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Improvements in abdominal imaging techniques have increased the detection of clinically inapparent adrenal masses, or incidentalomas (AI), the appropriate diagnosis and management of which have become a common clinical problem for health care professionals. Once an adrenal mass has been detected, the clinician needs to address two questions: 1) is the tumor hormonally active? and 2) is there any chance of the mass being malignant? The majority of AI is non-hypersecretory cortical adenomas, but an endocrine evaluation can lead to the identification of subtle hormone excess. An overnight low-dose dexamethasone suppression test, fractionated urinary or plasma metanephrine assay and, in hypertensive patients, establishing the upright plasma aldosterone/plasma renin activity ratio are recommended as preliminary screening steps. Masses greater than 4 cm are at greater risk of malignancy. Morphological imaging features may be helpful in the distinction between benign and malignant forms. Fine-needle aspiration biopsy is an important tool in the evaluation of oncological patients to establish any metastatic disease. Adrenalectomy is indicated by evidence of a functional adrenal mass, or a suspected malignant form. We advocate adrenalectomy of subtle hypercortisolism, especially in the presence of hypertension, obesity, diabetes or osteoporosis potentially aggravated by glucocorticoid excess. A close follow-up is needed, particularly in the first year after diagnosis.
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PMID:A comprehensive approach to adrenal incidentalomas. 1576 28

We report a rare case of primary aldosteronism due to an adrenocortical carcinoma. A 61-year-old woman with a history of hypertension and hypokalemia was referred for evaluation of a 4.2 cm measuring adrenal mass without secondary signs of malignancy. Endocrinological testing was consistent with primary aldosteronism. The patient underwent surgical resection of the adrenal mass; histology revealed an adrenocortical carcinoma. Postoperatively blood pressure, serum potassium, and aldosterone returned to normal. Four months after adrenalectomy, the patient presented again with hypokalemic hypertension and was found to have metastatic disease. Endocrinological investigation revealed primary aldosteronism and subclinical autonomous glucocorticoid hypersecretion. Careful hormonal investigation should be obtained in patients with adrenal masses causing excessive aldosterone secretion. In uncertain cases of primary aldosteronism, we would suggest to measure 18-hydroxycortisol levels, as excessive amounts may indicate adrenocortical carcinoma.
Exp Clin Endocrinol Diabetes 2005 Apr
PMID:Steroid profile in an adrenocortical carcinoma producing aldosterone. 1589 61

Hypertension (HTA) is a very common disease but its origin is well known only in 1 to 5% of the cases. HTA is present in half of the patients who have an adrenal incidentaloma. Clinical data, hormonal sampling, computed tomography and adrenal scintigraphies are necessary to identify hyperfunctioning adrenal tumors. Adrenalectomy is indicated in case of potential malignant tumors and hyperfunctioning tumors. If HTA seems to be not in relation with the adrenal mass, it is recommended to recognize a congenital enzymatic block in order to ovoid an unnecessary adrenalectomy and to search for a preclinical Cushing's syndrome. The last one is associated with HTA in 91% of the cases, and with a morbid obesity, mellitus diabetes or dyslipidemia in 50% of the cases. The removal of the adrenal mass improves the HTA for half of the patients. If the adrenocortical tumor is nonfunctioning, patients have to be followed during a long time. HTA will be considered as "essential" after a new comprehensive analysis performed 3 years later.
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PMID:[Management of adrenal incidentaloma combined with high blood pressure]. 1593 86

The primary adrenal localization of a non-Hodgkin's lymphoma (NHL) is a rare event. We report the case of a 70-yr-old woman, who was admitted at our Institute for a hormonal evaluation after the incidental discovery of a right adrenal mass during ultrasonography (US) performed for cardiovascular disease. At the physical examination, no sign of adrenal hyperfunction was present. She showed only an androgenetic alopecia and her blood pressure was 180/70 mm Hg, with an arrhythmic heart rate of 100 beats/min. No alterations in hormonal and biochemical data were observed. US studies showed a right adrenal mass (major diameter 16 mm), and an abdominal computed tomography (CT) scan confirmed this solid lesion (major diameter 15 mm) with a high density. [75Se] methylnorcholesterol adrenal scintigraphy exhibited a normal symmetrical radiotracer uptake. After 8 months of follow-up, an abdominal CT scan demonstrated a significant increase of the right adrenal mass (major diameter: 40 mm), with a solid tissue density and enhancement after i.v. contrast. [75Se] methylnorcholesterol adrenal scintigraphy showed an absent uptake on the right side versus the contralateral side. The hematological, hormonal and radiological evaluation did not reveal any sign of malignancy. Owing to the mass enlargement and the modification of scintigraphic pattern, the patient underwent unilateral adrenalectomy. Histological examination revealed a primary diffuse large B-cell NHL (REAL classification) of the adrenal gland. After surgery, she underwent a combined polychemotherapy (cyclophospamide, adriamycin, vincristine and prednisone) and subsequently one cycle of radiotherapy. At present, the patient is in good conditions and there are no signs or symptoms of recurrent disease.
Exp Clin Endocrinol Diabetes 2006 Mar
PMID:A primary adrenal non-Hodgkin's lymphoma presenting as an incidental adrenal mass. 1663 81

Incidental adrenal tumours are a common radiological finding. Phaeochromocytomas, though rare, are very important not to be missed, as the consequences could be fatal. Urinary catecholamines and metanephrines measurement is a commonly used method of screening for phaeochromocytomas. We report a patient who had a phaeochromocytoma presented as an incidental adrenal mass on abdominal CT during investigations for recurrent chest infections. The patient had symptoms suggestive of catecholamines excess and hypertension. Repeated urinary catecholamines measurements, plasma catecholamines, 24-hour urinary catecholamines and 24-hour urinary metanephrines all were normal. MIBG scan showed increased uptake in the adrenal mass. Histological examination of the adrenal mass was consistent with phaeochromocytoma. Removal of the adrenal mass resulted in normalisation of blood pressure and abolishing of the patient's symptoms. This case highlights the difficulty in deciding on further management of patients presenting with an adrenal incidentaloma. This case also highlights the difficulty in diagnosing phaeochromocytoma and represents a reminder that plasma and urine catecholamines measurements could be normal in patients with symptomatic phaeochromocytoma. This case also highlights that MIBG scan could reveal a phaeochromocytoma despite absence of biochemical markers.
Exp Clin Endocrinol Diabetes 2006 Apr
PMID:A diagnostic dilemma in diagnosing and managing an incidental phaeochromocytoma. 1670 54

Cushing's syndrome caused by adrenocorticotropic hormone (ACTH)-independent macronodular adrenal hyperplasia (AIMAH) is an extremely rare disease, which shows bilateral macronodular adrenal hypertrophy and autonomous cortisol production. We herein report a case of AIMAH treated successfully by minimally invasive simultaneous bilateral laparoscopic adrenalectomy. A 73-year-old woman with hypertension, diabetes mellitus, and osteoporosis was referred to our hospital because of an incidentally found huge bilateral adrenal mass. An abdominal computed tomography scan showed large bilateral adrenal glands with multiple nodules. A diagnosis of AIMAH was made and a simultaneous bilateral laparoscopic adrenalectomy was thus performed. The total operation time was 310 min and blood loss was 70 g. Both glands were hypertrophic (right 5 x 3 cm, 48.5 g and left 4 x 2 cm, 39.2 g) and consisted of multiple golden yellow macronodules. The postoperative course was uneventful. A simultaneous bilateral adrenalectomy for AIMAH performed by an experienced surgical team is therefore considered to be a safe and minimally invasive procedure.
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PMID:Simultaneous bilateral laparoscopic adrenalectomy for adrenocorticotropic hormone-independent macronodular adrenal hyerplasia: report of a case. 1679 2

Catecholamine-producing tumors may arise in the adrenal medulla (pheochromocytomas) or in extraadrenal chromaffin cells (secreting paragangliomas). Their prevalence is about 0.1% in patients with hypertension and 4% in patients with a fortuitously discovered adrenal mass. An increase in the production of catecholamines causes symptoms (mainly headaches, palpitations and excess sweating) and signs (mainly hypertension, weight loss and diabetes) reflecting the effects of epinephrine and norepinephrine on alpha- and beta-adrenergic receptors. Catecholamine-producing tumors mimic paroxysmal conditions with hypertension and/or cardiac rhythm disorders, including panic attacks, in which sympathetic activation linked to anxiety reproduces the same signs and symptoms. These tumors may be sporadic or part of any of several genetic diseases: familial pheochromocytoma-paraganglioma syndromes, multiple endocrine neoplasia type 2, neurofibromatosis 1 and von Hippel-Lindau disease. Familial cases are diagnosed earlier and are more frequently bilateral and recurring than sporadic cases. The most specific and sensitive diagnostic test for the tumor is the determination of plasma or urinary metanephrines. The tumor can be located by computed tomography, magnetic resonance imaging and metaiodobenzylguanidine scintigraphy. Treatment requires resection of the tumor, generally by laparoscopic surgery. About 10% of tumors are malignant either at first operation or during follow-up, malignancy being diagnosed by the presence of lymph node, visceral or bone metastases. Recurrences and malignancy are more frequent in cases with large or extraadrenal tumors. Patients, especially those with familial or extraadrenal tumors, should be followed-up indefinitely.
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PMID:Pheochromocytomas and secreting paragangliomas. 1715 52

Pheochromocytomas are rare catecholamine-secreting, chromaffin tumors of the autonomic nervous system. Most pheochromocytomas are sporadic, but up to 24% of pheochromocytomas are part of a familial disorder. Here we describe a female patient, who presented to our outpatient clinic 18 years after removal of a pheochromocytoma of the left adrenal gland in China. Now she reported flank pain on the left side and elevated blood pressure. 24-hour urinary catecholamines, metanephrines, and normetanephrines as well as plasma-norepinephrine were elevated. The transabdominal ultrasonography revealed a tumor with reduced echogenicity in the left suprarenal region, which was suspected to be a recurrent pheochromocytoma. This finding was confirmed by MRT and J (123)-MIBG-scan. Parathyroid hormone (PTH) and calcitonin were in the normal range. After surgical excision, histological examination of the adrenal mass proved to be a pheochromocytoma. Molecular genetic analysis with sequencing of the succinate dehydrogenase type B (SDHB) gene revealed a formerly unknown mutation of codon 214 (CAG-->TAG) leading to an amino acid change of glutamine to a stop-Codon (Q214X-mutation) in exon 6. This case report highlights the necessity of re-evaluating patients with nonsyndromic pheochromocytoma who are diagnosed without genetic testing to estimate the risk of a relapse and to initiate testing of first-degree relatives.
Exp Clin Endocrinol Diabetes 2007 Oct
PMID:Relapsing pheochromocytoma in a Chinese women caused by a novel mutation in exon 6 of the SDHB gene: a case report. 1794 98

Subclinical Cushing's syndrome (CS) is attracting increasing interest since the serendipitous discovery of an adrenal mass has become a rather frequent event owing to the routine use of sophisticated radiologic techniques. Cortical adenoma is the most frequent type of adrenal incidentaloma accounting for approximately 50% of cases in surgical series and even greater shares in medical series. Incidentally discovered adrenal adenomas may secrete cortisol in an autonomous manner that is not fully restrained by pituitary feedback, in 5 to 20% of cases depending on study protocols and diagnostic criteria. The criteria for qualifying subclinical cortisol excess are controversial and presently there is no consensus on a gold standard for the diagnosis of this condition. An increased frequency of hypertension, central obesity, impaired glucose tolerance, diabetes and hyperlipemia has been described in patients with subclinical CS; however, there is still no clear demonstration of the long-term complications of this condition whose management remains largely empirical. Either adrenalectomy or careful observation associated with treatment of the metabolic syndrome have been suggested as treatment options.
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PMID:Subclinical Cushing's syndrome. 1820 65

Unilateral adrenal hyperplasia (UAH) is very rare, and shows similar endocrine features to aldosterone-producing adenoma (APA). We report a case of UAH diagnosed preoperatively as APA. Pathological analysis showed that the adrenal mass did not contradict a diagnosis of APA. However, in situ hybridization and real-time PCR indicated that the hyperplastic zona glomerulosa cells rather than the adenoma cells demonstrated intense mRNA expression of steroidogenic enzymes necessary for production of aldosterone. UAH is believed to account for less than 1% of primary aldosteronism, but it is possible that some cases of UAH may be mistakenly considered to be APA, and that the actual frequency of UAH may thus be higher than presumed. Recently, partial adrenalectomy has been attempted. It should be borne in mind that cases exist in which it is difficult to differentiate between APA and UAH only by preoperative hormonal and radiologic investigations.
Exp Clin Endocrinol Diabetes 2009 Mar
PMID:A case of unilateral adrenal hyperplasia being difficult to distinguish from aldosterone-producing adenoma. 1856 Oct 92


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