We describe here the case of a 39-year-old woman with a cortisol-producing adrenal adenoma and she presented with endometrial hyperplasia and hypertension without the specific characteristics of Cushing's syndrome. The patient had consulted a gynecologist for menometrorrhagia 2 years prior to her referral and she was diagnosed with endometrial hyperplasia and hypertension. Her blood pressure and the endometrial lesion were refractory despite taking multiple antihypertensives and repetitive dilation and curettage and progestin treatment. On admission, the clinical examination revealed mild central obesity (a body mass index of 22.9 kg/m2, a waist circumference of 85 cm and a hip circumference of 94cm), but there was no hirsutism and myopathy. She showed impaired glucose tolerance on an oral glucose tolerance test. The biochemical hypercortisolemia together with the prolactin and androgen levels were evaluated to explore the cause of her anovulation. Adrenal Cushing's syndrome was confirmed on the basis of the elevated urinary free cortisol (454 microgram/24h, normal range: 20-70) with a suppressed ACTH level (2.0 pg/mL, normal range: 6.0-76.0) and the loss of circadian cortisol secretion. A CT scan revealed a 3.1 cm, hyperechoic, well-marginated mass in the left adrenal gland. Ten months post-adrenalectomy, the patient had unintentionally lost 9 kg of body weight, had regained a regular menstrual cycle and had normal thickness of her endometrium.
Adrenal Cortex Neoplasms/complications/*diagnosis/surgery ; Adrenalectomy ; Adrenocortical Adenoma/complications/*diagnosis/surgery ; Adrenocorticotropic Hormone/blood ; Adult ; Circadian Rhythm ; Cushing Syndrome/*diagnosis/etiology/physiopathology ; Diagnosis, Differential ; Endometrial Hyperplasia/*diagnosis ; Female ; Humans ; Hydrocortisone/secretion/urine

No abstract available.
ACTH-Secreting Pituitary Adenoma/complications/diagnostic imaging/surgery ; Adenoma/complications/diagnostic imaging/surgery ; Adolescent ; Biomarkers/urine ; Biopsy ; *Circadian Rhythm ; Creatinine/*urine ; Cushing Syndrome/*diagnosis/*urine ; Female ; Humans ; Hydrocortisone/*urine ; Magnetic Resonance Imaging ; Predictive Value of Tests ; Tomography, X-Ray Computed ; Treatment Outcome ; Urinalysis

The most common endogenous cause of Cushing's syndrome is Cushing's disease. The evaluation of patients with suspected Cushing's disease and syndrome requires an understanding of the proper use and limitations of the tests commonly included in the diagnostic work-up. The best screening test for Cushing's syndrome is a 24-hour urine collection with analysis for urinary free cortisol excretion. Low-dose and high-dose dexamethasone suppression tests, corticotropin assays, a corticotropin-release hormone stimulation test and inferior petrosal sinus cathaterization may be required for a definitive diagnosis. MRI is useful in localizing the lesion but some limitations. Surgical removal of the lesion by a trans-sphenoidal appraoch is usually successful, but long-term follow-up is required. We report a case of Cushing disease which shows such typical clinical characteristics of Cushing syndrome as weight gain, skin lesions, truncal obesity, striae, hypertension.
Adrenocorticotropic Hormone ; Cushing Syndrome ; Dexamethasone ; Diagnosis ; Follow-Up Studies ; Humans ; Hydrocortisone ; Hypertension ; Magnetic Resonance Imaging ; Mass Screening ; Obesity ; Petrosal Sinus Sampling* ; Pituitary ACTH Hypersecretion* ; Skin ; Urine Specimen Collection ; Weight Gain

Congenital adrenal hyperplasia (CAH) is characterized by decreased adrenal hormone production due to enzymatic defects and subsequent rise of adrenocorticotrophic hormone that stimulates the adrenal cortex to become hyperplastic, and sometimes tumorous. As the pathophysiology is basically a defect in the biosynthesis of cortisol, one may not consider CAH in patients with hypercortisolism. We report a case of a 41-yr-old man with a 4 cm-sized left adrenal tumorous lesion mimicking Cushing's syndrome who was diagnosed with CAH. He had central obesity and acanthosis nigricans involving the axillae together with elevated 24-hr urine cortisol level, supporting the diagnosis of Cushing's syndrome. However, the 24-hr urine cortisol was suppressed by 95% with the low dose dexamethasone suppression test. CAH was suspected based on the history of precocious puberty, short stature and a profound suppression of cortisol production by dexamethasone. CAH was confirmed by a remarkably increased level of serum 17-hydroxyprogesterone level. Gene mutation analysis revealed a compound heterozygote mutation of CYP21A2 (I173N and R357W).
17-alpha-Hydroxyprogesterone/blood ; Acanthosis Nigricans/complications ; Adrenal Hyperplasia, Congenital/complications/*diagnosis/drug therapy ; Adult ; Cushing Syndrome/diagnosis ; DNA Mutational Analysis ; Dexamethasone/therapeutic use ; Glucocorticoids/therapeutic use ; Heterozygote ; Humans ; Hydrocortisone/urine ; Male ; Mutation ; Obesity/complications ; Steroid 21-Hydroxylase/genetics ; Tomography, X-Ray Computed