scholarly journals Co Existence of a Corticotroph Adenoma and a Multifunctional Pituitary Cyst

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A578-A578
Author(s):  
David John McDonnell ◽  
Carmel Kennedy ◽  
Yazan Qaoud ◽  
Karen Heverin ◽  
Diarmuid Smith ◽  
...  
Keyword(s):  
Urinary Free Cortisol ◽  
Needle Aspiration ◽  
Solid Component ◽  
High Dose ◽  
Pituitary Hormone ◽  
Cystic Fluid ◽  
Corticotroph Adenoma ◽  
High Dose Dexamethasone ◽  
Free Cortisol ◽  
Pituitary Cyst

Abstract The co-existence of a corticotroph adenoma and a pituitary cyst is very unusual. We present the case of a 50-year-old female who presented with a Cushingoid phenotype, severe hypokalaemia, hyperglycaemia and hypertension. Urinary free cortisol was markedly elevated at 50 fold. ACTH levels were elevated at 121.4 pg/ml. She failed both the low and high dose dexamethasone suppression tests. The CRF test did not show a satisfactory rise in ACTH levels but the Inferior petrosal sinus sampling revealed a central to peripheral ACTH gradient highly suggestive of Pituitary dependant Cushing’s. MRI pituitary revealed a large cystic lesion with a small solid component. Computed Tomography (CT) of thorax abdomen and pelvis was normal. A trans-sphenoidal hypophysectomy was performed, during which a fine needle aspiration of the intra-cystic fluid was obtained. This showed markedly elevated pituitary hormone levels of ACTH (1399pg/ml), prolactin (353,084mIU/L), TSH (217IU/L) FSH (205mIU/ml) and GH (519 ng/ml) consistent with a multifunctional pituitary cyst. Neuropathology of the solid component confirmed a corticotroph adenoma. Post-operative am cortisol levels were persistently suppressed to less than 50 nmol/l with marked improvement in clinical features. This case highlights the challenges in the work up of Cushing’s syndrome and the limitations of diagnostic tests. The coexistence of a corticotroph adenoma and a multifunctional pituitary cyst is very unusual and to our knowledge has not been reported before.

scholarly journals MON-483 Familial Dysalbuminemic Hyperthyroxinemia with False Hypercortisolemia

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Koki Chiba ◽  
Hiraku Kameda ◽  
Hiroshi Nomoto ◽  
Kyu Yong Cho ◽  
Akinobu Nakamura ◽  
...  
Keyword(s):  
Clinical Endocrinology ◽  
Urinary Free Cortisol ◽  
High Dose ◽  
Acth Level ◽  
Factors Affecting ◽  
High Dose Dexamethasone ◽  
First Case ◽  
Free Cortisol ◽  
Control Serum ◽  
Dominant Disease

Abstract Background: Familial dysalbuminemic hyperthyroxinemia (FDH) is an autosomal dominant disease characterized by hyperthyroxinemia without symptoms of thyrotoxicosis, due to a high affinity of mutant albumin for thyroxine. No report has described cortisol-albumin binding in FDH patient, and here we present a case of FDH presenting with false hypercortisolemia. Clinical Case: A 46-year man, previously diagnosed with FDH by genetic test (1), was pointed out hypercortisolemia with normal ACTH level (ACTH 64.7 pg/mL, Cortisol 34.7 μg/dL) during the investigation for Parkinson’s syndrome and referred to our department for further examination. Cortisol was 7.1 μg/dL at midnight, 14.9 μg/dL after low dose dexamethasone overnight test, and DDAVP test was positive. ACTH and cortisol responded to CRH load, although basal and peak cortisol levels were high (35.4 and 53.9 μg/dL, respectively). High dose dexamethasone overnight test showed suppressed ACTH and cortisol, and MRI showed no obvious pituitary adenoma. In spite of significant high cortisol level, no Cushing sign’s or metabolic abnormalities were observed and urinary free cortisol was within the normal range (30.7 μg/day), suggesting the presence of factors affecting the laboratory testing. We removed albumin from serum with immunoprecipitation using anti-albumin antibody and measured cortisol with LC-MS/MS. The decrease of cortisol was 4% in control serum but 38% in the patient serum after removing albumin, suggesting the binding rate of cortisol to mutant albumin in the patient was increased, leading to false hypercortisolemia. Conclusion: This is the first case demonstrating the false hypercortisolemia in a FDH patient. Clinicians should consider the possibility of the abnormal cortisol binding to albumin in differential diagnosis of hypercortisolemia with normal ACTH level. Reference: (1) Norio Wada, et al: A Novel Missense Mutation in Codon 218 of the Albumin Gene in a Distinct Phenotype of Familial Dysalbuminemic Hyperthyroxinemia in a Japanese Kindred. Journal of Clinical Endocrinology and Metabolism 1997;82;3246–3250

scholarly journals Bilateral Adrenal Hyperplasia as a Possible Mechanism for Hyperandrogenism in Women With Polycystic Ovary Syndrome

2016 ◽  
Vol 101 (9) ◽  
pp. 3353-3360 ◽  
Author(s):  
Evgenia Gourgari ◽  
Maya Lodish ◽  
Meg Keil ◽  
Ninet Sinaii ◽  
Evrim Turkbey ◽  
...  
Keyword(s):  
Polycystic Ovary ◽  
Hormone Secretion ◽  
Urinary Free Cortisol ◽  
Adrenocortical Function ◽  
High Dose ◽  
High Dose Dexamethasone ◽  
Free Cortisol ◽  
Before And After ◽  
Pcos Group ◽  
Adrenal Volume

Abstract Context: Androgen excess may be adrenal and/or ovarian in origin; we hypothesized that a subgroup of patients with polycystic ovarian syndrome (PCOS) may have some degree of abnormal adrenocortical function. Objective: The objective of the study was to evaluate the pituitary adrenal axis with an oral low- and high-dose dexamethasone-suppression test (Liddle's test) in women with PCOS. Design: This was a case-control study. Setting: The study was conducted at the National Institutes of Health Clinical Center. Participants: A total of 38 women with PCOS and 20 healthy volunteers (HV) aged 16–29 years participated in the study. Main Outcome Measures: Urinary free cortisol (UFC) and 17-hydroxysteroids (17OHS) before and after low- and high-dose dexamethasone and assessment of adrenal volume by computed tomography scan were measured. Results: Twenty-four-hour urinary 17OHS and UFC were measured during day 1 to day 6 of the Liddle's test. Baseline UFC levels were not different between PCOS and HVs; on the day after the completion of high-dose dexamethasone administration (d 6), UFC was higher in the PCOS group (2.0 ± 0.7 μg/m2·d) than the HV group (1.5 ± 0.5) (P = .038). On day 5, 17OHS and UFC were negatively correlated with adrenal volumes (left side, rp = −0.47, P = .009, and rp = −0.61, P < .001, respectively). PCOS patients above the 75th percentile for UFC and/or 17OHS after high-dose dexamethasone (n = 15) had a significantly smaller total adrenal volume (6.9 ± 1.9 cm3 vs 9.2 ± 1.8 cm3, P = .003) when compared with the remaining PCOS patients (n = 22), but they did not have worse insulin resistance or hyperandrogenism. Conclusions: In a subset of young women with PCOS, we detected a pattern of glucocorticoid secretion that mimicked that of patients with micronodular adrenocortical hyperplasia: they had smaller adrenal volumes and higher steroid hormone secretion after dexamethasone compared with the group of PCOS with appropriate response to dexamethasone.

Use of In-111 pentetreotide scintigraphy in the diagnosis of a midgut carcinoid causing Cushing's syndrome

1997 ◽  
pp. 79-83 ◽  
Author(s):  
VB Segu ◽  
DM Mahvi ◽  
MA Wilson ◽  
SJ Hale ◽  
TF Warner ◽  
...  
Keyword(s):  
Carcinoid Tumor ◽  
Cushing’S Syndrome ◽  
Single Photon ◽  
Photon Emission ◽  
Urinary Free Cortisol ◽  
Cushing's Syndrome ◽  
High Dose ◽  
High Dose Dexamethasone ◽  
Malignant Carcinoid ◽  
Free Cortisol

A 57-year-old man presented with clinical features of hypercortisolism and was diagnosed with ACTH-dependent Cushing's syndrome. Biochemical testing showed partial suppression of urinary free cortisol with high dose dexamethasone. Initial computed tomography (CT) of the chest and abdomen, and magnetic resonance imaging of the pituitary were negative. In-111 pentetreotide scintigraphy with single photon emission computerized tomography revealed two 'hot' lesions in the abdomen which were then confirmed by subsequent directed thin-slice abdominal CT and small bowel barium study. At surgery, two segments of ileum, adjoining mesentery and lymph nodes were resected. Histopathology was consistent with a malignant carcinoid tumor of the ileum which stained intensely for ACTH. Plasma ACTH, and serum and urinary cortisol normalized postoperatively. To our knowledge, this is the first reported case of ileal carcinoid tumor causing Cushing's syndrome with premortem diagnosis. Another unique feature of this case is that In-111 pentetreotide scan provided the decisive clue to localization of the tumor.

scholarly journals Ectopic Acth-Producing Pulmonary Carcinoid: A Rare Case of Simulating the Cushing’s disease

2020 ◽  
Vol 3 (4) ◽  
pp. 01-04
Author(s):  
Manouchehr Nakhjavan
Keyword(s):  
Cushing’S Syndrome ◽  
Mediastinal Lymph Node ◽  
Left Lung ◽  
Cushing's Syndrome ◽  
Whole Body ◽  
High Dose ◽  
Ectopic Acth ◽  
Pulmonary Carcinoid ◽  
High Dose Dexamethasone ◽  
Free Cortisol

Excess amounts of blood glucocorticosteroids, either exogenous or endogenous, results in Cushing’s syndrome (CS). the current study presents a 45-year-old man, ACTH dependent Cushing’s syndrome. The basal cortisol and urine free cortisol level were significantly suppressed following high dose dexamethasone suppression test and pituitary MRI showed microadenoma with a size of less than 6 mm. After bilateral inferior petrosal sinus sampling, we investigated the ectopic source of ACTH production. The computed tomography revealed a mediastinal lymph node, and the whole body octreotide scan revealed octreotide avid lesion in the medial aspect of the left lung. The patient underwent left lung lobectomy and the pathological study indicated the carcinoid tumor of the left lung.

scholarly journals The pituitary–adrenal axis in adult thalassaemic patients

2010 ◽  
Vol 162 (1) ◽  
pp. 43-48 ◽  
Author(s):  
Massimo Scacchi ◽  
Leila Danesi ◽  
Agnese Cattaneo ◽  
Elena Valassi ◽  
Francesca Pecori Giraldi ◽  
...  
Keyword(s):  
Serum Cortisol ◽  
Urinary Free Cortisol ◽  
Stressful Events ◽  
Adrenocortical Function ◽  
High Dose ◽  
Plasma Acth ◽  
Mean Values ◽  
Free Cortisol ◽  
Basal Plasma ◽  
The Mean

ObjectiveWe previously described in young thalassaemic patients an altered cortisol and ACTH responsiveness suggesting an impaired adrenocortical reserve. Owing to iron overload, a worsening of adrenal function should be expected in adult patients.DesignIn 124 adults with β-thalassaemia, urinary free cortisol (UFC) and plasma ACTH levels were determined and compared with those measured in 150 controls. In 45 patients, cortisol was measured in response to: i) tetracosactide 1 μg as an i.v. bolus (low-dose test, LDT) and ii) tetracosactide 250 μg infused i.v. over 8 h (high-dose test, HDT).ResultsUFC and serum cortisol were within the reference range in all patients. Conversely, basal plasma ACTH values were above the upper limit of the normal range in 19 patients. There were no statistically significant differences in the mean values of UFC, basal serum cortisol and plasma ACTH between patients and controls. A subnormal cortisol response to the LDT was registered in 18 out of 56 patients. Three of these patients also displayed a subnormal response to the HDT, together with elevated baseline plasma ACTH levels. In the LDT, a positive correlation was found between basal and peak cortisol values (P<0.0001). The latter were negatively correlated with basal ACTH values in both LDT (P<0.0001) and HDT (P<0.0001).ConclusionsAdult thalassaemic patients often present a subtle impairment of adrenocortical function. This may become clinically relevant in case of major stressful events. Thus, we recommend an assessment of adrenocortical function in all adult thalassaemic patients.

scholarly journals MON-055 A Rare Case of a Macroadenoma Causing Cushing Syndrome in a Pediatric Patient

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Erica Patel ◽  
Marc Weigensberg
Keyword(s):  
Pediatric Patient ◽  
Cushing Syndrome ◽  
Pubertal Development ◽  
Urinary Free Cortisol ◽  
High Dose ◽  
Pituitary Macroadenoma ◽  
Central Adiposity ◽  
Rapid Weight Gain ◽  
Presenting Symptoms ◽  
Free Cortisol

Abstract Background: Pediatric Cushing Syndrome, a rare diagnosis, is almost always caused by pituitary microadenomas less than 5 mm in children older than 6 years old. We report on a 9-year-old girl with a 2.3 cm pituitary macroadenoma, whose ACTH and urinary 24-hour free cortisol were the highest recorded at our institution. Clinical Case: A 9-year-old pre-pubertal female presented with six months of frontal headaches, rapid weight gain, and hirsutism. Two months prior she developed fatigue and proximal muscle weakness and pain. On physical exam, she had plethoric round facies with acne and hirsutism, dorsal fat pad, central adiposity, and violet colored abdominal striae. Her pubertal development was tanner stage 3 for breast and 2 for pubic hair. BMI was 95th percentile and height was 40th percentile, previously 75th and 50th percentile respectively one year prior. 24 hour urinary free cortisol was 40,650 mcg/day [normal:100 mcg/day]. A 48 hour high dose dexamethasone suppression test was done as it is the most accurate in pediatric patients over 40 kg, morning cortisol was 100 mcg/dL [normal: 5-20 mcg/dL], ACTH 868 pg/ml [normal: 9-57 pg/ml], 24 Urinary Free Cortisol was 15,878 mcg/day [normal: 100 mcg/day]. A MRI Pituitary/Sella revealed a 2.3 cm pituitary macroadenoma superiorly displacing and flattening the optic chiasm, invading into the right cavernous sinus. She was referred to Neurosurgery, who did a partial transphenoidal resection, pathology consistent with ACTH producing tumor. Post-operatively she developed central diabetes insipidus and adrenal insufficiency for which she received desmopressin and oral hydrocortisone respectively. Her laboratory values eight months since surgery show normalization of ACTH and cortisol levels. The patient’s general health has improved, headaches have resolved, strength has returned, and her hirsutism is reduced. Her BMI remains elevated at 88% but is declining and growth velocity is increasing back to her pre-disease level. Conclusion: Cushing Syndrome is exceedingly rare in pediatric aged patients and pituitary macroadenomas are atypical in this population.This is a unique case of an ACTH producing macroadenoma in a Pediatric patient, which has seldom been reported in the literature, and should be considered in patients with similar presenting symptoms.

scholarly journals Prognostic markers of remission of cushing's disease after transsphenoidal endoscopic adenomektomii.

2018 ◽  
Vol 95 (12) ◽  
pp. 1106-1111
Author(s):  
Natalia V. Gussaova ◽  
W. A. Choi ◽  
V. Yu. Cerebillo ◽  
A. A. Paltsev ◽  
A. V. Savello ◽  
...  
Keyword(s):  
Cushing’S Disease ◽  
Serum Cortisol ◽  
Threshold Value ◽  
Optimal Threshold ◽  
Cushing's Disease ◽  
High Dose ◽  
High Dose Dexamethasone ◽  
Free Cortisol ◽  
One Year ◽  
Cortisol Suppression

Aim. To study the role of prognostic oral high-dose dexamethasone suppression test (HDDST) and pituitary MRI in the prognosis of Cushing’s disease (CD). remission after transsphenoidal endoscopic surgery (TSS). Material and methods. 59 patients with Cushing’s disease (9 men, 50 women, mean age 40 years (15-72) underwent TSS were included. Before the TSS HDDST and pituitary MRI were performed in all cases. Postoperative examination was done one year after surgery. Remission criteria were: combination of normal midnight serum cortisol levels, normal 24 hour urine free cortisol (UFC) excretion and serum cortisol suppression less than 50 nmol/l in 1-mg dexamethasone test or secondary adrenal insufficiency (the need for glucocorticoid replacement). The optimal threshold value of serum cortisol suppression in the HDDST for prediction of CD remission after TSS was calculated by ROC-analysis. Results. One year after surgery CD remission was confirmed in 39 patients, whereas in 20 patients hypercortisolism persisted. The optimal threshold value of serum cortisol suppression in the HDDST for prediction of CD remission after TSS was 72%. Test’s sensitivity and specificity were 82% and 84%, respectively. The probability of wrong prediction was 17% (p=0,0001). In our study, the results of TSS did not correlate with MRI adenoma size.

Hyperprolactinaemia is associated with a higher prevalence of pituitary–adrenal dysfunction in non-functioning pituitary macroadenoma

1996 ◽  
Vol 135 (3) ◽  
pp. 299-308 ◽  
Author(s):  
Geert Tjeerdsma ◽  
Wim J Sluiter ◽  
Joffre M Hew ◽  
Willemina M Molenaar ◽  
Willem E de Lange ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Serum Cortisol ◽  
Urinary Free Cortisol ◽  
Peak Serum ◽  
University Hospital ◽  
Serum Prolactin ◽  
Pituitary Hormone ◽  
Pituitary Macroadenoma ◽  
Central Hypothyroidism ◽  
Free Cortisol

Tjeerdsma G, Sluiter WJ, Hew JM, Molenaar WM, de Lange WE, Dullaart RPF. Hyperprolactinaemia is associated with a higher prevalence of pituitary—adrenal dysfunction in non-functioning pituitary macroadenoma. Eur J Endocrinol 1996;135:299—308. ISSN 0804–4643 In non-functioning pituitary macroadenoma (NFMA), hyperprolactinaemia (hyperPRL) is considered to be a sign of hypothalamic—pituitary dysregulation, but it is unknown whether hyperPRL is associated with an increased frequency of pituitary hormone deficiencies. Forty consecutive patients with histology-proven NFMA were studied and hyperPRL was defined as serum prolactin (PRL) >200 mIU/l in men and >600 mIU/l in women. The pituitary—adrenal axis was evaluated by measurement of urinary free cortisol (N = 38), peak cortisol to insulin-induced hypoglycaemia (IIH, N = 36) and to human corticotrophin-releasing hormone (hCRF, N = 40) and by urinary tetrahydro11-deoxycortisol (H4S, N = 39), plasma androstenedione increment (N = 39) and serum 11-deoxycortisol (N = 1) after metyrapone. Central hypothyroidism, gonadotrophin deficiency and growth hormone (GH) reserve were also assessed. Twenty patients had hyperPRL (serum PRL 331 (2231120)mIU/l (median, range) in men and 932 (660–3927)mIU/l in women); urinary free cortisol excretion (p < 0.03) and peak serum cortisol in response to IIH (p < 0.02) were lower in hyperPRL than in normoPRL patients; peak serum cortisol after hCRF was not different between groups but occurred later in hyperPRL patients (at 60 vs 30 min, p < 0.03); urinary H4S excretion and androstenedione response after metyrapone were lower in hyperPRL than in normoPRL patients (p < 0.05 for both); 60% of hyperPRL patients and 15% of normoPRL patients had an abnormal H4S response (p < 0.025); central hypothyroidism (overt + subclinical) was present in 74% of hyperPRL and in 60% of normoPRL patients (NS); 78% of hyperPRL and 55% of normoPRL patients had gonadotrophin deficiency (NS); growth hormone (GH) deficiency was present in 83% of hyperPRL and in 89% of normoPRL patients (NS); 73.3% of 75 evaluable pituitary hormone axes were abnormal in hyperPRL patients compared to 5 3.8% of 78 hormone axes in normoPRL patients (by metyrapone test to examine adrenal function, p < 0.025); and no significant differences in tumour grade and stage distribution were found between hyperPRL and normoPRL patients. It is concluded that hyperprolactinaemia in NFMA is associated with a higher prevalence of pituitary–adrenal dysfunction, which is likely to be explained at least in part by functional hypothalamic–pituitary interruption. RPF Dullaart. Department of Endocrinology, University Hospital Groningen, PO Box 30.001, 9700 RB Groningen, The Netherlands

How useful is 24 hour Urinary Free Cortisol as a screening tool for Cushing's syndrome?

2018 ◽  
Author(s):  
Ahmed Hanafy ◽  
Chinnadorai Rajeswaran ◽  
Saad Saddiq ◽  
Warren Gillibrand ◽  
John Stephenson
Keyword(s):  
Cushing’S Syndrome ◽  
Screening Tool ◽  
Urinary Free Cortisol ◽  
Cushing's Syndrome ◽  
Free Cortisol
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