Disease relevance of ACTH Syndrome, Ectopic

• In addition, the treatment of such conditions as the large ACTH-producing pituitary tumor, Nelson's syndrome, the malignant ectopic ACTH syndrome, and adrenal carcinoma is not entirely satisfactory [1].
• Other types of nonpituitary tumors responsible for ectopic ACTH syndrome presented much lower levels of both POMC and V3 receptor gene expression than those found in ACTH-secreting bronchial carcinoids [2].
• A patient with functioning islet cell carcinoma is described who had amelioration of her hypoglycemia during the development of ectopic ACTH syndrome [3].
• While the clinical picture was consistent with the ectopic ACTH syndrome, the biochemical pattern was that of pituitary ACTH-dependent hypercortisolism [4].
• The diagnosis of ectopic ACTH syndrome was first entertained when hypokalemic alkalosis was observed during evaluation of her carcinoma [5].

High impact information on ACTH Syndrome, Ectopic

• Therefore, CRH and Dex tests have limited usefulness in differentiating between Cushing's disease and ectopic ACTH syndrome [6].
• Ectopic ACTH syndrome caused by a bronchial carcinoid tumor responsive to dexamethasone, metyrapone, and corticotropin-releasing factor [7].
• The main analysis was limited to 45 cases with confirmed diagnosis: 44 with pituitary dependency, proven by confirmatory histology and/or significant biochemical improvement after pituitary surgery, and 1 with ectopic ACTH syndrome [8].
• One explanation for the mineralocorticoid excess state of the ectopic ACTH syndrome appears to be that cortisol gains inappropriate access to the mineralocorticoid receptor through failure of its normal metabolism by 11 beta HSD [9].
• Two measures of the peripheral metabolism of cortisol were found to be markedly decreased in two patients with the ectopic ACTH syndrome [10].

Chemical compound and disease context of ACTH Syndrome, Ectopic

• The pituitary V3 vasopressin receptor and the corticotroph phenotype in ectopic ACTH syndrome [2].
• These values were comparable to that found in the syndrome of apparent mineralocorticoid excess and consistent with the hypothesis that cortisol is a significant functioning mineralocorticoid in the ectopic ACTH syndrome [10].
• These data indicate that octreotide can be a highly effective treatment for patients with the ectopic ACTH syndrome [11].
• All patients were receiving high dose steroid therapy except one who had ectopic ACTH syndrome [12].
• The more severe mineralocorticoid manifestations in the ectopic ACTH syndrome compared to pituitary Cushing's disease have been attributed to hypersecretion of 11-deoxycorticosterone [10].

Anatomical context of ACTH Syndrome, Ectopic

• In the patient with the ectopic ACTH syndrome, ratios of central to peripheral ACTH were negative in jugular vein samples and petrosal sinus samples before and after CRH (< 2 and < 3, respectively) [13].
• The small cell lung carcinoma (SCLC) cell line DMS-79 has been used as a model for studying the molecular mechanism underlying the ectopic ACTH syndrome [14].
• Medullary thyroid carcinoma (MTC) rarely causes ectopic ACTH syndrome [15].
• Pseudo-tumours of the thymus after correction of hypercortisolism in patients with ectopic ACTH syndrome: a report of five cases [16].
• Exogenous steroid therapy for leukaemia seemed to suppress the clinical manifestations of the ectopic ACTH syndrome while subsequent endogenous steroid production controlled the peripheral lymphocyte count [17].

Gene context of ACTH Syndrome, Ectopic

• The high UFF/UFE ratio in the mineralocorticoid excess state seen in the ectopic ACTH syndrome is compatible with substrate-saturation of renal 11 beta-HSD2 [18].
• Somatostatin radioanalog scintigraphy should be considered as a new investigative tool in patients with suspected ectopic ACTH syndrome [19].
• In patients with Nelson's syndrome and those with ectopic ACTH syndrome, almost no intact hNT (less than 7% of the total) was present in urine; most of the IR-hNT appeared in the elution volumes with an apparent mol wt of 8-10K [20].
• Mineralocorticoid excess and inhibition of 11 beta-hydroxysteroid dehydrogenase in patients with ectopic ACTH syndrome [21].
• Patients with SCC and ectopic ACTH syndrome have a poor prognosis [22].

Analytical, diagnostic and therapeutic context of ACTH Syndrome, Ectopic

• The intraoperative measurement of ACTH by immunoradiometric assay in five patients with the occult ectopic ACTH syndrome during removal of suspicious intrapulmonary lesions is reported [23].
• Inferior petrosal sinus sampling (IPSS) is used to distinguish pituitary Cushing's disease from occult cases of the ectopic ACTH syndrome, but is limited in that it requires the use of ovine CRH (oCRH) and is not highly accurate at predicting the intrapituitary location of tumors [24].
• The RIA-ACTH in a normal human pituitary, 2 ectopic ACTH-secreting tumors and plasma from a patient with Nelson's syndrome and one with ectopic ACTH syndrome was divided into 3 molecular weight classes after gel exclusion chromatography [25].
• Therefore, RIA may still be necessary for differential diagnosis of ectopic ACTH syndromes [26].
• The third underwent bilateral adrenalectomy, and no evidence of ectopic ACTH syndrome has emerged as yet after 4 years follow-up [27].

References