The metabolic alterations caused by hyperaldosteronism are being increasingly recognized and have generated considerable interest among the medical fraternity. Hyperaldosteronism is suspected to have a pivotal role in the patho-physiology of congestive cardiac failure where it has been studied extensively. But its effects on calcium metabolism, parathyroid metabolism and renal handling of calcium are less well described. Recent experimental models have shed light into the roles played by previously unknown mechanisms in causing these metabolic alterations. We hereby report a case of primary hyperaldosteronism due to adrenal adenoma (Conn's syndrome) who presented with a myriad of clinical features including symptomatic hypocalcemia, significant weight loss along with uncontrolled hypertension for a prolonged period before eventually detected to have primary hyperaldosteronism. Surgical removal of the causative tumor resulted in prompt disappearance of all the symptoms and signs and regain of lost weight.
Adrenal Cortex Neoplasms/*complications/diagnosis/pathology/surgery ; Adrenocortical Adenoma/*complications/diagnosis/pathology/surgery ; Adult ; Female ; Humans ; *Hyperaldosteronism/complications/etiology/physiopathology ; Hypocalcemia/*etiology ; Pregnancy ; Treatment Outcome

The coexistence of myelolipoma within adrenal cortical adenoma is extremely rare, for both tumors present usually as separate entities. There are only 16 such cases reported worldwide. To the best of our knowledge, the case we reported here is the first one of myxoid adrenal cortical adenoma associated with myelolipoma reported. A 32-year-old Chinese woman with 4-year history of hypertension was presented in our study. Computed tomography (CT) of the abdomen showed a large heterogeneously-enhancing mass (4.5 cm in diameter) in the left suprarenal region. Clinical history and laboratory results suggest a metabolic disorder as Conn's syndrome. The patient underwent a left adrenalectomy, and a histopathological study confirmed the mass to be a myxoid adrenal cortical adenoma containing myelolipoma. The patient was postoperatively well and discharged uneventfully. In the present case report, we also discuss the etiology of simultaneous myelolipoma and adrenal adenoma associated with Conn's syndrome, and the methods of the diagnosis and differential diagnosis.
Adrenal Cortex Neoplasms ; complications ; diagnosis ; pathology ; Adrenocortical Adenoma ; complications ; diagnosis ; pathology ; Adult ; Biomarkers, Tumor ; metabolism ; Female ; Humans ; Hyperaldosteronism ; complications ; Inhibins ; metabolism ; Myelolipoma ; complications ; diagnosis ; pathology ; Neoplasms, Multiple Primary ; complications ; diagnosis ; pathology ; Synaptophysin ; metabolism ; Tomography, X-Ray Computed ; Vimentin ; metabolism

In patients with primary aldosteronism who have bilateral adrenal incidentalomas, it is important to identify which adrenal gland is secreting excess aldosterone. Traditionally, adrenal vein sampling (AVS) has been performed for lateralization despite its invasiveness. Here we report a case of bilateral adrenal incidentaloma in which 18-Fluorodeoxyglucose (FDG)-positron emission tomography (PET) was used to identify the functional adrenal mass. A 53-yr-old man was referred to our clinic due to bilateral adrenal incidentalomas (right: 1 cm, left: 2.5 cm) on computed tomography (CT). Given his history of colon cancer, FDG-PET/CT scanning was used to rule out metastasis. Although there was focal hot uptake lesion in the right adrenal gland, the patient was suspected primary aldosteronism clinically more than metastasis because of the patient's underlying hypertension with hypokalemia. It was consistent with the results of AVS. Based on these findings, we propose that FDG-PET/CT can be used instead of AVS to identify the source of primary aldosteronism between two bilateral adrenal incidentalomas.
Adrenal Gland Neoplasms/*diagnosis/pathology/radionuclide imaging ; Adrenal Glands/pathology/surgery ; Fluorodeoxyglucose F18/diagnostic use ; Humans ; Hyperaldosteronism/*diagnosis/pathology ; Hypertension/diagnosis ; Hypokalemia/diagnosis ; Male ; Middle Aged ; Positron-Emission Tomography and Computed Tomography

BACKGROUND: Primary aldosteronism describes a group of disorders characterized by long-standing aldosterone excess, with suppressed renin activity, resulting in hypertension and hypokalemia. The protean clinical and biochemical characteristics of this syndrome have important implications regarding its pathophysiology and responsiveness to treatment. METHODS: The cases of 12 primary aldosteronisms, diagnosed at Hanyang University Hospital between 1996 and 1999, were reviewed. RESULTS: The 12 cases were composed of 9 aldosterone-producing adenoma, 2 adrenal hyperplasia and a case of idiopathic hyperaldosteronism. There were 9 women and 3 men. The mean age was 46 yrs (range, 23 to 64 yrs). At the initial visit, the mean blood pressure was 160+/-26/104+/-14 mmHg, and one case of idiopathic hyperplasia had normal blood pressure. The mean serum K+ level was 2.6+/-0.5 mEq/L (range, 1.5 to 3.5 mEq/L). The mean plasma renin activity and plasma aldosterone concentration were 0.4+/-0.4 ng/ml/hr (range, 0.2 to 1.6 ng/ml/hr) and 407.5+/-199.8 pg/mL (range, 225 to 800 pg/mL), respectively. Different steps of diagnostic modalities were applied for the preoperative differential diagnosis. All patients, with the exception of the one with idiopathic hyperaldosteronism, were managed by a unilateral laparoscopic adrenalectomy, as they were all diagnosed under the impression of adrenal adenomas. Ultimately, 9 cases were proven to have adrenal adenomas. One hypertensive case, with hypokalemia, had adrenal hyperplasia, and the case with normotension was found to have adrenal nodular hyperplasia from the pathology. The size of the tumors ranged from 1.4 to 2.4 cm in diameter. Among the 11 cases that underwent an adrenalectomy, the blood pressures in 6 cases normalized after the operation, while the other 5, including the one with unilateral hyperplasia, were still in need of antihypertensives for the control of elevated blood pressures, even after the operation. The other case of idiopathic hyperaldosteronism was managed by the prescription of spironolactone. CONCLUSION: From these, it can be suggested that the clinical diversity of the syndrome, especially in the pathophysiology and response to operation, awaits the development of a better preoperative lateralization procedure
Adenoma ; Adrenalectomy ; Aldosterone ; Antihypertensive Agents ; Blood Pressure ; Diagnosis, Differential ; Female ; Humans ; Hyperaldosteronism* ; Hyperplasia ; Hypertension ; Hypokalemia ; Male ; Pathology ; Plasma ; Prescriptions ; Renin ; Spironolactone

Multiple endocrine neoplasia type 1 (MEN1) syndrome includes varying combinations of endocrine and non-endocrine tumors. There are also a considerable number of atypical MEN1 syndrome. In this case, a 68-yr-old woman was referred to the Department of Endocrinology for hypercalcemia. Five years ago, she had diagnosed as primary hyperaldosteronism and now newly diagnosed as parathyroid hyperplasia with laboratory and pathologic findings. Hurthle-cell thyroid cancer was also resected during the parathyroid exploration and small meningioma was found on brain MRI. Her general condition has markedly improved and her adrenal mass and meningioma are being closely observed now. We could find the loss of heterozygosity of the MEN1 locus in parathyroid glands, suggesting a MEN1-related tumor, but not a germline mutation. Considering a variety of phenotypic expression and a limitation of current molecular analysis, periodic follow up will be needed in patients with a MEN1-like phenotype.
Aged ; Base Sequence ; Brain/radionuclide imaging ; Female ; Humans ; Hyperaldosteronism/complications/*diagnosis ; Hyperparathyroidism, Primary/*diagnosis/etiology/pathology ; Loss of Heterozygosity ; Magnetic Resonance Imaging ; Meningeal Neoplasms/complications/*diagnosis/radionuclide imaging ; Meningioma/complications/*diagnosis/radionuclide imaging ; Mutation ; Parathyroid Glands/pathology ; Proto-Oncogene Proteins/genetics/metabolism ; Sequence Analysis, DNA ; Thyroid Neoplasms/complications/*diagnosis/pathology ; Tomography, X-Ray Computed