Variable expression of the V1 vasopressin receptor modulates the phenotypic response of steroid-secreting adrenocortical tumors.
Giorgio Arnaldi,Jean-Marie Gasc,Yves de Keyzer,Marie-Laure Raffin-Sanson,Véronique Perraudin,Jean-Marc Kuhn,Marie-Charles Raux-Demay,Jean-Pierre Luton,Eric Clauser,Xavier Bertagna +9 more
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TLDR
It is concluded that the vasopressin V1 receptor gene is expressed in normal and tumoral adrenocortical cells and high, and not ectopic, expression occurs in a minority of tumors that become directly responsive to vasoppressin stimulation tests.Abstract:
We studied the putative role of the vasopressin receptors in the phenotypic response of steroid-secreting adrenocortical tumors. A retrospective analysis of a series of 26 adrenocortical tumors responsible for Cushing’s syndrome (19 adenomas and 7 carcinomas) showed that vasopressin (10 IU, im, lysine vasopressin) induced an ACTH-independent cortisol response (arbitrarily defined as a cortisol rise above baseline of 30 ng/mL or more) in 7 cases (27%). In comparison, 68 of 90 patients with Cushing’s disease (76%) had a positive cortisol response. We then prospectively examined the expression of vasopressin receptor genes in adrenocortical tumors of recently operated patients (20 adenomas and 19 adrenocortical carcinomas). We used highly sensitive and specific quantitative RT-PCR techniques for each of the newly characterized human vasopressin receptors: V1, V2, and V3. The V1 messenger ribonucleic acid (mRNA) was detected in normal adrenal cortex and in all tumors. Its level varied widely between 2.0 × 102...read more
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Cushing's syndrome
TL;DR: The current understanding of pathogenesis, clinical features, diagnostic, and differential diagnostic approaches, and diagnostic algorithms and recommendations for management of Cushing's syndrome are reviewed.
Journal ArticleDOI
Ectopic and abnormal hormone receptors in adrenal Cushing's syndrome.
TL;DR: The mechanism by which cortisol is produced in adrenal Cushing's syndrome, when ACTH is suppressed, was previously unknown and was referred to as being “autonomous.” More recently several investigators have shown that some steroid-producing adrenal tumors or hyperplasias are under the control of ectopic (or aberrant, illicit, inappropriate) membrane hormone receptors.
Journal ArticleDOI
Leuprolide acetate therapy in luteinizing hormone--dependent Cushing's syndrome.
TL;DR: A woman with bilateral adrenal hyperplasia and corticotropin-independent Cushing's syndrome that was clinically manifested transiently during her pregnancies and became constant only after menopause is described, where the patient's cortisol secretion was stimulated by luteinizing hormone.
Journal ArticleDOI
ACTH-independent macronodular adrenal hyperplasia
TL;DR: The identification of aberrant receptors can offer a specific pharmacological approach to prevent progression and control abnormal steroidogenesis; alternatively, unilateral or bilateral adrenalectomy becomes the treatment of choice.
Journal ArticleDOI
Clinical and Genetic Heterogeneity, Overlap with Other Tumor Syndromes, and Atypical Glucocorticoid Hormone Secretion in Adrenocorticotropin-Independent Macronodular Adrenal Hyperplasia Compared with Other Adrenocortical Tumors
Hui-Pin Hsiao,Lawrence S. Kirschner,Isabelle Bourdeau,Margaret F. Keil,Sosipatros Boikos,Somya Verma,Audrey Robinson-White,Maria Nesterova,André Lacroix,Constantine A. Stratakis +9 more
TL;DR: AIMAH is a clinically and genetically heterogeneous disorder that can be associated with various genetic defects and aberrant hormone receptors; UFCs and other measures of adrenocortical activity can be misleadingly normal.
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