Adrenals in Ectopic ACTH Syndrome

PEDIATRICS ◽  
1971 ◽  
Vol 48 (2) ◽  
pp. 329-330
Author(s):  
N. S. Halmi ◽  
Gilbert S. Omenn
Keyword(s):  
Personal Experience ◽  
Ectopic Acth Syndrome ◽  
Elevated Plasma ◽  
Adrenal Hyperplasia ◽  
Plasma Acth ◽  
Ectopic Acth ◽  
Excellent Review ◽  
Bilateral Adrenal Hyperplasia ◽  
Crooke’S Hyalinization

In Dr. Gilbert S. Omenn's1 otherwise excellent review of ectopic hormone syndromes associated with tumors in childhood we find the following sentence: "Completely characterized cases of ectopic ACTH syndrome have bilateral adrenal hyperplasia, normal pituitary basophils, positive assay for ACTH in the tumor, and failure of suppression of elevated plasma ACTH by dexamethasone." I have no personal experience with the ectopic ACTH syndrome in childhood, but in three cases seen in adults the basophils (more accurately, the ACTH-MSH cells among them), far from being normal, showed the expected profound Crooke's hyalinization characteristic of severe hypercortisolism.2

Electron Microscopic investigation of crooke’s change in the pituitaries of patients with hypercorticism

1989 ◽  
Vol 47 ◽  
pp. 848-849
Author(s):  
E. Horvath ◽  
K. Kovacs ◽  
L. Stefaneanu ◽  
N. Losinski
Keyword(s):  
Nucleolar Organizer ◽  
Microscopic Investigation ◽  
Electron Microscopic Investigation ◽  
Ectopic Acth Syndrome ◽  
Nucleolar Organizer Regions ◽  
Electron Microscopic ◽  
Human Pituitary ◽  
Ectopic Acth ◽  
Crooke’S Hyalinization

Human pituitary corticotropins have unique morphologic markers: bundles of type-1 filaments, measuring approximately 70 A in width and representing cytokeratin. The extreme ring-like accumulation of type-1 filaments, known as Crooke's hyalinization, signals functional suppression of the corticotropins and occurs in endogenous and exogenous glucocorticoid excess, caused by ACTH-secreting pituitary adenoma, glucocorticoid secreting adrenocortical tumor, ectopic ACTH-syndrome and administration of pharmacologic doses of glucocorticoids. Cells of autonomous corticotroph adenomas usually do not show Crooke's hyalin change. A minority of these tumors, however, retains sensitivity to the negative feed-back effect of elevated blood glucocorticoid levels and display typical Crooke’s change.In the present study pituitary corticotropins in various phases of Crooke's hyalinization were investigated in patients with glucocorticoid excess of various origin, applying histology, immunocytochemistry, count of argyrophilic nucleolar organizer regions (AgNOR), and transmission electron microscopy.

TUMOUR AND PLASMA ACTH CONCENTRATIONS IN PATIENTS WITH AND WITHOUT THE ECTOPIC ACTH SYNDROME

1972 ◽  
Vol 1 (1) ◽  
pp. 27-44 ◽  
Author(s):  
J. G. RATCLIFFE ◽  
R. A. KNIGHT ◽  
G. M. BESSER ◽  
J. LANDON ◽  
A. G. STANSFELD
Keyword(s):  
Ectopic Acth Syndrome ◽  
Plasma Acth ◽  
Ectopic Acth

scholarly journals Cavernous Sinus Sampling Is Highly Accurate in Distinguishing Cushing’s Disease from the Ectopic Adrenocorticotropin Syndrome and in Predicting Intrapituitary Tumor Location1

1999 ◽  
Vol 84 (5) ◽  
pp. 1602-1610 ◽  
Author(s):  
Kathryn E. Graham ◽  
Mary H. Samuels ◽  
Gary M. Nesbit ◽  
David M. Cook ◽  
Oisin R. O’Neill ◽  
...  
Keyword(s):  
Cavernous Sinus ◽  
Cushing’S Disease ◽  
Tumor Location ◽  
Cushing's Disease ◽  
Ectopic Acth Syndrome ◽  
Plasma Acth ◽  
Catheter Position ◽  
Venous Flow ◽  
Ectopic Acth ◽  
Before And After

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. This study was designed to determine whether cavernous sinus sampling (CSS) is as safe and accurate as IPSS, whether CSS can eliminate the need for oCRH stimulation, and whether CSS can accurately predict the intrapituitary location of tumors. Ninety-three consecutive patients with ACTH-dependent Cushing’s syndrome were prospectively studied with bilateral, simultaneous CSS before and after oCRH stimulation. Prediction of a pituitary or ectopic ACTH source was based on cavernous/peripheral plasma ACTH ratios. Intrapituitary tumor location was predicted based on lateralization (side to side) ACTH ratios. These predictions were compared to surgical outcome in the 70 patients who had surgically proven pituitary (n= 65) or ectopic (n = 5) disease. CSS distinguished pituitary Cushing’s disease from the ectopic ACTH syndrome in 93% of patients with proven tumors before oCRH administration and in 100% of patients with proven tumors after oCRH. It was as safe and efficacious as published IPSS results. CSS accurately predicted the intrapituitary lateralization of the tumor in 83% of all patients and 89% of those patients with good catheter position and symmetric venous flow. CSS is as safe and accurate as IPSS for distinguishing patients with pituitary Cushing’s disease from those with the ectopic ACTH syndrome. In addition, CSS appears to be superior to IPSS for predicting intrapituitary tumor lateralization.

Concomitant Suppression of Plasma ACTH- and β-Endorphin-Like Immunoreactivity by Cyproheptadine, Naloxone, and Somatostatin in the Ectopic ACTH Syndrome

1985 ◽  
Vol 17 (04) ◽  
pp. 205-208 ◽  
Author(s):  
H. Nakashima ◽  
Y. Hirata ◽  
M. Uchihashi ◽  
T. Fujita ◽  
H. Taniguchi ◽  
...  
Keyword(s):  
Ectopic Acth Syndrome ◽  
Plasma Acth ◽  
Ectopic Acth

scholarly journals Intraoperative Adrenocorticotropin Levels During Transsphenoidal Surgery for Cushing’s Disease Do Not Predict Cure

1997 ◽  
Vol 82 (6) ◽  
pp. 1776-1779
Author(s):  
Kathryn E. Graham ◽  
Mary H. Samuels ◽  
Hershel Raff ◽  
Stanley L. Barnwell ◽  
David M. Cook
Keyword(s):  
Cushing’S Disease ◽  
Pituitary Tumor ◽  
Transsphenoidal Surgery ◽  
Tumor Resection ◽  
Cushing's Disease ◽  
Ectopic Acth Syndrome ◽  
Plasma Acth ◽  
Tumor Removal ◽  
Ectopic Acth ◽  
Percentage Decrease

Abstract Recently, intraoperative rapid immunochemiluminometric assay (ICMA) ACTH measurements have been used to evaluate the completeness of resection of ectopic ACTH-secreting tumors. This study evaluates whether this method can be applied to patients undergoing transsphenoidal surgery (TSS) for Cushing’s disease to predict complete pituitary tumor resection. Eighteen patients with Cushing’s disease undergoing TSS had plasma ACTH concentrations measured by a standard ICMA every 10 min for 1 h immediately after pituitary tumor removal. Patients were evaluated postoperatively for cure by standard criteria. ACTH levels were evaluated for percentage decrease from baseline at each time point. Patients who were cured (n = 11) had statistically greater decreases in ACTH levels (mean decrease 54%) than patients who were not (n = 7; 26% mean decrease, P < 0.04). By Receiver-Operator Characteristic (ROC) analysis, a reduction of at least 40% best predicted which patients were cured and which were not cured. This level of reduction was observed in 82% of cured patients, and a reduction of less than 40% was observed in 71% of those not cured. The analysis misclassified 4 of the 18 patients, resulting in a diagnostic accuracy of 78%. Although the mean maximal decrease in ACTH concentrations after tumor removal was significantly different between cured and not cured patients with Cushing’s disease, it was less dramatic than results in the previous ectopic ACTH study. This may relate to incomplete suppression and/or surgical manipulation of normal pituitary corticotrophs in patients with pituitary disease. In summary, in contrast to the ectopic ACTH syndrome, decline of plasma ACTH during TSS does not accurately predict complete tumor resection.

scholarly journals Ectopic ACTH syndrome in a dog with a mesenteric neuroendocrine tumour: a case report

2014 ◽  
Vol 59 (No. 7) ◽  
pp. 352-358 ◽  
Author(s):  
VA Castillo ◽  
PP Pessina ◽  
JD Garcia ◽  
P. Hall ◽  
MF Gallelli ◽  
...  
Keyword(s):  
Cushing Syndrome ◽  
Neuroendocrine Tumour ◽  
Clinical Signs ◽  
Ectopic Acth Syndrome ◽  
Histopathological Diagnosis ◽  
Left Adrenal Gland ◽  
Plasma Acth ◽  
Hormone Activity ◽  
Ectopic Acth ◽  
Ectopic Acth Secretion

Ectopic ACTH secretion is provoked by extra-pituitary tumours that secrete ACTH, constituting an infrequent type of Cushing Syndrome in the dog. Neuroendocrine tumours (NET) are characterised by the synthesis of peptides with hormone activity. A dog with clinical diagnosis of Cushing&rsquo;s syndrome and presenting an abdominal tumour located in the area of the left adrenal gland was sent to the hospital. Cortisol was not inhibited at four and eight hours after the application of low-dose dexamethasone and the cortisol/creatinine ratio was elevated (93 &times; 10<sup>-6</sup>, referencevalues &lt; 10 &times; 10<sup>&ndash;6</sup>). Plasma ACTH measurements were high (28.6 pmol/l, reference values 5.5&ndash;14.3 pmol/l). On computed tomography, the tumour was found in the meso-epigastrium, with both adrenal glands hyperplasic and no alteration of the pituitary image. The tumour was located between the two layers of the meso-colon and was removed using laparoscopy. After surgery, ACTH concentrations became normal and clinical signs remitted. The histopathological diagnosis was NET, with positive ACTH immunostaining.

scholarly journals MANAGEMENT OF ENDOCRINE DISEASE: Differential diagnosis, investigation and therapy of bilateral adrenal incidentalomas

2018 ◽  
Vol 179 (2) ◽  
pp. R57-R67 ◽  
Author(s):  
Isabelle Bourdeau ◽  
Nada El Ghorayeb ◽  
Nadia Gagnon ◽  
André Lacroix
Keyword(s):  
Differential Diagnosis ◽  
Hormone Secretion ◽  
Adrenal Incidentalomas ◽  
Adrenal Hyperplasia ◽  
Acth Secretion ◽  
Ectopic Acth ◽  
Ectopic Acth Secretion ◽  
Endocrine Disease ◽  
Bilateral Adrenal Hyperplasia ◽  
Hormonal Evaluation

The investigation and management of unilateral adrenal incidentalomas have been extensively considered in the last decades. While bilateral adrenal incidentalomas represent about 15% of adrenal incidentalomas (AIs), they have been less frequently discussed. The differential diagnosis of bilateral incidentalomas includes metastasis, primary bilateral macronodular adrenal hyperplasia and bilateral cortical adenomas. Less frequent etiologies are bilateral pheochromocytomas, congenital adrenal hyperplasia (CAH), Cushing’s disease or ectopic ACTH secretion with secondary bilateral adrenal hyperplasia, primary malignancies, myelolipomas, infections or hemorrhage. The investigation of bilateral incidentalomas includes the same hormonal evaluation to exclude excess hormone secretion as recommended in unilateral AI, but diagnosis of CAH and adrenal insufficiency should also be excluded. This review is focused on the differential diagnosis, investigation and treatment of bilateral AIs.

Comparative value of plasma ACTH and beta-endorphin measurement with three different commercial kits for the etiological diagnosis of ACTH-dependent Cushing's syndrome

1992 ◽  
Vol 126 (4) ◽  
pp. 308-314 ◽  
Author(s):  
A Tabarin ◽  
JB Corcuff ◽  
M Rashedi ◽  
A Navarranne ◽  
D Ducassou ◽  
...  
Keyword(s):  
Cushing’S Disease ◽  
Cushing's Disease ◽  
Molar Ratio ◽  
Ectopic Acth Syndrome ◽  
Discriminative Power ◽  
Plasma Acth ◽  
Ectopic Acth ◽  
Etiological Diagnosis ◽  
Commercial Kits ◽  
Better Than

Recent reports suggest that, contrary to radioimmunoassays (RIA), immunoradiometric assays (IRMA) artifactually decrease plasma ACTH levels in patients with the ectopic ACTH syndrome. Discrepancies between RIA and IRMA results may provide a means of discriminating this entity from Cushing's disease. We have compared the results of these two techniques, together with those of a β-endorphin assay, in 1 7 patients with Cushing's disease, 9 with the ectopic ACTH syndrome and 30 controls. ACTH-RIA and ACTH-IRMA levels in patients with Cushing's disease were similar (17.5±2.5 vs 15.1±2.8 pmol/l) and were correlated (rs=0.59, p<0.01). ACTH-RIA levels in patients with the ectopic ACTH syndrome were higher than ACTH-IRMA levels (27.3±2.9 vs 14.5±2.5, p<0.01) and these did not correlate. The ACTH-RIA and ACTH-RIA/ACTH-IRMA ratio levels in patients with the ectopic ACTH syndrome were higher than those of patients with Cushing's disease (p<0.01), but they overlapped with these in 27 and 31% of cases respectively. Plasma β-endorphin level was higher in patients with the ectopic ACTH syndrome than in patients with Cushing's disease (81.9±19.4 vs 26.4±5.6 pmol/l, p <0.01) and was correlated with ACTH only in patients with Cushing's disease. The overlap in β-endorphin and β-endorphin/ACTH-IRMA molar ratio levels between the two groups were 19 and 27% respectively. Although no parameter could be used to make clearcut distinction between Cushing's disease and the ectopic ACTH syndrome, the discriminative power of β-endorphin level was clearly better than that of the comparison between ACTH-RIA and ACTH-IRMA levels.

scholarly journals Malignant Gastric Carcinoid Causing Ectopic ACTH Syndrome: Discrepancy of Plasma ACTH Levels Measured by Different Immunoradiometric Assays

2005 ◽  
Vol 52 (6) ◽  
pp. 743-750 ◽  
Author(s):  
Kyoichiro TSUCHIYA ◽  
Isao MINAMI ◽  
Toru TATENO ◽  
Hajime IZUMIYAMA ◽  
Masaru DOI ◽  
...  
Keyword(s):  
Gastric Carcinoid ◽  
Ectopic Acth Syndrome ◽  
Plasma Acth ◽  
Ectopic Acth

scholarly journals Bilateral adrenal incidentalomas and NR3C1 mutations causing glucocorticoid resistance: is there an association?

2018 ◽  
Vol 179 (5) ◽  
pp. C1-C4 ◽  
Author(s):  
Nicolas C Nicolaides ◽  
George P Chrousos
Keyword(s):  
Glucocorticoid Receptor ◽  
Target Genes ◽  
Point Mutations ◽  
Clinical Manifestations ◽  
Glucocorticoid Resistance ◽  
Genetic Defects ◽  
Adrenal Incidentalomas ◽  
Adrenal Hyperplasia ◽  
Plasma Acth ◽  
Bilateral Adrenal Hyperplasia

Glucocorticoids signal through their cognate, ubiquitously expressed glucocorticoid receptor (GR), which influences the transcription of a large number of target genes. Several genetic defects, including point mutations, deletions or insertions in the NR3C1 gene that encodes the GR, have been associated with familial or sporadic generalized glucocorticoid resistance or Chrousos syndrome. One of the clinical manifestations of this rare endocrine condition is bilateral adrenal hyperplasia due to compensatory elevations of plasma ACTH concentrations. In this commentary, we discuss the interesting findings of the recently published French MUTA-GR study and present our perspective on the evolving field of NR3C1 pathology.

Sign in / Sign up

Export Citation Format

Share Document