Adrenal Cortex Neoplasms ; complications ; Adrenal Gland Neoplasms ; complications ; Adrenal Glands ; pathology ; Adrenocortical Adenoma ; complications ; Humans ; Pheochromocytoma ; complications

Non-neoplastic lesions were added in the 5th edition WHO classification of adrenal cortical tumor based on the recent update, including adrenal rests, adrenal cysts, congenital adrenal hyperplasia and adrenocortical nodular disease. A range of tumor concepts were updated or refined based on tumor cell origin, histopathology, oncology and molecular biology. The most significant nomenclature change in the field of adrenal cortical pathology involves the refined classification of adrenal cortical nodular disease, which now includes sporadic nodular adrenocortical disease, bilateral micronodular adrenal cortical disease, and bilateral macronodular adrenal cortical disease. The 5th edition WHO classification endorses the nomenclature of the HISTALDO classification to help the classification of aldosterone producing adrenal cortical lesions, which uses CYP11B2 immunohistochemistry to identify functional sites of aldosterone production. The 5th edition WHO classification does not change the Weiss and Lin-Weiss-Bisceglia histopathologic criteria for diagnosing adrenal cortical carcinomas, and underscores the diagnostic and prognostic impact of angioinvasion in these tumors. Reticulin algorithm and Helsinki scoring system were added to assist the differential diagnosis of adrenal cortical neoplasms in adults. Pediatric adrenal cortical neoplasms are assessed using the Wieneke system. The 5th edition WHO classification places an emphasis on an accurate assessment of tumor proliferation rate using both the mitotic count (mitoses per 10 mm²) and Ki-67 labeling index which play an essential role in the dynamic risk stratification of affected patients. This review highlights advances in knowledge of histological features, ancillary studies, and associated genetic findings that increase the understanding of the adrenal cortex pathologies in the 5th edition WHO classification.
Adult ; Humans ; Child ; Aldosterone ; Adrenal Cortex Neoplasms/chemistry* ; Adrenocortical Carcinoma/pathology* ; Adrenal Gland Neoplasms ; World Health Organization

Aldosterone-producing adrenocortical carcinoma comprises less than 7% of all functioning adrenocortical carcinomas. We report a rare case of adrenocortical carcinoma with a clinical picture of primary aldosteronism and subclinical Cushing’s syndrome and feminization. Complete surgical resection normalized blood pressures and aldosterone, cortisol and estradiol levels. Long-term monitoring is recommended with imaging and hormonal evaluation used as tumor markers for recurrence.
Adrenal Gland Neoplasms ; Adrenocortical Carcinoma ; Hyperaldosteronism ; Hypertension

OBJECTIVETo investigate the expression of chromogranin A (CgA) and synaptophysin (Syn) for differential diagnosis of different kinds of adrenal gland tumors.

METHODSThe samples of 69 adrenal gland tumors and 4 normal adrenal glands were immunohistochemically analyzed for the expression of chromogranin A and synaptophysin. The statistical analysis of the data was performed using chi-square test.

RESULTSIn the normal adrenal gland, CgA and Syn was exclusively detected in the medulla. CgA was detected in all pheochromocytomas 25/25 (100%), and gave less or no expression in adrenocortical tumors. Syn was detected in adrenocortical adenomas 27/28 (96.4%), adrenocortical carcinoma 7/8 (87.5%), pheochromocytoma 24/25 (96.0%) and adrenal metastatic carcinoma 6/8 (75.0%), respectively.

CONCLUSIONThere is statistically significant difference of CgA expression between adrenalcortical and adrenal medullary tumors, and also between benign and malignant pheochromocytomas. CgA and Syn are immunohistochemically reliable markers in the differential diagnosis of various kinds of adrenal gland tumors.

Adrenal Gland Neoplasms ; diagnosis ; metabolism ; Adrenocortical Adenoma ; diagnosis ; metabolism ; Adrenocortical Carcinoma ; diagnosis ; metabolism ; Chromogranin A ; biosynthesis ; genetics ; Diagnosis, Differential ; Female ; Humans ; Male ; Pheochromocytoma ; diagnosis ; metabolism ; Synaptophysin ; biosynthesis ; genetics

BACKGROUND: This study was designed to evaluate the clinical, endocrinological and histological characteristics of adrenal incidentalomas. METHODS: Eighty patients (41, males; 38, females; age range 17-80 years) who were diagnosed with adrenal incidentaloma at Korea University Hospital from 1992 to 2003 were studied retrospectively. RESULTS: Endocrinological investigation revealed 16 pheochromocytomas (20%), nine Cushing's syndromes (11%), eight primary aldosteronism (10%) and 46 non-functioning tumors (58%). Forty-four patients received operations, and biopsies were performed on two patients. Pathologic examination revealed 16 adrenocortical adenomas (20%), five carcinomas (6%), 13 pheochromocytomas (16%), three metastatic cancers (4%), and other tumors (10%). The diameter of the carcinomas (mean: 10.8 cm, range: 5-19 cm) were significantly larger than the diameter of benign adenomas (mean: 2.84 cm, range: 1-6 cm) (p=0.002). According to the receiver operating charactenstic (ROC) curve analysis, the cut-off value of tumor size for discriminate malignant tumor was 4.75 cm (sensitivity 90%, specificity 58%). Twenty-four patients with non-functioning tumors were followed up for a period of 3 to 72 months. During the follow up period, two patients showed an increase in tumor size of more than 1 cm, and one patient developed Cushing's syndrome. Changes in mass size and function were observed only between 10 and 26 months after the initial diagnosis. CONCLUSIONS: These data show that an endocrine evaluation should be performed in all adrenal incidentalomas, and an adrenalectomy is recommended for tumors 5 cm or greater or tumors with adrenocortical hyperfunction. In addition, these tumors should be monitored for changes in mass size and function for a follow up period of approximately 26 months.
Retrospective Studies ; Pheochromocytoma/diagnosis ; Middle Aged ; Male ; Korea ; *Incidental Findings ; Humans ; Female ; Carcinoma/diagnosis ; Aged, 80 and over ; Aged ; Adult ; Adrenocortical Adenoma/diagnosis ; Adrenal Gland Neoplasms/*diagnosis ; Adolescent

The adrenal glands, because of their retroperitoneal location, are difficult to evaluate by physical examination and conventional radiological techniques. This has limited the detection of benign and malignant adrenal neoplasms to those tumors causing local symptoms due to massive enlargement or systemic manifestations of excess hormone production. Technical advances now allow visualization of normal adrenal glands by computed tomography (CT), and the possibility arises that adrenal neoplasms may be diagnosed at an earlier or even preclinical stage. Treatment of these preclinically detected neoplasms would prevent the complications of hormone producing tumors and offer a better chance for cure of malignant tumors. This report describes our experiences with 5 patients who had adrenal tumors; three pheochromocytomas, one primary hyperaldosteronism, one nonfunctioning adrenal cortical carcinoma.
Adrenal Gland Neoplasms ; Adrenal Glands ; Adrenocortical Carcinoma ; Humans ; Hyperaldosteronism ; Pheochromocytoma ; Physical Examination

Except when caused by direct and definite mechanisms (e.g., injury of the vessels to the femoral head), the pathophysiology of avascular necrosis of the femoral head has not yet been fully elucidate. While non-traumatic avascular necrosis of the femoral head is known to be caused by alcohol, steroids and various diseases, it may also occur without such events in a patient's history. Herein, a case of bilateral avascular necrosis of the femoral head caused by asymptomatic adrenal cortex incidentaloma which was initially misdiagnosed as idiopathic is reported along with a literature review.
Adrenal Cortex ; Adrenal Gland Neoplasms ; Cushing Syndrome ; Femur Head Necrosis ; Head ; Humans ; Necrosis ; Steroids

BACKGROUND: In approach to an adrenal incidentaloma, early exclusion of pheochromocytoma is clinically important, due to the risk of catecholamine crisis. The aims of this study are to investigate the characteristics of incidentally detected pheochromocytomas, compared with that of the other adrenal incidentalomas, and to compare these characteristics with those of symptomatic pheochromocytomas. METHODS: In this retrospective study, we reviewed the medical records of 198 patients with adrenal incidentaloma from 2001 to 2010. We analyzed the clinical, laboratory and radiological data of pheochromocytomas, in comparison with those of the other adrenal incidentalomas. We also compared the characteristics of these incidentally detected pheochromocytomas with the medical records of 28 pathologically proven pheochromocytomas, diagnosed based on typical symptoms. RESULTS: Among the 198 patients with adrenal incidentaloma, nineteen patients were diagnosed with pheochromocytoma. Pheochromocytomas showed larger size and higher Hounsfield unit at precontrast computed tomography (CT) than did non-pheochromocytomas. All pheochromocytomas were larger than 2.0 cm, and the Hounsfield units were 19 or higher in precontrast CT. When both criteria of size > 2.0 cm and Hounsfield unit > 19 were met, the sensitivity and specificity for the diagnosis of pheochromocytoma were 100% and 79.3%, respectively. Compared with patients with pheochromocytoma, diagnosed based on typical symptoms, patients with incidentally detected pheochromocytoma were older, presented less often with hypertension, and showed lower levels of 24-hour urine metanephrine. CONCLUSION: Adrenal incidentaloma with < 2.0 cm in size or < or = 19 Hounsfield units in precontrast CT imaging was less likely to be a pheochromocytoma. Patients with incidentally discovered pheochromocytoma showed lower catecholamine metabolites, compared with those patients with symptomatic pheochromocytoma.
Adrenal Gland Neoplasms ; Adrenocortical Adenoma ; Humans ; Hypertension ; Medical Records ; Pheochromocytoma ; Retrospective Studies ; Sensitivity and Specificity

No abstract available.
Adrenal Cortex Function Tests ; *Adrenal Cortex Neoplasms/complications/diagnosis/metabolism/surgery ; *Adrenal Gland Neoplasms/complications/diagnosis/metabolism/surgery ; Adrenalectomy ; *Adrenocortical Adenoma/complications/diagnosis/metabolism/surgery ; Biopsy ; Cushing Syndrome/diagnosis/etiology ; Female ; Humans ; Immunohistochemistry ; *Incidental Findings ; Middle Aged ; *Neoplasms, Multiple Primary/complications/diagnosis/metabolism/surgery ; *Pheochromocytoma/complications/diagnosis/metabolism/surgery ; Predictive Value of Tests ; Tomography, X-Ray Computed ; Treatment Outcome ; Tumor Markers, Biological/metabolism

Adrenocortical carcinoma is a rare type of endocrine malignancy with an annual incidence of approximately 1–2 cases per million. The majority of these tumors secrete cortisol, and a few secrete aldosterone or androgen. Estrogen-secreting adrenocortical carcinomas are extremely rare, irrespective of the secretion status of other adrenocortical hormones. Here, we report the case of a 53-year-old man with a cortisol and estrogen-secreting adrenocortical carcinoma. The patient presented with gynecomastia and abdominal discomfort. Radiological assessment revealed a tumor measuring 21×15.3×12 cm localized to the retroperitoneum. A hormonal evaluation revealed increased levels of estradiol, dehydroepiandrosterone sulfate, and cortisol. The patient underwent a right adrenalectomy, and the pathological examination revealed an adrenocortical carcinoma with a Weiss' score of 6. After surgery, he was treated with adjuvant radiotherapy. Twenty-one months after treatment, the patient remains alive with no evidence of recurrence.
Adrenal Gland Neoplasms ; Adrenalectomy ; Adrenocortical Carcinoma ; Aldosterone ; Dehydroepiandrosterone Sulfate ; Estradiol ; Gynecomastia ; Humans ; Hydrocortisone ; Incidence ; Male ; Middle Aged ; Radiotherapy, Adjuvant ; Recurrence