scholarly journals Can Extra Adrenal Cortisol Secretion Occur in an Adrenalectomized Patient?

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A121-A122
Author(s):  
Sahar Iftikhar ◽  
Emma Margaret Bingham

Abstract Background: Established medical practice is that adrenalectomized patients require lifelong steroid replacement. We report a case which challenges that assumption. Clinical Case: A 38 year old female underwent transsphenoidal resection of a proven ACTH secreting pituitary adenoma. Post operatively her pituitary function was preserved, but due to residual tumour activity and the desire to preserve fertility, she had a bilateral adrenalectomy one year later and commenced hydrocortisone replacement. Subsequent pituitary imaging did not show recurrence of the tumour and her visual fields were stable, however her ACTH remained elevated at 100 and 130ng/L (09:00 <50 ng/L) despite steroid replacement.14 years after adrenalectomy she noticed weight gain and increased body hair. She also reported sometimes missing her hydrocortisone due to her busy job without ill effects. Her 9 am cortisol pre-hydrocortisone was elevated at 333 nmol/L (9am: 140–690 nmol/L) with ACTH of 203.0 ng/L (09:00 <50ng/L) She was able to suppress cortisol normally after a Dexamethasone suppression test. CT of her adrenals found no adrenal tissue. By now she had weaned herself off hydrocortisone, keeping an emergency supply. SST showed a cortisol of 320 nmol/L at 30 minutes, 360 nmol/L at 60 minutes with an elevated ACTH of 140 ng/L (09:00 <50 ng/L). Her weight gain and body hair growth stabilised. Discussion: We have demonstrated that this adrenalectomized patient has ACTH driven endogenous cortisol secretion which may be due to residual adrenal tissue due to seeding after surgery or the presence of steroid synthesis elsewhere. Extra adrenal sources for glucocorticoid production are known such as skin, gonads and thymus. However, the levels are insufficient to mount a significant stress response. There is evidence of adrenal regeneration in adrenalectomized animals. The regeneration is primarily of the adrenal cortex and does not involve the medulla. There has been one case report in literature of a 11 year old German boy who had adrenal regeneration detected on adrenal scintigraphy (Bilateral normal adrenal glands with normal activity) 13 years after adrenalectomy for Cushing’s disease. References: Gotlieb N, Albaz E, Shaashua L, et al. Regeneration of Functional Adrenal Tissue Following Bilateral Adrenalectomy. Endocrinology. 2018;159(1):248–259. doi:10.1210/en.2017-00505 Taves MD, Gomez-Sanchez CE, Soma KK. Extra-adrenal glucocorticoids and mineralocorticoids: evidence for local synthesis, regulation, and function. Am J Physiol Endocrinol Metab. 2011;301(1):E11-E24. doi:10.1152/ajpendo.00100.2011

1985 ◽  
Vol 110 (3) ◽  
pp. 296-301
Author(s):  
Axel Overlack ◽  
Miyoko Higuchi ◽  
Rainer Kolloch ◽  
Hans-Michael Müller ◽  
Klaus O. Stumpe ◽  
...  

Abstract. It has been suggested that the renal kallikreinkinin system is dependent on mineralocorticoid activity. This hypothesis was studied in a patient with cyclic Cushing's syndrome combined with cortisol suppressible, dexamethasone non-suppressible ACTH secretion. The 24-h urinary excretions of sodium, potassium, cortisol, active and inactive kallikrein, aldosterone, and prostaglandin E2 (PGE2) were studied during normal and excessive cortisol secretion and after bilateral adrenalectomy. Kallikrein, PGE2 and potassium rose during cortisol overproduction while aldosterone and sodium decreased. Kallikrein, PGE and potassium were positively related to cortisol excretion, whereas urinary aldosterone and sodium showed a negative relationship to cortisol. Kallikrein was inversely related to aldosterone. Excretion of inactive kallikrein followed closely the changes of active kallikrein. During cortisol excess, as in our patient, the mineralocorticoid activity of cortisol will exceed that of aldosterone. This suggests that the alterations in kallikrein, aldosterone and PGE2 during cortisol excess in the present study were due to the mineralocorticoid potency of the steroid.


Author(s):  
Christian Pina ◽  
Ahmed Khattab ◽  
Philip Katzman ◽  
Lauren Bruckner ◽  
Jeffrey Andolina ◽  
...  

AbstractA 14-year-old female with classical congenital adrenal hyperplasia because of 21-hydroxylase deficiency underwent bilateral adrenalectomy at 6 years of age as a result of poor hormonal control. Because the patient was adrenalectomized, extra adrenal androgen production was suspected. Imaging studies including pelvic ultrasound and pelvic magnetic resonance imaging (MRI) were obtained to evaluate for adrenal rest tumors of the ovaries. Abdominal MRI was obtained to evaluate for residual adrenal tissue. A cystic lesion arising from her right ovary suspicious for ovarian neoplasm was noted on pelvic MRI. Right salpingo-oophorectomy was performed and histopathological examination revealed ovarian serous adenocarcinoma, low-grade, and well-differentiated. Tumor marker CA-125 was elevated and additional ovarian cancer staging workup confirmed stage IIIC due to one lymph node positive for carcinoma. The patient then developed a large left ovarian cyst, which led to a complete total abdominal hysterectomy and removal of the left ovary and fallopian tube. Pathology confirmed ovarian serous adenocarcinoma with microscopic focus of carcinoma in the left ovary. After numerous complications, the patient responded well to chemotherapy, CA-125 levels fell and no evidence of carcinoma was observed on subsequent imaging. To our knowledge, this is the first reported case of an ovarian serous adenocarcinoma in a patient with CAH. Although rare, we propose that the ovaries were the origin of androgen production and not residual adrenal tissue. The relationship between CAH and ovarian carcinomas has yet to be established, but further evaluation is needed given the poor survival rate of high-grade serous ovarian carcinoma.


2021 ◽  
Vol 2021 ◽  
pp. 1-5
Author(s):  
W. G. P. Kanchana ◽  
P. A. D. M. Kumarathunga ◽  
Gajawathana Shakthilingham ◽  
Charles Antonypillai ◽  
Sonali Gunatilake ◽  
...  

Introduction. Synchronous bilateral adrenalectomy is undertaken less often due to numerous perioperative challenges and rare circumstances of patients needing this procedure. Bilateral adrenalectomy is an important second-line option for patients with persistent or recurrent hypercortisolism following transsphenoidal surgery for Cushing’s disease. Here, we present a challenging case of synchronous laparoscopic bilateral adrenalectomy for a young female patient with recurrent Cushing’s disease and fertility wishes. Case Presentation. A 21-year-old recently married patient who was diagnosed with Cushing’s disease with a pituitary microadenoma had undergone two attempts of transsphenoidal excision of the pituitary tumour. Follow-up evaluation showed an unresectable residual tumour with invasion of the intracavernous part of the left internal carotid artery. As the patient had the hypothalamic-pituitary-ovarian axis intact with strong fertility wishes, she was offered bilateral adrenalectomy instead of radiotherapy. She was prepared for the surgery with close perioperative support from the endocrinology and anaesthesia teams. Intravenous hydrocortisone infusion was started at the induction of anaesthesia. Transperitoneal approach was used with the patient positioned in left and right lateral positions for right and left glands, respectively. A meticulous surgical technique was used for the identification of adrenal veins to clip them before division followed by handling of the glands. The patient had minimal haemodynamic disturbances during surgery. Intraoperative blood loss was less than 100 ml, and operative time was 220 minutes. She had a gradual recovery following postoperative respiratory distress due to basal atelectasis and consolidation. Cortisol levels were less than 20 nmol/L postoperatively, suggesting successful surgical intervention. Two months after surgery, she continued on maintenance therapy of oral hydrocortisone and fludrocortisone and was encouraged to go ahead with pregnancy. Conclusion. Although bilateral adrenalectomy is considered a high-risk procedure, these risks can be mitigated and performed safely while maintaining close multidisciplinary perioperative support.


1972 ◽  
Vol 127 (5) ◽  
pp. 881-892 ◽  
Author(s):  
Gillian R. Bullock ◽  
Elizabeth E. Carter ◽  
P. Elliott ◽  
R. F. Peters ◽  
Phillida Simpson ◽  
...  

1. Five glucocorticoids, when administered daily to rats for 5–7 days at a dosage of 5mg/kg, were in the following order of effectiveness with respect to their ability to decrease the weight gain of whole animals and the vastus lateralis, vastus medialis and gluteus medius muscles: corticosterone<prednisolone<triamcinolone <betamethasone<dexamethasone<triamcinolone acetonide. 2. The low catabolic potencies of corticosterone and prednisolone were reflected in the high doses (20mg/kg) required to decrease significantly the incorporating activity in vitro of ribosomes isolated from the muscles 6h later. However, triamcinolone was as effective as triamcinolone acetonide in causing a significant effect at 0.05mg/kg, although it had a far lower catabolic potency than the acetonide. 3. The relationship between decrease in ribosomal activity in vitro and weight loss was better understood by considering the duration of the effect after single doses of steroids. Ribosomes returned to normal function between 6 and 12h after the administration of corticosterone (20mg/kg), but still had decreased activity 12h after a similar dose of prednisolone. Normal ribosomal function was renewed between 12 and 18h after the administration of triamcinolone and betamethasone at 5mg/kg, but decreased activity persisted for more than 48h after similar doses of dexamethasone or triamcinolone acetonide. 4. There was no difference in the rate of incorporation of amino acids into nascent protein on polyribosomes from muscle of control animals and animals treated 6h previously with triamcinolone acetonide or dexamethasone. 5. The rate of weight gain of the heart was not affected by any of the steroids tested and heart ribosomes maintained normal activity although concentrations of steroids in this organ 5min after administration were 2–3 times that in skeletal muscle. 6. Mitochondria, isolated from the muscle of animals that had received triamcinolone acetonide (20mg/kg) 12h previously, were shown, by using a Coulter counter, to be enlarged. Nevertheless oxidation was only slightly uncoupled 12h after drug administration and they had normal function 6h later. Similar results with respect to mitochondrial function were obtained after the administration of dexamethasone (20mg/kg) and betamethasone (20mg/kg). 7. From these results it is concluded that the mitochondria are not functionally involved in the early phase of steroid-induced muscle catabolism. The observed decrease in the incorporating ability of muscle ribosomes in vitro appears to be much more closely linked to the initial catabolic event.


1967 ◽  
Vol 37 (2) ◽  
pp. 139-146 ◽  
Author(s):  
BARBARA J. WHITEHOUSE ◽  
G. P. VINSON ◽  
P. A. JANSSENS

SUMMARY Incubation of hamster adrenal tissue in Krebs bicarbonate Ringer solution gave rapid conversion of added [4-14C]cortisol to cortisone. Moreover, incubation in Ringer solution with [4-14C]progesterone as precursor invariably yielded cortisone in greater amounts than cortisol. On the other hand it has been confirmed that cortisol is the major free ultraviolet absorbing steroid in the adrenal venous blood of the hamster. Incubation of adrenal tissue with [4-14C]progesterone in hamster whole blood gave relatively greater amounts of cortisol which were more in keeping with the findings in vivo. This suggested that the blood contains factors which affect the cortisone-cortisol equilibrium by modifying adrenal enzyme activity. Some reduction of [3H] cortisone to cortisol was also observed during incubation with blood alone.


1987 ◽  
Vol 64 (1) ◽  
pp. 10-16 ◽  
Author(s):  
MITSUHIDE NARUSE ◽  
KOICHI OBANA ◽  
KIYOKO NARUSE ◽  
HAJIME YAMAGUCHI ◽  
HIROSHI DEMURA ◽  
...  

Author(s):  
Julian B. Wilson ◽  
Mohan Zopey ◽  
Jaimie Augustine ◽  
Randolph Schaffer ◽  
Manfred Chiang ◽  
...  

AbstractBilateral adrenalectomy (BLA) is a treatment option for patients with Cushing’s Disease (CD) if transsphenoidal pituitary surgery fails or is not a therapeutic option. For most patients, BLA eliminates endogenous glucocorticoid and mineralocorticoid production, but for a small number of patients, endogenous secretion of adrenal hormones from adrenal tissue continues or recurs, leading to signs and symptoms of hypercortisolism. If adrenal tissue is confined to the adrenal bed, it is considered adrenal remnant tissue, while if it is outside the adrenal bed, it is considered adrenal rest tissue. We retrospectively evaluated morning serum cortisol, nighttime serum cortisol, nighttime salivary cortisol, and 24-h urine free cortisol on at least three occasions in 10 patients suspected of having endogenous cortisol production. Imaging of adrenal remnant tissue was also reviewed. Ten of 51 patients who underwent BLA during this time period had adrenal remnant/rest tissue marked by detectable endogenous glucocorticoid production; 9 of the 10 patients had signs and symptoms of hypercortisolism. Localization and treatment proved difficult. We conclude that the incidence of adrenal remnant/rest tissue in those undergoing BLA following unsuccessful pituitary surgery was 12% although there may have been a selection bias affecting this prevalence. The first indication of remnant tissue occurrence is a reduction in glucocorticoid replacement with symptoms of hypercortisolism. If this occurs, endogenous cortisol production should be tested for by cortisol measurements using a highly specific cortisol assay while the patient is taking dexamethasone or no glucocorticoid replacement. Endocrinologists need to monitor the development of both adrenal remnant tissue and Nelson’s syndrome following BLA.


1971 ◽  
Vol 68 (2) ◽  
pp. 209-218 ◽  
Author(s):  
K. Kurachi ◽  
M. Miyazaki ◽  
H. Mori ◽  
K. Matsumoto

ABSTRACT Adrenal tissue homogenates from foetal rabbits in late gestation and their mothers were incubated with [7α-3H] pregnenolone and analyzed for conversion products by reverse isotope dilution procedures. Deoxycorticosterone and corticosterone were synthesized as main products by foetal as well as by adult adrenals. In addition, small amounts of progesterone, 17-hydroxypregnenolone and cortisol were formed by both adrenals. No C19-steroids and 16α-hydroxysteroids could be found. The results indicate a similarity in the qualitative metabolic pattern of pregnenolone by adrenal cortical tissue of late gestation rabbit foetuses to that of adult rabbits. However, the activity of 3β-hydroxysteroid dehydrogenase per gram tissue in the foetal rabbit adrenal was of the order of one tenth that in the adrenals of their mothers. By using [3H] progesterone as substrate, the activities of 21-hydroxylase and 11β-hydroxylase in the foetal adrenal were similarly demonstrated to be less than that of the adrenal of the mother.


1976 ◽  
Vol 15 (06) ◽  
pp. 297-299
Author(s):  
O. A. Chomicki ◽  
W. Hartwig ◽  
A. Mikołajków ◽  
H. Siwicki ◽  
A. Kasperlik-Zaluska

SummaryA new adrenal scanning agent, Adosterol (131I-6β-iodomethyl-19-norcholest-5(10)-en-3β-ol) has been evaluated in three female patients with hypercortic-ism. In one case, characterised by recurrent adrenocortical hyperactivity after bilateral adrenalectomy, a remnant of adrenal tissue was detected. In two other cases, the presence of an adrenal tumour could be excluded. The scan made on the 6th day after the administration of Adosterol was found to be superior in quality to that made on the 13th day.


1980 ◽  
Vol 95 (4) ◽  
pp. 546-552 ◽  
Author(s):  
E. Marelyn Wintour ◽  
J. P. Coghlan ◽  
K. H. Hardy ◽  
D. P. Hennessy ◽  
Barbara E. Lingwood ◽  
...  

Abstract. The adrenal fails to develop in the hypophysectomised ovine foetus but adrenal growth, maturation and increased cortisol secretion occur in the intact foetus whilst plasma immunoreactive ACTH remains at a constant value. The presence of a feedback relationship between foetal adrenal and pituitary was examined in 8 ovine foetuses which were bilaterally adrenalectomised (adrx.) at the time of implantation of catheters in the carotid artery and jugular vein (90–127 days of gestation). Ten chronically cannulated foetuses served as controls. Gestation length in these sheep was 147 ± 5 days. Plasma immunoreactive (IR) ACTH (IR.ACTH) was measured with a Sorin Biomedica kit. The antibody cross-reacts with ACTH1–39, and ACTH1–24, but not with ACTH1–13, ACTH18–39, β-LPH or β-endorphin. Cortisol (F) and corticosterone (B) were measured by a double isotope dilution derivative assay. In intact foetuses the IR.ACTH was 69.6 ± 7.1 pg/ml (mean ± sem, n = 37) until the day before parturition when values increased to 326 ± 60 pg/ml (n = 6). In adrx. foetuses of 91–121. 122–135 and > 136 days gestation the IR.ACTH values were 50.3 ± 4 (n = 14), 444 ± 46 (n = 26) and 1155 ± 175 (n = 6) pg/ml, respectively. The foetal F and B concentrations were 0.12 ± 0.027 μg/100 ml and 40 ± 6 ng/100 ml (n = 14), respectively, in adrx. foetuses. These values are not significantly different from those in intact foetuses younger than 135 days. The mean F and B concentrations in adrx. foetuses were 22 ± 4.3% (n = 14) and 49 ± 5.4% (n = 14) of the respective maternal values. In summary prior to 120 days the IR.ACTH, F and B concentrations are similar in intact and adrx. foetuses. After 122 days the IR.ACTH secretion by the foetal pituitary first begins to exhibit a feedback relationship with some adrenal secretion (?not F or B) and after 130–135 days some subtle change in pituitary sensitivity to F and/or B may operate. It is suggested that about 120 days a significant alteration in the foetal pituitary capacity for ACTH synthesis occurs.


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