scholarly journals Limited Efficacy of Mifepristone Due to Poor Tolerance: A Clinical Challenge in Managing Cortisol Excess

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A140-A140
Author(s):  
Gabriel Jose Mora Osoria ◽  
Luis Norberto Madera Marin ◽  
Alegyari Figueroa Cruz ◽  
Yineli Ortiz Torres ◽  
Jose M Garcia-Mateo
Keyword(s):  
Adrenal Insufficiency ◽  
Medical Therapy ◽  
Adrenal Mass ◽  
Signs And Symptoms ◽  
Anxiety And Depression ◽  
Plasma Aldosterone Concentration ◽  
Poor Tolerance ◽  
Apparent Mineralocorticoid Excess ◽  
Uric Acid Nephrolithiasis ◽  
Adrenal Masses

Abstract A 53-year male with incidental bilateral adrenal masses along with symptoms of proximal muscle weakness, anxiety, and depression. Past medical history of uric acid nephrolithiasis, hypertension, hyperlipidemia, type 2 diabetes mellitus, anxiety, and depression. A right adrenal mass of 2.8cm and a left adrenal mass of 1.5cm both of <10 HU on non-enhancing CT. Hormonal activity workup was ordered which showed cortisol levels of 4.7mcg/dL on 1mg ONDST suggestive of autonomous cortisol excess and workup for primary hyperaldosteronism was negative. On follow up CT adrenal masses remained stable, but the patient underwent the annual hormonal workup for incidentaloma with 1mg ONDST and found with cortisol 4.5mcg/dL, eventually repeated with 2-day LDDST which showed no suppression of cortisol with levels at 4.5 mcg/dL confirming the diagnosis of autonomous cortisol excess. ACTH found to be suppressed at 3.1pg/dL (7.2–63.3 pg/dL) which helped confirm the diagnosis of non-ACTH dependent autonomous cortisol excess. Referred to surgery evaluation due to complications associated with cortisol excess as osteoporosis and uncontrolled hyperglycemia however surgical intervention was deferred until the source of cortisol excess could be identified within the bilateral adrenal masses and it was recommended to continue with medical therapy. As the cortisol excess source cannot be identified by localizing procedures and despite adequate medical therapy and stable adrenal masses on imaging, patient persisted with uncontrolled DM/hypertension and worsening BMD in the spine and hip with surgery not being an option. The patient was started on glucocorticoid receptor antagonist mifepristone in which upon reevaluation weakness and fatigue were noticeable along with hypokalemia of 3.1mmol/L after 2 weeks of therapy that was eventually replaced but 2 weeks later the patient discontinued therapy as he could not tolerate side effects related to adrenal insufficiency associated with mifepristone, such as weakness, fatigue, and dependence of potassium supplementation due to hypokalemia. This effect is physiologically important, because cortisol binds as avidly as aldosterone to the mineralocorticoid receptor, and the plasma cortisol concentration is approximately 100-fold higher than the plasma aldosterone concentration and it can lead to hypokalemia by the mechanism of apparent mineralocorticoid excess. Also, as mifepristone blocks cortisol action, the levels of ACTH and cortisol increase so high that hormonal measurement cannot be used to judge either therapeutic efficacy or adrenal insufficiency, we must go based on signs and symptoms.

scholarly journals SUN-173 A Gigantic Uterine Leiomyoma and Big Bilateral Adrenal Myelolipomas as a Result of Untreated Congenital Adrenal Hyperplasia

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Betty La ◽  
Ha Nguyen ◽  
Celestine Tung ◽  
Eugene Choi
Keyword(s):  
Congenital Adrenal Hyperplasia ◽  
Adrenal Insufficiency ◽  
Adrenal Mass ◽  
Uterine Leiomyoma ◽  
Pelvic Mass ◽  
Treatment Compliance ◽  
Total Testosterone ◽  
Adrenal Hyperplasia ◽  
Acth Stimulation ◽  
Adrenal Masses

Abstract Introduction Patients with untreated congenital adrenal hyperplasia (CAH) can present early with severe symptoms of salt wasting, adrenal insufficiency and hyperandrogenism. Late consequences as a result of long term untreated CAH are rarely seen nowadays. We present a patient who presented with a massive uterine leiomyoma and bilateral adrenal myelolipomas due to longstanding treatment noncompliance. Clinical Case A female was born with ambiguous genitalia and diagnosed with CAH at birth. She was raised as a female and received steroids until age 29 when she stopped taking steroids on her own with the intention of identifying as a male. At age 37, he presented with abdominal distension, vomiting, and hypotension. Physical exam was notable for hypotension, significantly distended abdomen, hirsutism, gynecomastia and clitoromegaly. Labs revealed sodium 126 meq/L (136–145) cortisol 78.5 ug/dL (3.7–19.4), ACTH 166 pg/mL (6–50), 17-hydroxyprogesterone 4356 ng/dL (≤285), androstenedione 7188 ng/dL (35–250), total testosterone 737 ng/dL (2–45), estradiol 142 pg/mL (48–440), aldosterone <1 ng/dL (3–16), renin 0.45 ng/mL/hr (0.25–5.82), metanephrines 56 pg/mL (≤205), normetanephrines 56 pg/mL (≤148). CT abdomen and pelvis revealed a large 31 x 35 x 31 cm pelvic mass, a 5.9 x 2.4 cm right adrenal mass and an 11.8 x 8.8 cm left adrenal mass. The patient underwent total abdominal hysterectomy and bilateral adrenalectomy. Pathology of the pelvic mass was consistent with uterine leiomyoma (gross tumor was 12.4 kg) and pathology of the bilateral adrenal masses were consistent with bilateral adrenal myelolipomas. Discussion Glucocorticoids and mineralocorticoids are the mainstays of treatment in CAH, with the goal of providing adequate replacement while reducing levels of ACTH and adrenal androgens. Persistently elevated levels of ACTH and androgens can lead to many serious sequela, even outside of adrenal insufficiency and virilization. Due to the conversion of androgens to estrogens, untreated females with CAH have significantly elevated levels of both hormones. These high levels of androgens and estrogen can then stimulate growth of estrogen-dependent organs as exemplified by our patient. Chronic ACTH stimulation can cause adrenal hyperplasia, but has also been associated with the development of other adrenal masses including adrenal myelolipomas. Adrenal myelolipomas can become hormonally functional or cause mass effect, hemorrhage, necrosis when reaching a large enough size. Conclusion This case demonstrates the importance of CAH treatment compliance as there are many serious sequela outside of the expected adrenal insufficiency and virilization. Even when the desired effect is virilization with physical male features, other means of hormonal therapy should be considered as there remains the risks of abnormal growth of certain organs sensitive to the excessive hormones.

Adrenal Lymphoma: Case Report and Review of Literature

2010 ◽  
Vol 2 (1) ◽  
pp. 39-43 ◽  
Author(s):  
Jaya Bhanu Kanwar
Keyword(s):  
Weight Loss ◽  
Adrenal Insufficiency ◽  
Adrenal Mass ◽  
Serum Lactate ◽  
Normal Serum ◽  
Serum Lactate Dehydrogenase ◽  
Chop Regimen ◽  
First Case ◽  
Adrenal Lymphoma ◽  
Adrenal Masses

ABSTRACT Adrenal lymphoma is a rare diagnosis in subject presenting with bilateral adrenal masses. Adrenal insufficiency is a common complication of this disease. Most cases of primary adrenal lymphoma (PAL) present with adrenal insufficiency and huge bilateral adrenal masses. These are very aggressive tumors with poor prognosis. We, hereby, report two subjects of adrenal lymphoma presenting with bilateral adrenal masses. First case is a 50 years old male presented with features of adrenal insufficiency like anorexia, weight loss, nausea, vomiting, and generalized hyperpigmentation. Routine investigations revealed hyponatremia, normal serum K; serum lactate dehydrogenase was moderately elevated. Primary adrenal insufficiency was confirmed by low basal and cosyntrophin stimulated cortisol. CT abdomen revealed huge bilateral heterogeneous enlargement of adrenal glands. Lymphoma was confirmed by histopathology and immunohistochemistry, diagnosis of PAL was made after further staging. He was treated with Rituximab-CHOP regimen. Second case, 50 years old lady presented with fever, anorexia, weight loss. She had generalized lymphadenopathy. On imaging found to have bilateral adrenal mass, and histopathology from lymph node, breast mass and adrenal mass was suggestive of non-Hodgkin's lymphoma.

scholarly journals Bilateral Adrenal Incidentalomas, Uncommon Presentation and Very Rare Pathology

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A119-A119
Author(s):  
Mohammad Khair Ahmad Ibraheem Hamad ◽  
Ahmed Osman Saleh ◽  
Emad Naem
Keyword(s):  
Ct Scan ◽  
Adrenal Insufficiency ◽  
Adrenal Mass ◽  
Adrenal Glands ◽  
Adrenal Incidentaloma ◽  
B Cell Lymphoma ◽  
Primary Adrenal Lymphoma ◽  
Adrenal Lymphoma ◽  
History Of ◽  
Adrenal Masses

Abstract Background: Adrenal incidentaloma is an adrenal mass larger than 1cm accidentally discovered by radiological examination. The incidentalomas should be evaluated for malignancy and functionality. The prevalence of adrenal incidentaloma is 4–10%,1 and around 10–15% of incidentally discovered adrenal masses can be bilateral.2 We present a patient with bilateral adrenal masses due to primary adrenal lymphoma. Clinical Case: 74-year-old gentleman, with past medical history of type-2 Diabetes mellitus, hypertension, presented to our hospital with 2 months history of right-sided flank pain, 20 kg weight loss, loss of appetite and dizziness. Otherwise, no fever, night sweats, bleeding per rectum or urinary symptoms. On physical examination, vital signs were within normal, no remarkable findings other than tenderness over the right flank. Complete blood count and chemistry panel were within normal. Abdominal CT scan was done for evaluation of the abdominal pain which showed two large masses replacing the adrenal glands measure about 10cm in diameter with patchy areas of enhancement. Adrenal insufficiency was suspected based on the symptoms and the CT scan findings. Short syncatin test (high-dose test 250mcg) was done, which showed a cortisol baseline 152nmol/L, 30 minutes 168nmol/L, and 60 minutes 169nmol/L. This was suggestive of adrenal insufficiency, so he was started on hydrocortisone. Screening for pheochromocytoma was done as the patient developed uncontrolled BP readings prior to a scheduled adrenal biopsy. 24-hour urine metanephrines and normetanephrine were negative. CT-guided adrenal mass biopsy showed findings suggestive of large B-cell lymphoma. FDG PET-CT scan showed huge bilateral intense FDG uptake in the adrenal glands with no extra-abdominal manifestations noted. He was started on R-CHOP chemotherapy and after 4cycles, a PET scan showed a significant decrease in the size of previous adrenal masses. Conclusion: Bilateral adrenal incidentaloma should be evaluated the same as unilateral adrenal mass. Although adrenal involvement is common in non-Hodgkin lymphoma, primary adrenal lymphoma is extremely uncommon. References 1.Bovio S, Cataldi A, Reimondo G, Sperone P, Novello S, Berruti A, Borasio P, Fava C, Dogliotti L, Scagliotti GV, Angeli A, Terzolo M. Prevalence of adrenal incidentaloma in a contemporary computerized tomography series. J Endocrinol Invest. 2006 Apr;29(4):298–302. doi: 10.1007/BF03344099. PMID: 16699294. 2.Angeli A, Osella G, Alì A, Terzolo M. Adrenal incidentaloma: an overview of clinical and epidemiological data from the National Italian Study Group. Horm Res. 1997;47(4–6):279–83. doi: 10.1159/000185477. PMID: 9167965.

Could we assess the functional status, of hormone secreting, or non-secreting of the adrenal masses regarding their Magnetic Resonance Imaging (MRI) characteristics?

2021 ◽  
Vol 22 ◽  
Author(s):  
Gamze Akkus ◽  
Ferhat Piskin ◽  
Barış Karagun ◽  
Murat Sert ◽  
Mehtap Evran ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Chemical Shift ◽  
Functional Status ◽  
Signal Intensity ◽  
Adrenal Mass ◽  
Resonance Imaging ◽  
Intensity Index ◽  
Adrenal Masses ◽  
Magnetic Resonance Imaging Mri

Background: Diagnostic imaging techniques including magnetic resonance imaging (MRI) should also perform on all patients with incidentalomas. However, there is a limited study whether the quantitative measurements (signal intensity index, adrenal to spleen ratio) in MRI could predict the functional status of adrenal adenomas. Material-Method: Between 2015-2020; 404 patients (265 females, 139 males) with adrenal mass who were referred to the university hospital for further investigation were included. After detailed diagnostic hormonal evaluation, all patients underwent MRI 1.5 T device (Signa, GE Medical Systems; Milwaukee, USA). The signal intensities of the adrenal lesions on T2W images were qualitatively evaluated and noted as homogenous or heterogeneous in comparison with the liver signal intensity (SI). A chemical-shift SI index and chemical shift adrenal-to-spleen SI ratio were also calculated. Results: While 331(81.9%) of the patients had nonfunctional adrenal mass, the rest of them (n=73, 18.1%) were patients with functional (autonomous cortisol secretion-ACS, cushing syndrome-CS, pheochromocytoma, primary hyperaldosteronism-PA) adrenal masses. In phase vs phase values of patients with NFAI, Pheo(n=17), ACS (n=30), CS (n=11), and PA (n=15) were 474.04±126.7 vs 226.6±132.4, 495.3±182.8 vs 282.17±189.1, 445.2±134.8 vs 203.3±76.2, 506.8±126.5 vs 212.2±73.6 and 496.2±147.5 vs 246.6±102.1, respectively. Mean signal intensity index (SII) and adrenal to spleen ratio (ASR) of all groups (NFAI, Pheo, ACS, CS, PA) were 52.0±24.8 and 0.51, 44.9±22.5 and 0.55, 49.5±24.5 and 0.53, 56.2±16.4 and 0.43, 47.6±25.1 and 0.54, respectively. Based the current accepted measurements in the case of ASR and SII, all lesions were similar and shown as fat rich adenomas (p*= 0.552, p** = 0.45). Conclusion: The quantitative assessment (SII, ASR) of intracellular lipids in an incidentally discovered adrenal tumour could only help distinguish adrenal masses in case of adenomas or non-adenomas As initial diagnostic evaluation, clinical and laboratory assessment ,to distinguish hormone secretion, should be taken in all patients with adrenal incidentalomas.

scholarly journals A Case Report on Bilateral Adrenal Histoplasmosis

2020 ◽  
Vol 23 (2) ◽  
pp. 210-212
Author(s):  
Md Sazzad Hossain ◽  
Mohammad Zahid Hasan ◽  
Mir Ehteshamul Haque ◽  
Mostakim Maoya
Keyword(s):  
Adrenal Mass ◽  
Adrenal Glands ◽  
Histoplasma Capsulatum ◽  
Needle Aspiration ◽  
Immunosuppressed Patient ◽  
Aspiration Cytology ◽  
Fine Needle ◽  
Disseminated Histoplasmosis ◽  
Adrenal Masses ◽  
Needle Aspiration Cytology

Disseminated histoplasmosis affecting the adrenal glands is a very rare infection and an uncommon cause of adrenal insufficiency. We present a case of bilateral adrenal histoplasmosis in a 77 years old immunosuppressed patient who presented with bilateral adrenal masses. The fine needle aspiration cytology of the adrenal mass was positive for Histoplasma capsulatum. Bangladesh Journal of Urology, Vol. 23, No. 2, July 2020 p.210-212

scholarly journals Incidental Finding of Giant Coronary Artery Aneurysms Successfully Treated with Medical Therapy

2019 ◽  
Vol 2019 ◽  
pp. 1-5
Author(s):  
Rony Shammas ◽  
Prasanna Sengodan ◽  
Assad Movahed
Keyword(s):  
Coronary Artery ◽  
Medical Therapy ◽  
Chest Radiography ◽  
Incidental Finding ◽  
Lower Lobe ◽  
Signs And Symptoms ◽  
Coronary Artery Aneurysms ◽  
Coronary Aneurysms ◽  
Cardiac Evaluation

We report a case of a 30-year-old male who presented with signs and symptoms of respiratory infection with left lower lobe consolidation and cardiomegaly on a chest radiography. The presence of cardiomegaly lead to further cardiac evaluation revealing giant coronary aneurysms. The patient was treated conservatively with Coumadin and aspirin and has done well at four years of follow-up.

scholarly journals Therapeutic Patient Education for Adrenal Insufficiency under COVID-19 Pandemic Conditions

2020 ◽  
Author(s):  
Tina Kienitz ◽  
Stefanie Hahner ◽  
Stephanie Burger-Stritt ◽  
Marcus Quinkler
Keyword(s):  
Patient Education ◽  
Adrenal Insufficiency ◽  
Replacement Therapy ◽  
Medical Therapy ◽  
Medical Personnel ◽  
Daily Basis ◽  
Background Information ◽  
Therapeutic Patient Education ◽  
Dose Adaptation ◽  
Glucocorticoid Replacement Therapy

AbstractAdrenal insufficiency (AI) requires life-long treatment with glucocorticoid replacement therapy. Over- or under-substitution carries the risk of increased morbidity in the form of side effects or adrenal crises. Glucocorticoid replacement therapy needs to be flexible with dose adaptation in special situations. This could not be managed by medical personnel on a daily basis, but requires an educated patient who has a good knowledge of the disease, understands his medical therapy and is able to perform situational dose adaptation. The rarity of the disease in combination with the need to respond to stressful situations with rapid glucocorticoid dose adjustment underlines that a well-trained patient is crucial for optimal management of the disease.In this literature review we provide background information further clarifying the need of education in patients with AI including the current shortcomings of medical therapy and of the treatment of patients with AI. We outline the aims of therapeutic patient education, present the concept of structured patient education in Germany, and discuss available results of patient group education programs. Furthermore, we propose strategies how therapeutic patient education for adrenal insufficiency can be organized under COVID-19 pandemic conditions.

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