Section of Specialized Endocrinology, Department of Endocrinology, Oslo University Hospital, Oslo, Norway.
Medizinische Klinik und Poliklinik IV, Klinikum der LMU, Ludwig-Maximilians-Universität München, Munich, Germany.
Neuroendocrinology. 2019;109(1):57-69. doi: 10.1159/000497205. Epub 2019 Jan 24.
Prolactin-secreting tumors (prolactinomas) represent the most common pituitary tumor type, accounting for 47-66% of functional pituitary tumors. Prolactinomas are usually benign and controllable tumors as they express abundant levels of dopamine type 2 receptor (D2), and can be treated with dopaminergic drugs, effectively reducing prolactin levels and tumor volume. However, a proportion of prolactinomas exhibit aggressive features (including invasiveness, relevant growth despite adequate dopamine agonist treatment, and recurrence potential) and few may exhibit metastasizing potential (carcinomas). In this context, the clinical, pathological, and molecular definitions of malignant and aggressive prolactinomas remain to be clearly defined, as primary prolactin-secreting carcinomas are similar to aggressive adenomas until the presence of metastases is detected. Indeed, standard molecular and histological analyses do not reflect differences between carcinomas and adenomas at a first glance and have limitations in prediction of the aggressive progression of prolactinomas, wherein the causes underlying the aggressive behavior remain unknown. Herein we present a comprehensive, multidisciplinary review of the most relevant epidemiological, clinical, pathological, genetic, biochemical, and molecular aspects of aggressive and malignant prolactinomas.
催乳素分泌性肿瘤（泌乳素瘤）是最常见的垂体肿瘤类型，占功能性垂体肿瘤的 47-66%。泌乳素瘤通常是良性和可控制的肿瘤，因为它们表达丰富的多巴胺 D2 受体（D2），可以用多巴胺能药物治疗，有效地降低催乳素水平和肿瘤体积。然而，一部分泌乳素瘤表现出侵袭性特征（包括侵袭性、尽管多巴胺激动剂治疗充分但仍有相关生长、以及复发的可能性），少数可能表现出转移潜能（癌）。在这种情况下，恶性和侵袭性泌乳素瘤的临床、病理和分子定义仍有待明确界定，因为原发性催乳素分泌性癌与侵袭性腺瘤相似，直到发现转移为止。事实上，标准的分子和组织学分析并不能在第一时间反映出癌和腺瘤之间的差异，并且在预测泌乳素瘤的侵袭性进展方面存在局限性，而侵袭性行为的原因仍不清楚。本文对侵袭性和恶性泌乳素瘤的最相关的流行病学、临床、病理、遗传、生化和分子方面进行了全面的多学科综述。
