scholarly journals Pituitary Macroadenoma Masked by Iatrogenic Adrenal Insufficiency: A Diagnostic Blind Spot

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A151-A151
Author(s):  
Ivy Hoi Yee Ng ◽  
Elaine Yun Ning Cheung
Keyword(s):  
Adrenal Insufficiency ◽  
Hpa Axis ◽  
Optic Chiasm ◽  
Blind Spot ◽  
Pituitary Hormones ◽  
Pituitary Macroadenoma ◽  
Pituitary Mass ◽  
Blurred Vision ◽  
Central Adrenal Insufficiency ◽  
Short Synacthen Test

Abstract Background: Exogenous steroid use is the most common cause of central adrenal insufficiency. Depending on the duration and strength used, it may take months to years for the hypothalamic-pituitary-adrenal (HPA) axis to recover after the steroid is stopped. We report a case of iatrogenic hypoadrenalism with persistent suppression of the HPA axis for 13 years, discovered later to be due to a second pathology. Case: A 48 year old lady presented in 2005 with weight gain of 20 kg over 1 year and florid Cushingoid features. 9AM cortisol was undetectable (<12 nmol/L). History taking revealed use of oral dexamethasone at various dosages over the past 9 years for her knee pains. A diagnosis of iatrogenic adrenal insufficiency was made. She was started on hydrocortisone replacement, and was advised to stop the over-the-counter steroids. By 2011 her short Synacthen test (SST) showed much improved functioning of the HPA axis (cortisol 182 (0 min) -> 329 (30 min) -> 408 nmol/L (60 min) [N peak>500 nmol/L]), and she was back to her usual body weight. However, subsequent monitoring revealed declining trend of 9AM cortisol from 135 nmol/L (2013) -> 99 nmol/L (2014) -> 57 nmol/L (2015) -> 64 nmol/L (2016) -> 18 nmol/L (2017) [N 166–507 nmol/L]. Hydrocortisone compliance and abstinence from exogenous steroids was confirmed with the patient. The ongoing hypofunction of the HPA axis was continually attributed by multiple physicians to her history of prolonged use of dexamethasone. In 2018, at the age of 60, the lady presented with new onset headaches, blurred vision, and bitemporal hemianopia for 3 months. MRI showed a 1.8x1.8x3.5 cm (WxAPxH) pituitary mass with suprasellar extension compressing the optic chiasm. Blood tests revealed panhypopituitarism: SST cortisol 17 -> 59 -> 49 nmol/L, ACTH 2.7 pmol/L [N <10.1 pmol/L]; fT4 9.5 pmol/L [N 12–22 pmol/L], TSH 0.97 mIU/L [N 0.27–4.2 mIU/L]; LH <0.1 IU/L, FSH 0.52 IU/L (menopause at age of 48); IGF-1 27 µg/L [N 41–279 µg/L]; prolactin 17 mIU/L [N 102–496 mIU/L]. After partial excision of the mass her vision improved, but remained dependent on hydrocortisone and thyroxine supplements. The lesion was pathologically proven to be a pituitary macroadenoma. Discussion: This case presents the uncommon course of a patient who had almost recovered from iatrogenic hypoadrenalism, only to lapse back into worsened central adrenal insufficiency, as part of panhypopituitarism related to an undiagnosed pituitary mass. In retrospect, the unusually protracted state of hypocortisolemia and the atypical waxing and waning HPA axis should have alerted one to consider alternative etiologies at work. As LH-FSH and GH deficiencies commonly develop before ACTH and TSH deficiencies in most pituitary macroadenomas, a lower threshold for testing the other anterior pituitary hormones followed by imaging of the pituitary could have picked up the tumor earlier in this patient.

scholarly journals A Case of Rathke’s Cleft Cyst Associated with Transient Central Adrenal Insufficiency and Masked Diabetes Insipidus

2014 ◽  
Vol 2014 ◽  
pp. 1-6
Author(s):  
Masahiro Asakawa ◽  
Rina Chin ◽  
Yoshihiro Niitsu ◽  
Tetsuo Sekine ◽  
Arisa Niwa ◽  
...  
Keyword(s):  
Diabetes Insipidus ◽  
Adrenal Insufficiency ◽  
Optic Chiasm ◽  
Nasal Administration ◽  
Rathke’S Cleft Cyst ◽  
Rathke's Cleft Cyst ◽  
Pituitary Mass ◽  
Silent Thyroiditis ◽  
Central Adrenal Insufficiency ◽  
Rathke's Cleft

A 73-year-old woman admitted to our hospital because of headache, poor appetite, malaise, weight loss, and vomiting was found to have central adrenal insufficiency and thyrotoxicosis due to silent thyroiditis. Polyuria developed after replacement with glucocorticoid (masked diabetes insipidus), which was controlled with nasal administration of desmopressin. Magnetic resonance imaging of the brain showed a large cystic pituitary mass (18 × 18 × 12 mm) extending suprasellarly to the optic chiasm. Transsphenoidal surgery revealed that the pituitary tumor was Rathke’s cleft cyst. Following surgery, replacement with neither glucocorticoid nor desmopressin was needed any more. Therefore, it is suggested that Rathke’s cleft cyst is responsible for the masked diabetes insipidus and the central insufficiency. Furthermore, it is speculated that thyrotoxicosis with painless thyroiditis might induce changes from subclinical adrenal insufficiency to transiently overt insufficiency.

scholarly journals Low-dose short synacthen test with salivary cortisol in patients with suspected central adrenal insufficiency

2021 ◽  
Vol 10 (9) ◽  
pp. 1189-1199
Author(s):  
Filippo Ceccato ◽  
Elisa Selmin ◽  
Giorgia Antonelli ◽  
Mattia Barbot ◽  
Andrea Daniele ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Hpa Axis ◽  
Low Dose ◽  
University Hospital ◽  
Glucocorticoid Treatment ◽  
Basal Serum ◽  
Diagnostic Threshold ◽  
Synacthen Test ◽  
Central Adrenal Insufficiency ◽  
Short Synacthen Test

Context The low-dose short synacthen test (LDSST) is recommended for patients with suspected central adrenal insufficiency (AI) if their basal serum cortisol (F) levels are not indicative of an intact hypothalamic–pituitary–adrenal (HPA) axis. Objective To evaluate diagnostic threshold for salivary F before and 30 min after administering 1 μg of synacthen, performed before 09:30 h. Design A cross-sectional study from 2014 to 2020. Setting A tertiary referral university hospital. Patients In this study, 174 patients with suspected AI, 37 with central AI and 137 adrenal sufficient (AS), were included. Main outcome measure The diagnostic accuracy (sensitivity (SE), specificity (SP)) of serum and salivary F levels measured, respectively, by chemiluminescence immunoassay and liquid chromatography-tandem mass spectrometry. Results Low basal serum or salivary F levels could predict AI. For the LDSST, the best ROC-calculated threshold for serum F to differentiate AI from AS was 427 nmol/L (SE 79%, SP 89%), serum F > 500 nmol/L reached SP 100%. A salivary F peak > 12.1 nmol/L after administering synacthen reached SE 95% and SP 84% for diagnosing central AI, indicating a conclusive reduction in the likelihood of AI. This ROC-calculated threshold for salivary F was similar to the 2.5th percentile of patients with a normal HPA axis, so it was considered sufficient to exclude AI. Considering AS those patients with salivary F > 12.1 nmol/L after LDSST, we could avoid unnecessary glucocorticoid treatment: 99/150 subjects (66%) had an inadequate serum F peak after synacthen, but salivary F was >12.1 nmol/L in 79 cases, who could, therefore, be considered AS. Conclusions Salivary F levels > 12.1 nmol/L after synacthen administration can indicate an intact HPA axis in patients with an incomplete serum F response, avoiding the need to start glucocorticoid replacement treatment.

scholarly journals SAT-272 Pituitary Macroadenoma Co-Secreting TSH and PRL Responsive to Cabergoline

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Saritha Tirumalasetty ◽  
Robert Galagan ◽  
Dragana Lovre ◽  
Julia David
Keyword(s):  
Tumor Size ◽  
Pituitary Tumors ◽  
Residual Tumor ◽  
Optic Chiasm ◽  
Pituitary Hormones ◽  
Thyroid Stimulating Hormone ◽  
Pituitary Macroadenoma ◽  
Mri Imaging ◽  
Free T4 ◽  
Α Subunit

Abstract Background: Thyroid-stimulating hormone (TSH) secreting tumors (TSHoma) account for 0.5-2% of all pituitary adenomas with a prevalence of 1-2 cases per million, indicating that TSHomas are very rare. The majority of TSHomas solely secrete TSH however 9.7% co-secrete Prolactin (PRL). We are reporting a case of co-secreting TSH and PRL pituitary macroadenoma responsive to Cabergoline (CAB) Case: A 52 year old multiparous female presented with symptoms of galactorrhea and amenorrhea. Lab investigation revealed elevated PRL 73 ng/mL (n [normal] = 1-24), Total T3 305 ng/dL (n = 71-180), Free T4 (FT4) 2.37 ng/dL (n = 0.6-1.15), TSH 6.09 UIU/mL (n = 0.5-5.0), and α subunit 7.7 ng/mL. Estradiol was low at 16.9 pg/mL and FSH 6.3 MIU/mL LH 1.7 MIU/mL. Visual field testing showed a right nasal step. MRI imaging demonstrated a 21x24x32mm pituitary macroadenoma with optic chiasm distortion. Partial Transsphenoidal surgery (TSS) was performed and immunostaining of tumor tissue was positive for PRL and negative for other pituitary hormones. One month post-surgical MRI revealed 14x17x15mm residual tumor. One month post-op TFTs were normal: TSH 0.99 ng/dL, FT4 0.61 ng/dL; PRL decreased to 34.8 ng/mL. Six month post-op TSH increased to 5.86 UIU/mL, FT4 1.43 ng/dL, and PRL 44.7 ng/mL. Two years post-op TSH 8.06 ng/dL with elevated α subunit 3.4 ng/mL and PRL 56.8 ng/mL. Octreotide was then initiated for TSHoma treatment however she was unable to tolerate the medication due to diarrhea so was switched to CAB. After starting CAB at 0.5mg twice a week, residual sellar mass increased in size to 19.5x16x23mm with TFTs: TSH 5.66 UIU/mL, FT4 2.48 ng/dL. CAB dose was eventually uptitrated to 1mg twice a week. Repeat MRI showed slight decrease in pituitary lesion to 19x21x18mm and downtrending TFTs: TSH 2.28 UIU/mL, FT4 1.17 ng/dL. Discussion: In patients with pituitary tumors associated with elevated PRL and TSH, TSHoma should be part of the differential diagnosis. This patient’s initial lab evaluation with elevated PRL, TSH, FT4, Total T3, and α subunit confirm the diagnosis of a pituitary macroadenoma with co-secretion of PRL and TSH. Elevated PRL, TSH, FT4 and α subunit levels occurred 6 months after partial TSS resection with growing tumor size eventually requiring medical therapy. On CAB therapy, there were reductions in PRL, TSH, and FT4 levels as well as a decrease tumor size. This is the first reported case of a TSHoma responsive to CAB.

scholarly journals MON-253 A Rare Case of Pituitary Aspergillosis Diagnosed by CSF PCR in an Immunocompetent Patient with Headaches and Photophobia

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Rakesh Popli ◽  
Tina Nikoomanesh ◽  
Gavin Jackson
Keyword(s):  
Adrenal Insufficiency ◽  
Vital Signs ◽  
Immunocompetent Patient ◽  
Empiric Antibiotic Therapy ◽  
Temporal Region ◽  
Free T4 ◽  
Pituitary Mass ◽  
Central Hypothyroidism ◽  
History Of ◽  
Central Adrenal Insufficiency

Abstract Background: Pituitary aspergillosis is a rare infection usually found in the immunocompromised population. It is oftentimes mistaken for a pituitary adenoma based on similar clinical presentation and characteristic findings on MRI. Most cases require removal of the pituitary mass in order to make a diagnosis. Here we present the case of an immunocompetent patient with headaches and photophobia diagnosed with pituitary aspergillosis by CSF PCR and treated medically with voriconazole. Clinical Case: A 40-year-old woman with a questionable history of Brucellosis presented with a 3 month history of headaches along with 2 days of nausea and vomiting. Vital signs were notable for intermittent hypotension but were otherwise within normal limits. Physical exam was notable for tenderness at the left temporal region, diaphoresis and photophobia. Patient was otherwise alert and oriented and had no visual field deficits or extraocular muscle dysfunction. Patient was found to have central adrenal insufficiency with undetectable AM cortisol (<0.5 mcg/dL, n 3.7-19.4 mcg/dL), inappropriately normal ACTH (7 pg/mL, n 6-58 pg/mL) and central hypothyroidism with low TSH (0.057 mcIU/mL, n 0.358-3.8 mcIU/mL) and low free T4 (0.48 ng/dL, n 0.76-1.46 ng/dL). Patient initially presented with hyponatremia (Na 119 mmol/L, n 137-145 mmol/L) likely secondary to central adrenal insufficiency and central hypothyroidism. Gadolinium-enhanced pituitary MRI showed a heterogeneous 1.8 cm pituitary mass with rim enhancement concerning for hypophysitis. Patient was started on stress-dose steroids with IV hydrocortisone 100 mg IV q8h, levothyroxine 50 mcg PO daily and empiric antibiotic therapy with ceftriaxone, doxycycline and rifampin due to suspicion for neurobrucellosis. Lumbar puncture was obtained showing low glucose (39 mg/dL, n 40-70 mg/dL), normal protein (47 mg/dL, n 12-60 mg/dL) and an elevated white count (WBC 9/mcL, n 0-5/mcL) with lymphocyte predominance (97% lymphocytes, n 40-80%). Blood and CSF cultures showed no growth at 2 weeks. CSF was sent for multiplex PCR which came back positive for Aspergillus. Patient was discharged with voriconazole 300 mg PO BID for 1 year, levothyroxine 75 mcg PO daily and hydrocortisone 10 mg PO Qam and 5 mg PO Qpm. Three months later, repeat MRI showed resolution of the pituitary mass and patient felt well without headaches, nausea or vomiting. Conclusion: This case demonstrates an atypical example of pituitary aspergillosis diagnosed without pituitary mass biopsy and treated medically with voriconazole. It demonstrates the possible role of CSF PCR to diagnose the condition and guide antifungal treatment.

scholarly journals Central adrenal insufficiency: open issues regarding diagnosis and glucocorticoid treatment

2019 ◽  
Vol 57 (8) ◽  
pp. 1125-1135 ◽  
Author(s):  
Filippo Ceccato ◽  
Carla Scaroni
Keyword(s):  
Adrenal Insufficiency ◽  
Pituitary Tumors ◽  
Cranial Irradiation ◽  
Glucocorticoid Treatment ◽  
Acth Deficiency ◽  
Reference Sections ◽  
Pubmed Database ◽  
Clinical Background ◽  
Central Adrenal Insufficiency ◽  
Short Synacthen Test

Abstract Background Central adrenal insufficiency (CAI) is characterized by impaired adrenocorticotropin (ACTH) secretion because of a disease or injury to the hypothalamus or the pituitary, leading to a reduced cortisol production. CAI suspicion arises more frequently in patients with pituitary tumors, cranial irradiation/surgery/injury/infections, as well as after exogenous glucocorticoid withdrawal. Nevertheless, a late diagnosis is not uncommon because CAI may present with nonspecific signs or symptoms, as fatigue or hyponatremia. Content The PubMed database was searched (years 1980–2018), using “central adrenal insufficiency” and “ACTH deficiency” as keywords. Subsequently, reference sections of the retrieved articles were searched. Summary Dynamic tests are needed when morning basal cortisol levels are not sufficient to exclude or to confirm CAI. Short Synacthen Test (SST) is the most used, and Endocrine Society’s guidelines recommend a cortisol peak >500 nmol/L to exclude CAI. Despite thresholds, understanding the pretest probability of ACTH deficiency (the clinical background of the patient) is essential because the diagnostic accuracy of SST in case of a negative result is suboptimal. Glucocorticoid replacement therapy, able to replicate cortisol circadian rhythm, is required in patients with CAI; fludrocortisone treatment is not necessary. Short-acting glucocorticoid drugs (hydrocortisone or cortisone acetate) are the most used; lower doses than previously used are nowadays recommended to reduce cortisol-related comorbidities. Promising results have been obtained with modified-release hydrocortisone, especially regarding glucose metabolism in patients with primary adrenal insufficiency. Outlook An accurate clinical diagnosis and a careful individualized therapy are mandatory in patients with CAI.

scholarly journals Acknowledging the Blind Spot of Bitemporal Hemianopsia: Keeping a High Index of Suspicion for Pituitary Macroadenomas in Uncommon Presentations

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A569-A569
Author(s):  
Joseph Raco ◽  
Maria Macias ◽  
Rohit Jain
Keyword(s):  
Vision Loss ◽  
Visual Fields ◽  
Optic Chiasm ◽  
Blind Spot ◽  
Surgical Removal ◽  
Double Vision ◽  
Neurologic Examination ◽  
Pituitary Macroadenoma ◽  
Free T4 ◽  
Gait Disturbances

Abstract Background: Hyperglycemia in patients with type 2 diabetes mellitus commonly manifests as symptoms of polyuria, polydipsia, fatigue, and weight loss as a result of insulin resistance. In cases of severe hyperglycemia, patients may also experience visual disturbances and dizziness as a result of swelling of the lens and dehydration respectively. These symptoms are not generally accompanied by gait disturbance or fixed, focal deficits on neurological examination. As such, symptoms such as double vision, peripheral field vision loss, cranial nerve deficits, or significant unsteadiness may warrant a more extensive neurologic workup rather than simply attributing all symptoms to hyperglycemia. Clinical Case: A 57-year-old woman presented to the emergency department with five days of fatigue, polyuria, polydipsia and pre-syncope associated with dizziness. She also described double vision in her peripheral visual fields and episodes of gait disturbances causing her to have to lower herself to the ground on multiple occasions, without loss of consciousness. Her neurologic examination demonstrated mildly ataxic finger-to-nose testing and concern for peripheral field vision loss. Intake lab work revealed a blood glucose of 725 mg/dL and a hemoglobin A1C of 13.4%. Initial neuroimaging with computed tomography was unremarkable. Though her symptoms were thought to be due to severe hyperglycemia, an MRI brain was obtained due to abnormal neurologic examination. MRI demonstrated a 1.9 cm pituitary macroadenoma abutting the optic chiasm with concern for hemorrhage. Subsequent lab evaluation determined the pituitary macroadenoma non-functional with TSH, free T4, free T3, AM cortisol, AM ACTH, and prolactin all within normal limits. Her hyperglycemia was treated with insulin with clinical improvement in all regards except visual symptoms. She was deemed safe for discharge with neurosurgical follow-up regarding surgical removal of her macroadenoma. Conclusion: Although hyperglycemia may present with broad symptoms including vague neurologic symptoms, it is critical to keep a broad differential diagnosis when atypical symptoms such as persistent vision changes and gait disturbances are present, especially after improvement in glycemic control has been obtained. A low threshold should be held for obtaining neuroimaging, as it is prudent to rule-out life-threatening causes of neurologic dysfunction.

scholarly journals Megace Mystery: A Case of Central Adrenal Insufficiency

2015 ◽  
Vol 2015 ◽  
pp. 1-4 ◽  
Author(s):  
Kunal Mehta ◽  
Irene Weiss ◽  
Michael D. Goldberg
Keyword(s):  
Progesterone Receptor ◽  
Glucocorticoid Receptor ◽  
Adrenal Insufficiency ◽  
Hpa Axis ◽  
Active Treatment ◽  
Clinical Syndrome ◽  
Essentially Normal ◽  
Central Adrenal Insufficiency ◽  
Cortisol Deficiency ◽  
Biochemical Features

Megestrol acetate (MA) is a synthetic progestin with both antineoplastic and orexigenic properties. In addition to its effects on the progesterone receptor, MA also binds the glucocorticoid receptor. Some patients receiving MA therapy have been reported to develop clinical features of glucocorticoid excess, while others have experienced the clinical syndrome of cortisol deficiency—either following withdrawal of MA therapy or during active treatment. We describe a patient who presented with clinical and biochemical features of central adrenal insufficiency. Pituitary function was otherwise essentially normal, and the etiology of the isolated ACTH suppression was initially unclear. The use of an exogenous glucocorticoid was suspected but was initially denied by the patient; ultimately, the culprit medication was uncovered when a synthetic steroid screen revealed the presence of MA. The patient’s symptoms improved after she was switched to hydrocortisone. Clinicians should be aware of the potential effects of MA on the hypothalamic-pituitary-adrenal (HPA) axis.

scholarly journals Recovery of the Hypothalamo-Pituitary-Adrenal Axis After Transsphenoidal Adenomectomy for Non–ACTH-Secreting Macroadenomas

2019 ◽  
Vol 104 (11) ◽  
pp. 5316-5324 ◽  
Author(s):  
Riccardo Pofi ◽  
Sonali Gunatilake ◽  
Victoria Macgregor ◽  
Brian Shine ◽  
Robin Joseph ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Hpa Axis ◽  
Secondary Adrenal Insufficiency ◽  
Baseline Cortisol ◽  
Transsphenoidal Adenomectomy ◽  
Synacthen Test ◽  
Acth Secreting ◽  
Short Synacthen Test ◽  
Pituitary Adrenal Axis ◽  
Hpa Function

Abstract Context Secondary adrenal insufficiency is a potential complication of transsphenoidal adenomectomy (TSA). Most centers test recovery of the hypothalamo-pituitary-adrenal (HPA) axis after TSA, but, to our knowledge, there are no data predicting likelihood of recovery or the frequency of later recovery of HPA function. Objective To assess timing and predictors of HPA axis recovery after TSA. Design Single-center, retrospective analysis of consecutive pituitary surgeries performed between February 2015 and September 2018. Patients Patients (N = 109) with short Synacthen test (SST) data before and at sequential time points after TSA. Main outcome measures Recovery of HPA axis function at 6 weeks, and 3, 6, and 9 to12 months after TSA. Results Preoperative SST indicated adrenal insufficiency in 21.1% Among these patients, 34.8% recovered by 6 weeks after TSA. Among the 65.2% (n = 15) remaining, 13.3% and 20% recovered at 3 months and 9 to 12 months, respectively. Of the 29% of patients with adrenal insufficiency at the 6-week SST, 16%, 12%, and 6% subsequently recovered at 3, 6, and 9 to 12 months, respectively. Preoperative SST 30-minute cortisol, postoperative day 8 cortisol, and 6-week postoperative SST baseline cortisol levels above or below 430 nmol/L [15.5 μg/dL; AUC ROC, 0.86]; 160 nmol/L (5.8 μg/dL; AUC ROC, 0.75); and 180 nmol/L (6.5 μg/dL; AUC ROC, 0.88), were identified as cutoffs for predicting 6-week HPA recovery. No patients with all three cutoffs below the threshold recovered within 12 months after TSA, whereas 92% with all cutoffs above the threshold recovered HPA function within 6 weeks (OR, 12.200; 95% CI, 5.268 to 28.255). Conclusion HPA axis recovery can occur as late as 9 to 12 months after TSA, demonstrating the need for periodic reassessment of patients who initially have SST-determined adrenal insufficiency after TSA. Pre- and postoperative SST values can guide which patients are likely to recover function and potentially avoid unnecessary lifelong glucocorticoid replacement.

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