持续疾病或复发疾病可能性增加的部分消融（例如，MEN1 中的甲状旁腺肿瘤）；或 3）不消融组织，无论是否使用药物治疗（例如，MEN1 中的胃泌素瘤中使用分泌胃酸的阻滞剂）。
肿瘤多发性通常是由前体细胞组织中所有细胞的缺陷引起的。即使是优化的管理也涉及到妥协。尽管如此，对病理生理学和治疗选择的理解应该指导优化的管理。
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