Aberrant G-protein coupled receptor (GPCR) expression is highly prevalent in cortisol-secreting primary bilateral macronodular adrenal hyperplasia (PBMAH) and unilateral adenomas. The aberrant expression of diverse GPCRs and their ligands play an important role in the over-function of various endocrine tumours. Examples include aberrant expression of MC2R, 5-HT4R, AVPR1A, LHCGR, and GnRHR in primary aldosteronism; GCGR, LHCGR, and 5-HT4R in phaeochromocytomas and paragangliomas; TRHR, GnRHR, GIPR, and GRP101 in pituitary somatotroph tumours; AVPR2, D2DR, and SSTR5 in pituitary corticotroph tumours; GLP1R, GIPR, and somatostatin receptors in medullary thyroid carcinoma; and SSTRs, GLP1R, and GIPR in other neuroendocrine tumours. The genetic mechanisms causing the ectopic expression of GIPR in cortisol-secreting PBMAHs and unilateral adenomas have been identified, but distinct mechanisms are implicated in other endocrine tumours. Development of functional imaging targeting aberrant GPCRs should be useful for identification and for specific therapies of this wide spectrum of tumours. The aim of this review is to show that the regulation of endocrine tumours by aberrant GPCR is not restricted to cortisol-secreting adrenal lesions, but also occurs in tumours of several other organs.

中文翻译：

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异常激素受体调节多种内分泌肿瘤


异常的 G 蛋白偶联受体 （GPCR） 表达在分泌皮质醇的原发性双侧大结节性肾上腺皮质增生症 （PBMAH） 和单侧腺瘤中高度普遍。多种 GPCR 及其配体的异常表达在各种内分泌肿瘤的过度功能中起重要作用。例如，原发性醛固酮增多症中 MC2R、5-HT4R、AVPR1A、LHCGR 和 GnRHR 的异常表达;嗜铬细胞瘤和副神经节瘤中的 GCGR、LHCGR 和 5-HT4R;垂体生长激素细胞瘤中的 TRHR、GnRHR、GIPR 和 GRP101;垂体促肾上皮质激素细胞瘤中的 AVPR2、D2DR 和 SSTR5;甲状腺髓样癌中的 GLP1R、GIPR 和生长抑素受体;以及其他神经内分泌肿瘤中的 SSTRs、GLP1R 和 GIPR。在分泌皮质醇的 PBMAH 和单侧腺瘤中导致 GIPR 异位表达的遗传机制已经确定，但其他内分泌肿瘤也涉及不同的机制。针对异常 GPCR 的功能成像开发应该有助于识别和针对这种广泛肿瘤的特异性治疗。本综述的目的是表明异常 GPCR 对内分泌肿瘤的调节不仅限于分泌皮质醇的肾上腺病变，还发生在其他几个器官的肿瘤中。