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Adrenal infections are an important but under-recognized clinical entity. The adrenal gland can be infected by a myriad of pathogens including fungi, viruses, parasites, and bacteria. Infection can directly or indirectly cause tissue damage and alteration in endocrine function. Direct damage occurs via microbial replication and local production of toxic compounds, such as endotoxins. Indirect damage results from alterations in the regulation of a host's immunologic and endocrine mediators in response to damage by a microbe at a distant site. Variations in pathogen tropism, adrenal anatomy, and host immune integrity contribute to the progression of active disease and discernable adrenal dysfunction. Early recognition and intervention in the case of adrenal infection can significantly improve outcome, demonstrating the need for increased clinical suspicion in the appropriate clinical setting.

A 34-year-old man presented with a two-month history of intermittent fever and left loin pain. A large left suprarenal mass was detected on computed tomography and magnetic resonance imaging. Blood pressure, serum electrolytes, serum cortisol and urinary catecholamines were normal. Laparoscopic adrenalectomy was planned but the mass was found to be an isolated adrenal abscess due to Nocardiosis. He was later found to have AIDS. The clinical utility of various imaging modalities and management of adrenal cysts are reviewed.

Adrenal pseudocyst is the commonest type of benign lesions of adrenal gland althought is a very rare entity. Most of them are found as "incidentalomas" during imaging studies. A case of a patient with a left non-functioning and asymptomatic adrenal pseudocyst is reported. The diagnostic and therapeutic options are discussed and the literature is reviewed.

Background/aims: Heterotopy is defined as abnormal localization of well-differentiated tissue. Heterotopic tissues usually tend to be asymptomatic and noncomplicated but sometimes may cause serious clinical problems. Malignancy potential is the most important issue in this clinical entity. In this study we reviewed medical records of 24 patients with heterotopic tissues., Methods: Between 1995-2004, 24 patients with heterotopic tissues who were diagnosed during gastrointestinal system or abdominal wall surgery or upper gastrointestinal endoscopy were included in this study. Patients' medical data were retrospectively reviewed., Results: Sixteen patients with heterotopy were younger than 30 years and eight patients were older than 30 years. Nineteen (0.21%) heterotopic tissues were diagnosed in 8,945 patients who underwent gastrointestinal system surgery or upper gastrointestinal endoscopy, whereas five (0.21%) heterotopic tissues were diagnosed in 2,320 patients who underwent abdominal wall surgery. Overall, 24 (0.21%) heterotopic tissues were found in a total of 11,265 patients. The majority were pancreatic heterotopy, followed in decreasing order by gastric, adrenal and osseous heterotopy. In patients who underwent gastrointestinal surgery-endoscopy, pain was the main symptom (n=13)(68.4%), followed by dyspepsia (n=3)(15.7%) and vomiting- nausea (n=2)(10.5%). The main symptom in patients who underwent abdominal wall surgery was palpable mass. heterotopic tissues presented as wall thickening in 13, polypoid mass in five and whole solitary mass or intraparenchymal lesion in six patients., Conclusion: Although incidence of heterotopic tissues is low, in case of its suspicion or diagnosis, early treatment should be performed by surgical or endoscopic resection or patients must be followed up carefully due to risk of malignancy.

The laparoscopic approach to the adrenal gland has evolved to be the gold standard for most cases of adrenal conditions requiring surgical treatment. There is general consent about the safety, efficacy, and reproducibility of laparoscopic adrenal surgery. Compared to the open surgery, significant advantages with regard to shorter hospitalization time, decreased postoperative morbidity, improved cosmetics, and quicker convalescence are evident. The anatomic location of the adrenal gland led to the development of various approaches, including lateral transperitoneal, anterior transperitoneal, lateral retroperitoneal, posterior retroperitoneal, and even transthoracic approaches. The lateral transperitoneal approach is the technique most frequently used for laparoscopic adrenalectomy. A large operative field provides good orientation and visualization of familiar landmarks known from open surgery. In particular in the early learning curve this represents an advantage of the transperitoneal laparoscopic approach. This article describes in detail the indications, contraindications, preoperative evaluation, surgical technique, management of intraoperative complications, and outcome after lateral transperitoneal adrenalectomy.

A case of a morphologically distinctive tumor of the adrenal medulla occurring in a 54-year-old woman is described. On microscopic examination, the tumor was well circumscribed and characterized by the presence of ill-defined, irregular nests of spindle cells with oval to elongated nuclei, tiny nucleoli, and abundant eosinophilic cytoplasm. The tumor was associated with a moderate infiltrate of lymphocytes and plasma cells with occasional lymphoid follicles. Necrosis, marked cellular atypia, and mitoses were absent. Immunohistochemical studies demonstrated the tumor cells to be strongly reactive for vimentin, S-100 protein, and CD56, and nonreactive for glial fibrillary acidic protein, chromogranin, synaptophysin, melanoma-associated antigens, and dendritic cell markers. Ultrastructural examination showed elongated cells with interdigitating cytoplasmic processes devoid of a basal lamina. No secretory granules were noted. The morphology, immunophenotype, and ultrastructure of this unique neoplasm suggest derivation from sustentacular cells of the adrenal medulla. We propose the designation "sustentaculoma" for this hitherto undescribed neoplasm of the adrenal gland.

We report a rare case of ACTH-independent macronodular adrenal hyperplasia (AIMAH) with primary hyperparathyroidism (PHPT). A 57-year-old woman was admitted to our hospital for further examination of secondary hypertension and bilateral adrenal macrotumors. Midnight serum cortisol elevation with undetectable plasma ACTH, increased 24-hour urinary free cortisol excretion, and loss of the normal circadian rhythm in cortisol secretion established the diagnosis of Cushing's syndrome. Total resection of the enlarged left adrenal gland was performed with subsequent steroid replacement. Her general condition improved but serum calcium level increased 3 weeks after surgery. PHPT was diagnosed on the basis of endocrinological examination, although imaging studies failed to detect parathyroid lesion. In summary, we believe this to be the first report of a case of AIMAH with PHPT.

A 45-year-old woman whose MR images revealed a lobulated, complicated cyst with septations on the superior pole of left kidney underwent retroperitoneoscopic cyst marsupialization. In pathologic examination beneath the atrophic adrenal gland, hyalinized, calcification foci cyst wall without any surrounding epithelium was seen microscopically. It was reported to be "adrenal pseudocyst".

A case of isolated, bilateral, adrenal tuberculosis is presented. A 25-year-old male was admitted to the hospital due to lumbar pain, fever, weight loss and anorexia. Abdominal ultrasonography and computed tomography demonstrated bilateral adrenal enlargement. Laboratory investigations were remarkable for adrenal insufficiency. The Mantoux reaction was positive but there was no evidence of lung or urinary infection. CT-guided biopsy of the left adrenal gland was performed and established the diagnosis of adrenal tuberculosis., (Copyright (c) 2006 S. Karger AG, Basel.)

Unlabelled: Clinically silent adrenal masses (incidentaloma) are incidentally discovered lesions when noninvasive imaging methods (ultrasonography--USG, computer tomography--CT, magnetic resonance imaging--MRI) are performed for the reason other than known or suspected adrenal disease. Most of studies report the prevalence of adrenal incidentaloma range between 1 and 10% in radiological series., Material and Methods: Between 1993 and 03.2004 we observed 198 patients with incidentalomas of adrenal glands (144 females--72.7% and 54 males--27.3%)., Results: After endocrinological evaluation, 164 patients were qualified for surgery. In 119 (72.5%) cases open adrenalectomy was performed, and in 45 (27.5%) laparoscopic adrenalectomy was done. Adrenocortical adenoma was diagnosed in 54.9%, adrenal hyperplasia in 8.5%, adrenal carcinoma in 6.7%, pheochromocytoma in 12.9% (in 3.1% of all cases malignant pheochromocytoma was diagnosed), in 4.9% others malignant tumors (primary or metastatic), in 4.9% adrenal cysts and in 7.1% other rare adrenal pathologies were found., Conclusion: All malignancies were found in tumors with the diameter over 3 cm. In tumors with diameter over 6 cm malignant cases were found in 70.8%.

This study aimed at describing the magnetic resonance (MR) imaging features of the adrenal glands in beta-thalassemic patients and at investigating the relation between adrenal and hepatic siderosis. Adrenal signal intensity (SI) was retrospectively assessed on abdominal MR studies of 35 patients with beta-thalassemia major undergoing quantification of hepatic siderosis and 12 healthy controls, using T1-(120/4/90), intermediate-(120/4/20), and T2*-(120/15/20) weighted GRE sequences. Adrenal SI was graded as grade 0 (normal SI on all sequences), grade 1 (hypointensity on T2* alone), or grade 2 (hypointensity on at least T2*). Adrenal size was measured in the thalassemic patients and compared with normative data. Liver-to-muscle (L/M) SI ratios, expressing hepatic siderosis, were estimated on each sequence. Serum ferritin levels were recorded. Adrenal hypointensity (grades 1 and 2) was noted in 24/35 (68.6%) patients. L/M ratios correlated significantly with adrenal SI in all sequences. Patients with grade 1 and grade 2 adrenal SI had significantly decreased L/M ratios compared with grade 0. Serum ferritin correlated significantly with L/M values but not with adrenal SI. Adrenal size was within normal limits. Diffuse hypointensity in normal-sized adrenals is a common MR finding in beta-thalassemic patients and correlates with the degree of hepatic siderosis.

Background: Laparoscopic adrenalectomy is an attractive alternative to open surgery, but making the transition can be difficult., Aim: To evaluate the initial experience of a general surgical team at a single institution at making the transition., Methods: The details of 15 patients undergoing laparoscopic adrenalectomy were prospectively recorded over a 21-month period., Results: Fifteen glands were removed from fifteen patients. Nine of these were left-sided. The mean gland size was 3.4 cm. Pathology included six non-functioning adenomas, four Conn's syndrome, two Cushing's syndrome and three phaeochromocytomas. Mean operating time was 74 minutes (range 31-172 minutes), with one conversion to open procedure. There were no morbidities and no mortality., Conclusion: Our initial experience demonstrates this approach to be the ideal technique for removal of benign adrenal tumours with significant advantages for the patient.

Staphylococcus aureus has increasingly been recognized as a cause of severe invasive illness. We describe three children who died at our institution after rapidly progressive clinical deterioration from this infection, with necrotizing pneumonia and multiple-organ-system involvement. The identification of bilateral adrenal hemorrhage at autopsy was characteristic of the Waterhouse-Friderichsen syndrome, a constellation of findings usually associated with fulminant meningococcemia. The close genetic relationship among the three responsible isolates of S. aureus, one susceptible to methicillin and two resistant to methicillin, underscores the close relationship between virulent methicillin-susceptible S. aureus and methicillin-resistant S. aureus isolates now circulating in the community., (Copyright 2005 Massachusetts Medical Society.)

We report on a DAX1 gene investigation in a patient with X-linked adrenal hypoplasia congenita (AHC) and hypogonadotropic hypogonadism (HH) in order to identify mutations causing this disorder and to confirm the clinical diagnosis. The description of the clinical course of the condition with a detailed documentation of longitudinal data is also reported. A male newborn was referred at 45 days of life because of vomiting, dehydration, and weight loss. The diagnosis was primary adrenal insufficiency. The appropriateness of glucocorticoid therapy during the prepubertal period was difficult to judge because of elevated ACTH levels on one hand and progressive retardation of bone age on the other hand. Basal and GnRH stimulated gonadotropin levels remained low during the entire period of examination and exogenous gonadotropin treatment was begun. This had to be interrupted at age 14.6 years because of the occurrence of a 3rd degree anaplastic ependimoma of the left posterior-parietal region, without apparent lepto-meningeal involvement. The molecular analysis of DAX1 gene of the propositus showed deletion of nucleotides AAT in exon 2, resulting in the loss of the Asn430. No alterations were found in the mother and grandmother. This deleted residue lies in one of the helices forming the hydrophobic core of the ligand-binding domain (LBD); thus this mutation may be the cause of the observed phenotype. Further investigations are needed to verify its causal role in AHC associated with HH.

Adult mouse mutants homozygous for an engineered proopiomelanocortin (POMC)-null allele lack macroscopically distinct adrenal glands and circulating adrenal hormones. To understand the basis for this adrenal defect, we compared the development of adrenal primordia in POMC-null mice and littermate controls. POMC-null mutant mice are born with adrenal glands that are morphologically indistinguishable from those of their wild-type littermates. However, in mutants adrenal cells fail to proliferate postnatally and adrenals atrophy until they have disappeared macroscopically in the adult. While present, mutant adrenals are differentiated as evidenced by the presence of enzymes for the final steps in the synthesis of corticosterone, aldosterone, and catecholamines. However, in contrast to adrenals of wild-type littermates, adrenals of POMC-null mutants do not produce corticosterone, not even in response to acute stimulation with exogenous ACTH. They do produce aldosterone; however, it is produced at reduced levels correlating with adrenal size. Transplantation of POMC-null mutant adrenals to adrenalectomized wild-type littermates results in adrenals with normal morphology and production of both corticosterone and aldosterone. These findings demonstrate that POMC peptides are not required for prenatal adrenal development and that POMC peptides in addition to ACTH are required for postnatal proliferation and maintenance of adrenal structures capable of producing both glucocorticoids and mineralocorticoids.

Technological developments in cross-sectional imaging have revolutionized the localization and characterization of functioning adrenal pathology. With effective use of modern imaging, the diagnosis of the cause and nature of functioning adrenal pathology can be reached speedily, accurately and efficiently in the majority of patients. We review the appearance of primary and secondary adrenal pathology, evaluate the diagnostic performance of imaging modalities, highlight newer technical developments, and propose a rational use of these tests in identifying functioning adrenal disease.

Adrenal cysts are uncommon lesions and most of them are found incidentally during abdominal imaging. We report on two benign extraadrenal lesions mimicking adrenal tumors in abdominal imaging. The histopathological investigation of the lesions revealed a foregut duplication cyst of the lesser gastric curvature and an epithelial inclusion cyst (epidermoid cyst) in an intrapancreatic accessory spleen respectively.

Granulocytic sarcoma (GS) is a collection of immature myeloid cells contained outside the bone marrow often seen in association with acute myeloid leukemia or as a sign of leukemic transformation in myeloproliferative disorders. Rarely, GS is an isolated finding unaccompanied by another hematological disorder. GS can occur at a variety of sites, most commonly involving the bone, soft tissue, lymph nodes, or skin. We report GS presenting as bilateral adrenal masses in a patient without an antecedent hematologic illness., (2005 Wiley-Liss, Inc.)

In the abdominal ultrasonography, the representation of normal adrenal glands is frequently problematic, also for experienced practitioners in ultrasonography. During a seminary at the congress of the SGUM in Davos, in June 2004, it was specially entered to this problematic by anatomical illustrations and live demonstrations. These statements will be summarized in the following article. Also, the technics of examination of the adrenal glands will be explained, especially in comparison to anatomical cut-preparations. It will be entered to particular pathological statements of the adrenal glands. The proceeding will be described by the localisation of accidentally detected tumours of adrenal glands.

We report a case of non-typhi Salmonella adrenal abscess in a patient with human immunodeficiency virus (HIV) infection and thalassaemia minor. Non-typhi Salmonella bacteraemia is common in HIV-infected patients, but infection localized in the adrenal gland is rare. Focal salmonellosis is common in haemoglobinopathy but unusual in thalassaemia minor. This patient was diagnosed by computed tomography (CT) guided aspiration and successfully treated with ciprofloxacin.

ACTH-independent, cortisol-producing hyperplasia is caused by 2 distinct disorders, primary pigmented nodular adrenocortical disease (PPNAD) and ACTH-independent macronodular adrenal hyperplasia (AIMAH). We will review recent findings on the clinical and molecular aspects of PPNAD and AIMAH. Inactivating mutations of PRKAR1A on 17q22-24, which codes for the type 1A regulatory subunit of protein kinase A, have been found in a subgroup of patients with PPNAD with and without Carney complex. AIMAH is a rare condition in which cortisol secretion may be mediated by non-ACTH circulating hormones such as gastric inhibiting polypeptide (leading to food-dependent Cushing's syndrome), vasopressin, catecholamines, luteinizing hormone, serotonin, angiotensin-II or leptin. The primary etiology of AIMAH remains unclear. Recently, we studied the expression profile of AIMAH by genomic cDNA microarray analysis. Several candidate genes were identified, suggesting pathways that affect the cell cycle, adhesion and transcription as possible mediators of adrenocortical hyperplasia.

We report a case of a right-sided cystic adrenal mass, detected after the 21st week of gestation, associated with fetal macrosomia. The diagnosis of Beckwith-Wiedemann syndrome was evoked. Prenatal sonography and magnetic resonance imaging did not allow establishing the origin of the suprarenal mass. The differential diagnosis of cystic neuroblastoma, pseudocystic adrenal haemorrhage, and adrenocortical macrocysts was discussed. A laparotomy was performed 2 weeks after birth due to the increasing size of the tumour and due to its possible malignant origin. Histological findings were haemorrhagic lesions without evidence of malignancy and adrenal cortical cytomegaly. The diagnosis of an adrenocortical macrocyst component of Beckwith-Wiedemann syndrome was established., (2004 S. Karger AG, Basel.)

The detection of adrenal lesions has increased with the expanding use of cross-sectional imaging. Magnetic resonance (MR) imaging is often useful for characterizing adrenal masses. Adrenal masses can be classified into various groups on the basis of the presence of intracellular lipid, macroscopic fat, hemorrhage, and cystic changes and the vascularity and shape of the tumor. These imaging features can be used by the radiologist to suggest or confirm a diagnosis for most adrenal masses, including adenoma, hyperplasia, simple and complicated cysts, lymphangioma, myelolipoma, pheochromocytoma, hemorrhage, cortical carcinoma, neuroblastoma, lymphoma, and metastases. Adenomas and metastases are common, and a decrease in signal intensity on out-of-phase images can be used to differentiate between them. Carcinoma is a possible diagnosis if that decrease in signal intensity is heterogeneous. Benign disease is diagnosed if macroscopic fat or a homogeneous cystlike lesion is seen. Recognition of the typical MR imaging features is important because it often changes the treatment approach and may obviate surgery., (Copyright RSNA, 2004)

The adrenal gland is one of the organs which tuberculosis infects. In most clinical settings bilateral adrenal tuberculosis has been clarified after adrenal insufficiency is overt. On the contrary, active adrenal tuberculosis is rarely detected during the survey of infectious disease. A 68-year-old man was admitted because of intermittent fever. The fever had continued for the last 3 months. The intermittent fever was accompanied with leukocytosis and elevation of C-reactive protein. Serum soluble interleukin-2 receptor was 1920 U/ml, and beta2-microglobulin was 4.0 mg/l. Bacterial cultures of blood, sputa, urine, bone marrow and cerebrospinal fluid did not show any particular bacteria. Mycobacterium tuberculosis was negative in culture of sputa, and there was no tuberculin reaction. Plasma ACTH and serum cortisol were 18.5 pmol/l and 527.0 nmol/l, respectively. Abdominal CT scan showed right adrenal mass with a size of 28 x 20 mm, which was low density and had a well-encapsulated homogenous appearance. After the adrenalectomy, histology verified active adrenal tuberculosis. The intermittent fever disappeared, and white blood cells and C-reactive protein normalized. These findings indicate an atypical, rare case of unilateral, active adrenal tuberculosis closely linked to intermittent fever, and without any other organ involvement.

Background: Cystic lesions of the adrenal gland are uncommon and demonstrate a spectrum of histologic changes and may vary from pseudocysts to malignant cystic neoplasms., Methods: The authors analyzed a series of primary cystic lesions of the adrenal gland to evaluate the clinicopathologic features and histologic spectrum and to determine features that are helpful in distinguishing benign from malignant cystic adrenal tumors., Results: A total of 41 cases of macroscopically cystic lesions among patients who underwent surgery at the Mayo Clinic were identified over a 25-year period. Of these 41 cases, 32 were pseudocysts, 8 were endothelial cysts, and 1 was an epithelial cyst. Of the 32 pseudocysts, 6 were associated with adrenal neoplasms, including 2 adrenal cortical carcinomas, 2 adrenal cortical adenomas, and 2 pheochromocytomas. One pheochromocytoma case was identified in association with an endothelial cyst. Both patients with cystic adrenal cortical carcinomas died of disease., Conclusions: Adrenal neoplasms, including adrenal cortical carcinomas, may be associated with benign-appearing cysts. Extensive pathologic sampling of resected tissues is important to rule out malignancy in patients with cystic adrenal lesions., ((c) 2004 American Cancer Society.)

We report a case of asymptomatic adrenal hematoma that enlarged during a 3-year follow-up. A 66-year-old woman exhibited a 4 cm right adrenal mass, incidentally discovered by computed tomography of abdomen, which progressively enlarged to 6 cm in diameter during the 3 years. The patient underwent right adrenalectomy with a diagnosis of a suspected non-functional adrenal tumor. Histopathological examination indicated adrenal hematoma without tumor cells. The patient had received aspirin medication for 4 years, and it is possible that the enlargement of the mass might have been due to aspirin medication.

Non-iatrogenic anatomical findings at autopsy provide insight into preterm infant physiology. The different patterns of lipid accumulation in the adrenal may correspond to long-term differences in stress response. Cardiac papillary muscle infarction occurs with asphyxia or shock and can explain myocardial dysfunction. Underdevelopment of preterm kidneys may correlate with susceptibility to renal disease and hypertension in adult life. Immaturity of the lung or immature responses to inflammation, rather than high oxygen concentrations or high ventilation pressures, may underlie chronic lung disease in premature infants. Hepatic extramedullary haematopoiesis is normal but, if excessive or abnormally persistent, can be an indicator of fetal disease. Hypertrophic somatostatin islet cells found with intra-uterine growth retardation may correlate with low serum insulin. Thymic involution may mark the degree of stress. Small thyroglobulin stores may limit the premature neonate's initiation of thermogenesis., (Copyright 2004 Elsevier Ltd.)

We describe an unusual adrenal pseudocyst mimicking radiologically and clinically renal mass. The cyst measured 12 cm in diameter and had a fibrotic external envelope that was fused with the renal capsule. The possible diagnostic pitfalls encountered in this case are discussed.

We present the case of a patient who had a large pseudocyst in the right adrenal gland, which was ruptured following blunt abdominal trauma, leading to a voluminous hemorrhage in retroperitoneum. A 29-year old female patient was admitted in the emergency room following a fall from stairs with trauma in right flank. She underwent a computerized tomography that evidenced a large retroperitoneal collection, with no apparent renal damage. She was submitted to surgery, where a large ruptured cyst was observed, originating from the upper portion of the right adrenal gland. Cystic diseases of adrenal gland are rare. Highly voluminous cysts can be damaged in cases of blunt trauma to the lumbar region leading to large hematomas in retroperitoneum.

Unlabelled: After introduction of the transperitoneal endoscopic adrenalectomy in 1992 and retroperitoneal adrenalectomy (RA) in 1994, the both techniques were widely accepted in the routine surgery practices. Now some authors described them as a "golden standard" in the surgical treatment of adrenal disorders., Material and Method: 34 retroperitoneal endoscopic adrenalectomy were performed in our clinic for the period of May 1996-December 2002., Results: The mean operating time was 125 minutes (45-220 min.) The average itraoperative blood loss was 70 ml. Only in one case the blood loss was 300 ml, causing conversion to open adrenalectomy. In 6 cases we converted to open adrenalectomy for other reasons--17.6%. In the last 15 cases we had no need of conversion to open adrenalectomy. Intraoperative complications were minor--2 cases (5.88%). Consumption of analgesics was low. Hospitalization period was 3 days., Conclusions: In summary, EA is associated with fewer complications than have been reported with OA. Despite the limitations of retrospective analysis, the results of this technique are very impressing in relation to diminishing the operative trauma, postoperative pain syndrome and faster recovery. EA is to become a method of choice in surgical treatment of the benign adrenal disorders.

During the past 15 years, considerable progress has been made in our understanding of the genetic basis of adrenal development and function. More than 30 single gene disorders have now been identified that can affect the hypothalamic-pituitary-adrenal axis in humans (fig. 1, 2; table 1). This review highlights recent advances in the molecular pathology of: (1) adrenal hypoplasia, (2) adrenal destruction, (3) disorders of adrenal steroidogenesis, (4) adrenal steroid resistance and (5) activation of the adrenal axis/tumorigenesis. Characterizing the molecular basis and natural history of these conditions is providing fascinating insight into adrenal development and function and can help to focus treatment and counselling of patients appropriately. However, ongoing translation of research findings into clinical practice is needed if patient care is to be influenced significantly., (2004 S. Karger AG, Basel.)

Objective: To investigate the value of ultrasonography in the diagnosis of adrenal gland cysts., Methods: Six patients with adrenal gland cysts were examined by two-dimensional ultrasonography and color Doppler flow imaging (CDFI), and the results were compared with the pathological findings., Results: The diagnoses by ultrasonography and CDFI were consistent with the pathological findings in 5 cases., Conclusion: Ultrasonography and CDFI can be an accurate diagnostic modality for adrenal gland cysts.

ACTH-independent macronodular adrenal hyperplasia (AIMAH) is a rare cause of Cushing's syndrome. Bilateral adrenalectomy is considered to be a standard therapy for AIMAH, although lifetime replacement of glucocorticoids is necessary after the procedure. This paper describes a subject with AIMAH who underwent unilateral adrenalectomy of the predominantly enlarged gland and subsequently displayed an improvement in insulin resistance and diabetes mellitus, the cardinal symptoms before the operation, concomitant with alleviation of abnormal cortisol secretion. The patient was a 61-year-old man with a body mass index of 25.6 kg/m2. He was diagnosed as having diabetes mellitus, hypertension, and hyperlipidemia at 50 years of age. Eight years after diagnosis, bilateral enlargement of the adrenal glands was revealed by chance upon computed tomography of the abdomen. Typical manifestations of Cushing's syndrome were not demonstrated. Basal levels of serum and urinary cortisol had not increased, although the serum cortisol level displayed no circadian rhythm and no response to the administration of dexamethasone. Despite sulfonylurea treatment, the patient's HbA1C level was as high as 7.6% (normal range 4.3-5.8%). Fasting insulin concentration was increased to 42.6 microU/ml, and the homeostasis model insulin resistance index (HOMA-R) was calculated to be 15.5 (with a normal range of less than 2.5), indicating severe insulin resistance. Unilateral adrenalectomy of the predominantly enlarged gland revealed that the resected gland consisted of multiple nodules of various sizes. Based on endocrinological, radiological, and pathological findings, a diagnosis of AIMAH was made. Ten months after the unilateral adrenalectomy, cortisol circadian rhythms were restored, and serum cortisol concentration was suppressed in response to the administration of low doses of dexamethasone, suggesting an improvement in the cortisol secretory pattern. Levels of HbA1C, fasting insulin, and HOMA-R decreased to 5.7%, 12.7 microU/ml, and 2.2, respectively. An improvement in hyperlipidemia was also observed. Insulin resistance and glucose intolerance are recognized as features of mild hypercortisolism. In the present case, unilateral adrenalectomy was effective in ameliorating insulin resistance and improving glycemic control. Unilateral adrenalectomy might be an alternative therapy for improvement of glucose and lipid metabolism in subjects with AIMAH.

We report our experience with two cases of adrenal cytomegaly, both of which were detected as cystic adrenal masses during prenatal ultrasonographic examinations. In Case 1, a left suprarenal cystic mass was detected in the fetus at 25 weeks of gestation. The mass, measuring 7 cm in diameter, did not show any change in size and was resected 26 days after birth. In Case 2, a right suprarenal lesion was found at 30 weeks of gestation. The cystic lesion, measuring 2 cm x 1.5 cm, did not change in size and was resected 3 months after birth. Adrenal cytomegaly is still not well known. It is characterized by the presence of large polyhedral cells with eosinophilic granular cytoplasm and enlarged nuclei in the adrenal cortex. This condition is thought to be a degenerative process but not a malignancy. Adrenal cytomegaly rarely forms cysts. It seemed to be impossible to diagnose preoperatively in our cases. Because of the difficulty of differentiating between cystic adrenal cytomegaly and other cystic diseases such as neuroblastoma, operative intervention is required in cases where the cysts do not decrease in size. Further study of a larger number of cases is needed to establish an optimal treatment protocol for these tumours.

Adrenal cysts (AC) are rare, particularly in the newborn period. Almost all ACs in newborns were diagnosed on exploratory laparotomy or at autopsy. We report a newborn with an AC that was diagnosed after an exploratory laparotomy due to its rare presentation. This case shows that ACs should be considered in the differential diagnosis of an abdominal mass in the newborn period.

We report a case of chronic expanding hematoma in the adrenal gland. Mixed signal intensity in a mosaic pattern was seen on T2-weighted images. A focal area suggesting a subacute hematoma was also noted. The dynamic CT scan showed heterogeneous contrast enhancement in the arterial phase and heterogeneous spread of the enhanced area within the tumor. Awareness of this finding may prevent the misdiagnosis of a tumor as a neoplastic intratumoral hemorrhage.

Objectives: We report six cases of adrenal cyst, a rare pathologic entity which poses diagnostic problems and therapeutic controversies., Methods: We report six cases of adrenal cysts in 4 female and 2 male patients ages 26 to 68 years. All cases underwent surgical treatment., Results: Pathologic studies showed that all were adrenal cysts without malignancy in any case., Conclusions: Adrenal cysts are a rare disease of which correct diagnosis is difficult so that many times patients undergo surgery without a proper preoperative diagnosis. There are controversies about the right management of adrenal cysts.

This case report describes a 53-year old man with Cushing's syndrome due to Adrenocorticotropic Hormone (ACTH) independent bilateral adrenocortical macronodular hyperplasia (AIMAH). His plasma cortisol showed no diurnal rhythm and was unsuppressable by high-dose dexamethasone. Plasma ACTH was undetectable and did not respond to corticotrophin-releasing hormone. The aetiology of this rare disease remains uncertain. The treatment is based on bilateral adrenalectomy.

The aim of this study was to analyze the imaging spectrum of adrenal pseudocysts on CT. The CT images of seven patients with pathologic diagnosis of adrenal pseudocysts in our hospital were reviewed for the size, cystic part, solid part, septum, calcification, acute hematoma, and layering appearance. The presence or absence of contrast enhancement of solid parts in each lesion was also assessed if possible. Of the seven adrenal pseudocysts, there were three pure cystic, three mixed cystic and solid, and one solid lesions on CT. Two of the three cystic pseudocysts were septated with calcifications. Layering appearance was present in two mixed lesions. There were central calcifications and acute hematomas in one solid mass. In our study, there was no contrast enhancement of the solid parts of adrenal pseudocysts. The CT appearances of adrenal pseudocysts may range from cystic, mixed, to solid masses. The presence of solid parts of adrenal pseudocysts on CT mimics those of adrenal neoplasms; however, no contrast enhancement of the solid part in the lesion may help in the diagnosis of adrenal pseudocysts and their differentiation from adrenal neoplasms.

The serotonin4 (5-HT4) receptor agonists cisapride and/or metoclopramide have been shown to stimulate cortisol secretion in some patients with ACTH-independent bilateral macronodular adrenal hyperplasias (AIMAH) causing Cushing's syndrome. In the present study, we have investigated quantitatively and qualitatively the expression of the 5-HT4 receptor in both normal adrenal cortex and tissues removed from six patients (P1-P6) with cisapride-responsive AIMAH and Cushing's syndrome. Real-time quantitative PCR assay revealed that the 5-HT4 receptor was overexpressed in four of the six hyperplasias studied when compared with normal adrenal cortex. In these tissues, 5-HT4 receptor mRNA expression was 3 to 16 times higher than in normal glands, likely explaining the abnormal in vivo cortisol response to cisapride. Characterization of 5-HT4 receptor splice variants by RT-PCR in both hyperplastic and normal adrenals showed that the variants present in the two hyperplasias that did not overexpress the 5-HT4 receptor, i.e. P2 and P5, could also be detected in the normal adrenal tissue. In addition, sequencing of the full-length cDNAs encoding 5-HT4 receptors in hyperplasias P2 and P5 did not reveal any mutation. Taken together, our results show an overexpression of the 5-HT4 receptor in cisapride-responsive AIMAH. However, in two cases, the level of expression of the receptor in the hyperplastic adrenal cortex was similar to that of normal adrenal gland. The enhanced sensitivity of these two tissues to 5-HT4 receptor agonists was not due to ectopic expression of 5-HT4 receptor isoforms or to the occurrence of somatic gain-of-function mutation of the receptor.

By performing a comprehensive MR imaging examination, it is not only possible accurately to characterize and stage cystic and solid lesions of the kidney, but also to provide important preoperative information to the surgeon. In addition, MR imaging can characterize many adrenal lesions and frequently can obviate the need to obtain biopsies. The continued development and growth of MR technology combined with the current trend toward minimally invasive surgery will expand the role of MR imaging in the future.

Bilateral massive adrenal swelling (BAS) on computed tomography (CT) scan with no enhancement after injection of intravenous contrast media has been observed in two completely different clinical settings. On the one hand, BAS is the result of ischemic necrosis and subsequent hemorrhagic infarction in patients with sepsis and hypotension in critically ill situations. On the other hand, BAS is the result of microvascular thrombosis, ischemia, and secondary inflammatory swelling in the setting of thrombotic conditions such as antiphospholipid syndrome (APS), heparin-induced thrombocytopenia and thrombosis (HITT), and thrombocythemia. In this study we present evidence that the etiology of unilateral or BAS in reported cases of essential thrombocythemia (ET) and polycythemia vera (PV) is similar to the etiology of microvascular circulation disturbances in thrombocythemia caused by platelet-mediated inflammation and thrombosis in the peripheral, cerebral, and/or coronary endarterial microvascular circulation.

Pathological changes usually occur independently in the adrenal cortex and medulla because of their distinct embryonic origins, and changes involving both the cortex and medulla are rare. We report 4 cases of corticomedullary mixed pathological changes adrenal glands. CT scanning of the adrenal glands showed unilateral abnormalities in all the 4 cases, 3 of which were diagnosed as aldosteronism and the other pheochromocytoma before surgery. Unilateral adrenalectomy was performed in the 4 patients 3 being cured and discharged. The other 1 had recurrence 18 months postoperatively with suspected pathological changes on the other side. Subsequent pathological examination confirmed the suspicion in both the cortex and medulla of the other adrenal gland. In cases with clinical presentations as simultaneous onset of aldosteronism and catecholamine responses, pathological changes in both the cortex and medulla of the adrenal glands should be considered. Perioperative management of such cases should be the same as that in cases of catecholamine responses, and the diagnosis relies on histopathological examination.