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Cystic lesions of the adrenal gland are uncommon, often presenting with nonspecific clinical and radiologic findings, and are thus underrecognized. They are occasionally associated with malignant neoplasms, which can greatly mimic benign lesions and carry detrimental clinical consequences if misdiagnosed. Here we present our 20-year experience (1992-2012) with these lesions at an academic medical center. Among more than 4500 adrenal gland specimens, 31 cases of adrenal lesions with a predominant cystic component were identified in 30 patients with an age range of 34 to 86 years (median, 55.5 years) and a male/female ratio of 13:17. Macroscopic descriptions, available histologic and immunostain slides, and available radiologic records were reviewed for all included cases. Radiologic studies and gross examination correlated well, and hemorrhage (26 cases; 84%) and encapsulation (25 cases; 81%) appeared to be nonspecific radiologic/gross features shared across histologic subtypes. Microscopic review identified 12 cases (39%) of pseudocysts, 2 cases (6%) of endothelium-derived cysts, and 17 cases (55%) of epithelium-derived cysts. Among these 31 cystic adrenal lesions, 2 cases (6%) were malignant neoplasms (1 epithelioid angiosarcoma, 1 adrenocortical carcinoma). Radiologic impression and histopathologic diagnosis were concordant in 11 (73%) of the 15 cases for which radiologic records were available. This study represents the second largest case series to date on cystic adrenal lesions and presents a comprehensive review on their demographic, clinical, radiologic, and gross and microscopic pathologic features, as well as their differential diagnoses., (Published by Elsevier Inc.)

Context: Primary adrenal insufficiency due to bilateral adrenal hemorrhage-adrenal infarction is a rare and life-threatening manifestation of the antiphospholipid syndrome (APLS). Data on the long-term outcome are scarce., Objective: The aims of the present study were to analyze the long-term outcome related to APLS per se and to characterize the course of adrenal involvement., Design: We conducted a retrospective study of patients with bilateral adrenal hemorrhage-adrenal infarction secondary to APLS seen in the Department of Internal Medicine of Pitié-Salpêtrière Hospital in Paris (France) between January 1990 and July 2010., Results: Three patients died during the acute phase related to APLS manifestations. Sixteen patients (7 males; 9 females) were followed up during a median period of 3.5 years (range 0.3-28.1 years). Three episodes of recurrent thrombosis were noted. One patient died from cerebral hemorrhage 3 months after the onset of adrenal insufficiency. Repeated Synacthen tests showed complete absence of response in 8 of the 10 patients assessed; cortisol and aldosterone increased appropriately in one patient and to some extent in another one. Dehydroepiandrosterone levels and 24-hour urinary epinephrine levels remained abnormally low in all evaluated patients. Adrenal imaging performed more than 1 year after the initial event revealed completely atrophic glands in 9 of 11 patients., Conclusions: This particular subset of APLS patients who survive the acute phase has a rather favorable long-term outcome. Although adrenal dysfunction is generally irreversible, adrenocortical function may, at least partially, recover in rare cases. In this view, measurement of early morning cortisol during follow-up is indicated to detect these patients.

Hypoadrenocorticism or Addison's disease (AD) is a functional disorder in which insufficient mineralocorticoid and glucocorticoid hormones are produced by the adrenal cortex. Human AD is usually attributed to lymphoplasmacytic adrenalitis with autoimmune destruction of the adrenal cortex. Lymphoplasmacytic adrenalitis is also reported in some descriptions of canine AD; however, the histological aspects of adrenalitis or adrenocortical atrophy have not been well characterized because microscopical examination is not required for diagnosis of AD. In this study, sections of adrenal glands from 33 dogs with adrenalitis were compared with those of 37 dogs without adrenal lesions. The affected dogs were classified clinically as having AD (n = 3), being suspected of having AD (n = 17), not having AD (n = 11) or were unclassified (n = 2). The adrenal inflammation was lymphoplasmacytic in 17 dogs, lymphocytic in four, lymphohistiocytic in one, granulomatous in three and neutrophilic in eight cases. Adrenal glands from control dogs lacked leucocyte infiltration and had a cortical to medullary area ratio of 1.1-7.2. All three dogs with AD, 8/17 dogs with suspected AD and 1/11 dogs without AD had a cortical to medullary area ratio <1.1. Because the area ratio was correlated (r = 0.94) with a linear cortical to medullary thickness ratio, a thickness ratio <1.1 could also indicate severe adrenocortical atrophy. Severe adrenocortical atrophy was associated typically with lymphoplasmacytic infiltration and nearly complete loss of cortical cells; however, the zona glomerulosa was partially spared in three dogs with lymphoplasmacytic adrenalitis and severe cortical atrophy. In contrast, non-lymphoid inflammation was generally part of systemic disease, multifocal and was unaccompanied by severe adrenocortical atrophy., (Copyright © 2012 Elsevier Ltd. All rights reserved.)

Single-port endo-laparoscopic surgery has gained support in the surgical community because it is perceived to offer a better postoperative outcome as it requires only a single incision. We write this prospective observational study to ascertain the feasibility and safety of this technique in patients otherwise requiring two operations. Five patients who underwent double procedures with a single-port device were reviewed: Case 1, a transabdominal preperitoneal hernia repair and gastric wedge resection; Case 2, cholecystectomy and diaphragmatic hernia repair; Case 3, oophorectomy and incisional hernia repair; Case 4, anterior resection of the rectum and hepatic segmentectomy; and Case 5, left adrenalectomy and cholecystectomy. Patient demographics, type of port used, operative time, complications and incision length were collected. Mean operative time for the cases ranged from 100 to 315 min. Incision length for the single-port device was 2 cm. In Case 2, an additional 5-mm port was used and an intraoperative complication involving a laceration of the liver occurred during the suturing of the gallbladder fundus. An additional 8-cm lower abdominal incision (Pfannenstiel) was required in Case 4 to complete the colonic anastomosis and for specimen retrieval. Single-port endo-laparoscopic surgery is a feasible and safe technique for approaching double procedures. It drastically reduces the number of scars that a double procedure creates, and if difficulty arises, another port can always be added to ease the operation. It can also potentially reduce the number of admissions and anesthesia that a patient undergoes., (© 2013 Japan Society for Endoscopic Surgery, Asia Endosurgery Task Force and Wiley Publishing Asia Pty Ltd.)

The first part of the study aimed to describe prospectively the ultrasonographic features of the adrenal glands in 94 healthy cats and 51 chronically sick cats. It confirmed the feasibility of ultrasonography of adrenal glands in healthy and chronically sick cats, which were not statistically different. The typical hypoechoic appearance of the gland surrounded by hyperechoic fat made it recognisable. A sagittal plane of the gland, not in line with the aorta, may be necessary to obtain the largest adrenal measurements. The reference intervals of adrenal measurements were inferred from the values obtained in the healthy and chronically sick cats (mean ± 0.96 SD): adrenal length was 8.9-12.5 mm; cranial height was 3.0-4.8 mm; caudal height was 3.0-4.5 mm. The second part of the study consisted of a retrospective analysis of the ultrasonographic examination of the adrenal glands in cats with adrenal diseases (six had hyperaldosteronism and four had pituitary-dependent hyperadrenocorticism) and a descriptive comparison with the reference features obtained in the control groups from the prospective study. Cats with hyperaldosteronism presented with unilateral severely enlarged adrenal glands. However, a normal contralateral gland did not preclude a contralateral infiltration in benign or malignant adrenal neoplasms. The ultrasonographic appearance of the adrenal glands could not differentiate benign and malignant lesions. The ultrasonographic appearance of pituitary-dependent hyperadrenocorticism was mainly a symmetrical adrenal enlargement; however, a substantial number of cases were within the reference intervals of adrenal size.

Pyogenic granuloma (PG) is a tumor-like lesion that typically arises on human skin. Intravenous pyogenic granuloma (IVPG) is the vascular counterpart, mostly observed in the venous structures of the neck and upper extremities. Chronic irritation of the skin, traumatic injury, and hormonal alterations seem to be implicated in the pathogenesis of PG. The incidence of PG, and IVPG, is very low in the reported scientific literature, and this underlines the need for understanding unresolved questions concerning the uncommon presentation, and correct diagnosis before surgical intervention. This report describes a case of IVPG diagnosed in a 55-year-old female that presented for observation of chronic abdominal pain associated with nausea and anorexia. A well-defined mass located in the right adrenal gland was documented by ultrasonography and finally confirmed by contrast enhanced CT of the abdomen. There were no radiological signs of liver, kidney, or vascular infiltration. The 35 × 22 mm adrenal gland lesion, suspected to be an adrenal gland tumor, was resected using a minimally invasive approach. Laparoscopic right adrenalectomy was successfully performed. The histology documented the typical morphological features of IVPG in the context of a normal right adrenal gland. This report describes and discusses the unusual presentation of intraabdominal IVPG located in the adrenal gland region together with a review of the current literature.

Aims: To elucidate the relationship between the rare phenomenon of adrenal cysts and adreno-hepatic fusion (AHF)-related intra-adrenal bile ductules., Methods and Results: We examined 673 right and 662 left postmortem adrenal glands. AHF was defined as an adhesion between the liver and an adrenal with closely intermingled parenchymal cells or partial absence of the capsule dividing the two organs. AHF was found in seven (1.0%) right adrenals. AHF-related intra-adrenal bile ductules were observed in four (0.6%) adrenals, and were accompanied by aberrant hepatocytes (two adrenals), microcystic changes (two adrenals) and, in one case, a 15-mm-sized cyst. The cyst-lining cells, which focally resembled the cells of the neighbouring intra-adrenal bile ductules, were positive for cytokeratin 7 (CK7), CK19 and epithelial membrane antigen, and negative for CK20, vimentin, CD34 and calretinin. Identical findings were made in bile ductules located within fused adrenals and livers. CD10 staining was weaker or absent in the microcystic or cystic bile ductules found within adrenals., Conclusions: The 15-mm cyst we describe may have been an adrenal epithelial cyst derived from AHF-related intra-adrenal bile ductules, a possible pathogenesis for the rare phenomenon of right adrenal epithelial cysts., (© 2012 Blackwell Publishing Ltd.)

The adrenal gland, while small in size, provides a major punch to human metabolism. The interplay between the adrenal cortex hormones aldosterone and cortisol provides needed regulation to human metabolism. Aldosterone regulates the body sodium content affecting blood pressure thru fluid-volume regulation by the kidney. Cortisol, also from the adrenal cortex, contributes to regulation of glucose and protein metabolism. Diseases like addison's disease and Cushing's syndrome that affect the normal levels of these hormones can lead to serious pathologies that need to be detected thru clinical laboratory testing. The inner core of the adrenal gland, called the medulla, houses the catecholamine epinephrine, a fast acting neuropeptide hormone that can influence body action and energy levels quickly. The pheochromocytomas pathology of the adrenal medulla adversely affects the medulla hormones and needs to be recognized by clinical laboratory testing. The overview of the adrenal gland and its potential pathologies needs to be looked at anew in relation to post-traumatic stress disorder to find any linkage that may aid in the treatment and cure of our affected military soldiers. This interrelationship between cortisol and epinephrine in PTSD should be closely evaluated to determine if the suspected linkages are significant.

Inflammation in the course of systemic inflammatory response syndrome (SIRS) or sepsis often results in dysregulation of the hypothalamic-pituitary-adrenal axis; however, the underlying mechanisms are not well understood. The adrenal gland is highly vascularized; thus, we hypothesized that endothelial dysfunction may actively participate in inflammation-related adrenal insufficiency. To address this hypothesis, we used the properties of developmental endothelial locus-1 (Del-1), which is an endothelial-derived anti-inflammatory factor that antagonizes integrin-dependent leukocyte adhesion. Here we identified that Del-1 is expressed in the adrenal gland and that its expression was down-regulated upon SIRS induction by systemic lipopolysaccharide administration. Furthermore, we observed increased leukocyte accumulation, inflammation, and higher apoptosis in the adrenal glands of Del-1-deficient mice as compared with wild-type mice. Strikingly, Del-1 deficiency was also associated with reduced corticosterone and ACTH levels 24 hours after lipopolysaccharide administration. Together, these data suggest that Del-1 may act as a gatekeeper of adrenal gland inflammation and may regulate the integrity of the hypothalamic-pituitary-adrenal axis stress response, thereby modulating adrenal (dys)function in the course of SIRS.

Purpose: The condition hereditary leiomyomatosis and renal cell carcinoma is characterized by cutaneous leiomyomas, uterine fibroids and aggressive papillary renal cell carcinoma. A number of our patients with hereditary leiomyomatosis and renal cell carcinoma had atypical adrenal nodules, which were further evaluated to determine whether these nodules were associated with hereditary leiomyomatosis and renal cell carcinoma., Materials and Methods: Patients with hereditary leiomyomatosis and renal cell carcinoma underwent a comprehensive clinical and genetic evaluation. We reviewed the clinical presentation, anatomical and functional imaging, endocrine evaluation, pathological examination and germline mutation testing results., Results: Of 255 patients with hereditary leiomyomatosis and renal cell carcinoma 20 (7.8%) had primary adrenal lesions, including 4 with bilateral adrenal lesions and 4 with multiple nodules. Two patients had adrenocorticotropic hormone independent hypercortisolism. A total of 27 adrenal lesions were evaluated. The imaging characteristics of 5 of these lesions (18.5%) were not consistent with adenoma by noncontrast computerized tomography criteria. Positron emission tomography was positive in 7 of 10 cases (70%). A total of 12 nodules were surgically resected from 10 adrenal glands. Pathological examination revealed macronodular adrenal hyperplasia in all specimens., Conclusions: Unilateral and bilateral adrenal nodular hyperplasia was detected in a subset of patients with hereditary leiomyomatosis and renal cell carcinoma. A functional endocrine evaluation is recommended when an adrenal lesion is discovered. Imaging frequently reveals lesions that are not typical of adenomas and positron emission tomography may be positive. To date no patient has had adrenal malignancy, and active surveillance of hereditary leiomyomatosis and renal cell carcinoma adrenal nodules appears justified., (Copyright © 2013 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Background: Because of controversy in the management of nonfunctional adrenal masses <6 cm with lipid-poor imaging characteristics, the study was conducted to compare the costs of observation versus immediate laparoscopic adrenalectomy., Methods: A total of 370 patients who were evaluated for incidental adrenal masses between January 1999 and December 2007 were identified, and 32 (8.7%) patients had lesions with imaging characteristics that were inconsistent with a benign adenoma (ie, atypical appearing). Sixteen patients underwent immediate surgery and 16 had observation with serial imaging and biochemical studies. The associated total costs were subjected to intention-to-treat analysis., Results: In the observation cohort, 7 patients converted and underwent adrenalectomy after a mean of 13.1 months. Initially, costs of immediate surgery exceeded those of observation ($12,015.72 vs $11,601.18, P = .10). After projecting costs of annual surveillance, a cost advantage for immediate surgery was demonstrated after 9 years (P = .02)., Conclusions: In patients with <6 cm atypical-appearing adrenal lesions, the costs of surgery and of observation are initially equal. After 9 years, the costs of surveillance exceed that of initial laparoscopic adrenalectomy., (Copyright © 2012 Elsevier Inc. All rights reserved.)

The article reviews current magnetic resonance imaging (MRI) techniques and illustrates the MRI features of the commonly encountered lesions of the adrenal gland. MR may not always be able to characterize an adrenal mass. In these instances, reviewing the patient's clinical history and prior imaging can usually differentiate benign from malignant lesions, even if you cannot establishing an exact tissue diagnosis. The reader is referred to other reviews of adrenal imaging that emphasizes the use of CT and imaging-management algorithms that are beyond the purview of this focused review (1-6)., (Copyright © 2012 Wiley Periodicals, Inc.)

Objective: To explore the clinical characteristics of Wolman disease and diagnostic methods using enzymatic and molecular analysis., Method: Lysosomal acid lipase activity was measured using 4-methylumbelliferyl oleate in the leukocytes of an infant suspected of Wolman disease and LIPA gene mutational analysis was performed by PCR and direct sequencing in the proband and his parents. After the diagnosis was confirmed, the clinical, biochemical, radiological and histopathological findings in this case of Wolman disease were retrospectively reviewed., Result: The sixteen-day-old boy was failing to thrive with progressive vomiting, abdominal distention and hepatosplenomegaly. Abdominal X-ray revealed adrenal calcifications which were confirmed on abdominal CT scan. Xanthomatosis were observed on enlarged liver, spleen and lymph nodes during abdominal surgery. Liver and lymph node biopsy showed foamy histiocytes. The lysosomal acid lipase activity in leukocytes was 3.5 nmol/(mg·h) [control 35.5 - 105.8 nmol/(mg·h)]. Serum chitotriosidase activity was 315.8 nmol/(ml·h) [control 0 - 53 nmol/(ml·h)]. The patient was homozygote for a novel insert mutation allele c.318 ins T, p. Phe106fsX4 in exon 4 on LIPA gene. His both parents were carriers of the mutation., Conclusion: The clinical features of Wolman disease include early onset of vomiting, abdominal distention, growth failure, hepatosplenomegaly and bilateral adrenal calcification after birth. A plain abdominal X-ray film should be taken to check for the typical pattern of adrenal calcification in suspected cases of Wolman disease. The enzymatic and molecular analyses of lysosomal acid lipase can confirm the diagnosis of Wolman disease.

A 56-year-old woman presented with an incidental adrenal adenoma and physical examination findings that included moderate obesity, a slight cervicothoracic fat pad ("buffalo hump"), increased supraclavicular fat pads, and white abdominal striae. Biochemical workup revealed elevated levels of 24-hour urinary free cortisol but normal serum morning cortisol and suppressed levels of corticotropin, suggestive of adrenal-dependent Cushing syndrome. The resected adrenal gland revealed macronodular cortical hyperplasia with a dominant nodule. Other findings included an absent cortisol response to corticotropin stimulation, presence of serum anti-21-hydroxylase antibodies, and mononuclear cell infiltration--consistent with adrenalitis. The findings represent, to the authors' knowledge, the first known case of a patient with coexistent functional cortisol-secreting macronodular adrenal tumor resulting in Cushing syndrome and immune-mediated adrenalitis resulting in Addison disease.

Isolated adrenal tuberculosis is rare, and represents between 1-2% of the etiologies of adrenal masses called incidentalomas. A 32-year-old woman, without notable medical history, was hospitalized for pain in the left hypochondrium, lasting for two months in a context of apyrexia and weight loss amounted to 5 kg. Clinical examination was normal, but abdominopelvic CT objectified bilateral adrenal hypertrophy predominantly left with bilateral linear calcifications. The chest radiograph was normal, adrenal hormones were normal. The research of BK in sputum and urine were negative on direct examination and culture. The tuberculin was 12 mm and HIV status was negative. A left adrenal biopsy was done and histopathological study of tuberculous lesions was found confirming caseofolliculaire adrenal tuberculosis. The patient has been treated with antibacillaire with favorable evolution. In light of this observation, the authors make the point on this rare disease., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

Epithelial-lined or true cysts of the adrenal gland are exceedingly rare accounting for only 9% of all adrenal cysts. A 55-year-old woman with a medical history for cholecystectomy and liver hydatidosis presented with an 8-month history of abdominal pain in the upper left quadrant. Physical examination was unremarkable and laboratory tests were within normal range. Computed tomography displayed a homogeneous and finely calcified cystic mass of the left adrenal gland measuring 12 x 10 cm. A hydatid cyst of the adrenal gland was suspected preoperatively and the patient underwent uneventful excision of the cyst. Histological examination of the surgical specimen revealed that the cyst wall was lined by cuboidal to flattened cells with bland cytologic features. Immuohistochemically, the cyst lining expressed intensely cytokeratin but was negative for CD 34 and calretinin. The final pathological diagnosis was epithelial adrenal cyst.

Unlabelled: Cysts are a rare pathology of adrenal glands. As the development of new diagnostic techniques takes place, the occurrence of adrenal cystic lesions has been rapidly increasing. The majority of them are solid adrenal lesions, but localized fluid collections are also more frequently diagnosed. In case of solid adrenal lesions, there are straight indications for surgery, but on the other hand there are no clear guidelines and recommendations in case of adrenal cysts. The aim of the study was to analyze surgical methods and evaluate treatment effects in patients who were qualified for laparoscopic adrenalectomy due to adrenal cystic lesions. METARIAL AND METHODS: Identical criteria were used to qualify patients with solid and cystic lesions of the adrenal gland for surgery. Out of the whole number of 345 patients who underwent laparoscopic surgery for adrenal tumors, 28 had adrenal cysts. 16 of them (57%) were women and 12 (43%) men. The average age of the studied group was 46.4 years (25-62 years). The average cyst diameter in CT was 5.32 cm (1.1-10 cm). Most of the lesions were hormonally inactive (22 patients), but in 6 cases increased level of adrenal hormones was observed., Results: Pathological analysis revealed 4 (14%) pheochromocytomas and 2 (7%) dermoid cysts. In case of 22 (79%) patients, the postoperative material was profiled by pathologists as insignificant according to potential neoplasmatic transformation risk: 5 (17.5%) - endothelial vascular cysts, 3 (11%) endothelial lymphatic cysts, 7 (25.5%) pseudocysts, 3 (11%) simple cysts, 2 (7%) bronchogenic cysts, 1 (3.5%) - cortical adenoma and 1 (3.5%) cyst was of myelolipoma type., Conclusions: Based on the performed research and previous experience in treating patients with adrenal lesions we can conclude that application of the same evaluating algorithm for both cystic and solid lesions is valid.

Purpose: It is difficult to definitively rule out or establish malignancy pre-operatively in patients with adrenal lesions referred for adrenal surgery. The aim of this study was to identify risk factors for a malignant diagnosis in patients treated with adrenalectomy., Methods: Consecutive adrenalectomies at the Department of Surgery, Lund University Hospital between 2000 and 2009 were identified. Pre-operative clinical, radiological and biochemical data, as well as details on the operation and histology, were retrieved from patients' medical records., Results: There were 175 adrenal operations in 172 patients (98 female and 77 male). There were 28 malignancies. Malignant tumours were larger than in benign cases (p = 0.003), had radiological signs of malignancy more often (p = 0.001) and the patients were more likely to have a history of malignancy (p = 0.001). There were no differences regarding age, gender, body mass index or mode of detection (incidental vs. clinical) between patients with benign and malignant diagnoses. A unilateral uptake on fluorine-18 deoxyglucose positron emission tomography was more frequent in malignant cases than in benign; however, this association did not reach statistical significance., Conclusions: Size of adrenal lesion, suspicious radiological findings and history of malignancy were factors associated with malignancy.

Introduction: Laparoscopic adrenalectomy has become the standard of care for the surgical treatment of benign adrenal pathology. We present the following case series documenting our experience in refinement of this approach., Paients and Methods: Analysis of patient records identified those in whom laparoscopic adrenalectomy was performed from January 1997 through February 2010. Study variables included indications, operative time, blood loss, length of hospital stay, histopathological evaluation, and complications., Results: Laparoscopic adrenalectomy was performed in 306 patients using the transperitoneal lateral approach. No major operative complications were noted, and postoperative complications included a pulmonary embolism and 2 cases of pneumonia. Conversion to the open approach was necessitated in two cases. The median operative time was 95±29 minutes (range, 45-145 minutes). Estimated blood loss was 60 mL (range, 30-150 mL). The mean size of the removed gland was 5.9±1.6 cm (range, 3-13 cm). The mean size of the tumor was 5±2 cm (range, 0.5-12 cm). The median hospitalization was 4±3.7 days (range, 2-22 days). Adrenal pathology included adenoma (n=164), pheochromocytoma (n=79), hyperplasia (n=35), metastatic carcinoma (n=22), cyst (n=9), myelolipoma (n=9), hemangioma (n=3), ganglioneuroma (n=3), and melanoma (n=2)., Conclusion: Laparoscopic adrenalectomy is a safe and feasible approach to adrenal pathology, providing the patients with all the benefits of minimally invasive surgery.

Objective: The aim of this article is to define the relationship between body mass index (BMI) and outcomes of laparoscopic adrenalectomy., Method: A total of 172 patients were eligible for inclusion in the study. The patients were divided into 3 groups: group I, normal weight; group II, overweight; and group III, obesity. Perioperative outcomes were compared between the groups., Results: The median operative time was 72, 75, and 90 minutes in groups I, II, and III, respectively. The median blood loss was <50 mL in all groups. There were no intraoperative blood transfusions. There was no statistical difference in the rate of intraoperative incidences and postoperative complications. Moderately increased operative time was the only perioperative parameter that statistically differed from nonobese patients. The regression analysis found significant but weak correlation between BMI and operative time. The BMI did not correlate with other surgical outcomes., Conclusions: Laparoscopic adrenal surgery for obese patients can be done as safely as for nonobese patients.

The ability to measure steroid hormone concentrations in blood and urine specimens is central to the diagnosis and proper treatment of adrenal diseases. The traditional approach has been to assay each steroid hormone, precursor, or metabolite using individual aliquots of serum, each with a separate immunoassay. For complex diseases, such as congenital adrenal hyperplasia and adrenocortical cancer, in which the assay of several steroids is essential for management, this approach is time consuming and costly, in addition to using large amounts of serum. Gas chromatography/mass spectrometry profiling of steroid metabolites in urine has been employed for many years but only in a small number of specialized laboratories and suffers from slow throughput. The advent of commercial high-performance liquid chromatography instruments coupled to tandem mass spectrometers offers the potential for medium- to high-throughput profiling of serum steroids using small quantities of sample. Here, we review the physical principles of mass spectrometry, the instrumentation used for these techniques, the terminology used in this field and applications to steroid analysis.

Hypertension is a common systemic disorder affecting the cardiovascular system and kidneys with important morbidity and mortality. However, hypertension is often thought of by radiologists as an "invisible" disease. The aim of this article is to first review hypertension first in terms of its diagnosis, epidemiology and clinical importance. Second, those causes of secondary hypertension that may be diagnosed with MRI techniques are described along with the effects of hypertension on the cardiovascular system and kidneys that are demonstrable with MRI with particular emphasis on renovascular disease. Lastly the use of integrated MRI protocols to evaluate the hypertensive patient and areas for future research are considered., (Copyright © 2011 Wiley Periodicals, Inc.)

Objective: To analyze the change in the incidence of pheochromocytomas in adrenal medulla or extra-adrenal and multiple endocrine neoplasm type 2 (MEN2), to summarize the clinical characteristics of benign, potentially malignant and malignant pheochromocytomas and to investigate the correlation between clinical manifestations and pathological changes., Methods: Statistic analysis was performed to detect the incidence, constituent ratio, mean diagnostic age, sex proportion and correlation between clinical manifestions and pathologic changes in pheochromocytomas in adrenal medulla or extra-adrenal gland and MEN2 from 1993 to 2008 in the Department of Pathology, the General Hospital of Tianjin Medical University with Runs test, ANOVA, t test and chi-square test., Results: The total number of biopsies within the 16 years was 167 702 cases (average 10 481 cases per year). The numbers (detectable rate) of total adrenal diseases, pheochromocytomas in adrenal medulla and extra-adrenal glands were 910 (0.54%), 139 (0.08%), and 42 (0.03%) cases, respectively. The numbers (constituent ratio) of benign, potentially malignant and malignant of pheochromocytomas in adrenal medulla were 102 cases (73.4%), 29 cases (20.9%) and 8 cases (5.7%), respectively; in the 102 cases of benign tumors, patients with MEN2 were 8 (7.8%); the three groups of the tumors in extra-adrenal sites were 18 (42.8%) cases, 12 (28.6%) cases and 12 (28.6%) cases. There were no changes in the detectable rate and constituent ratio of adrenal diseases, benign, potential malignant and malignant pheochromocytomas in adrenal medulla or extra-adrenal glands and patients with MEN2 during the past 16 years (P > 0.05), but there was a tendency that malignant transformation was gradually increased with age, which was more commonly found in male patients than females. The mean age at diagnosis of patients with benign and potentially malignant pheochromocytomas was 42.7 years (ranged from 10 - 74 years), and 40.1 years (13 - 66 years), respectively, which were younger than patients with malignant pheochromocytomas (51.6 years, P < 0.05); the mean age of patients with benign and potentially malignant pheochromocytomas in extra-adrenal sites was 43.1 years (ranged from 20 - 75 years) and 45.2 years (28 - 65 years) that were older than those with malignant (37.8 years, ranged from 14 - 58 years, P < 0.05). It was spectacular that patients with malignant pheochromocytoma in adrenal medulla (51.6 years) were older than that in extra-adrenal sites (37.8 years); all the patients with MEN2 were female benign pheochromocytoma in adrenal medulla, whose age (38.9 years) was younger than that of benign lesions (42.7 years, P < 0.05), in which thyroid medullary carcinoma appeared early than pheochromocytomas in adrenal medulla. The detectable rate of hypertension in patients with malignant pheochromocytomas in adrenal medulla and in extra-adrenal sites were less than that in benign and potentially malignant ones (P < 0.05). The bilateral lesions more commonly found in malignant pheochromocytoma (25.0%) than benign (15.7%) and potentially malignant pheochromocytomas (6.9%) only in adrenal medulla. Relapse rates in both adrenal and extra-adrenal tumors were rising from benign (11.8%, 0), potentially malignant (13.8%, 25.0%), to malignant (33.3%, 37.5%) groups; the average diameter of pheochromocytomas in both adrenal and extra-adrenal sites was increasing from benign (4.2 cm, 4.0 cm), potentially malignant (5.3 cm, 5.6 cm) to malignant (7.3 cm, 6.9 cm) groups (P < 0.05)., Conclusions: The diagnostic criteria of benign, potentially malignant and malignant pheochromocytomas in adrenal medulla and in extra-adrenal sites are well established according to the WHO classification of endocrine tumors (2004). The closer relationship is found between clinical manifestations and pathologic changes. The definite type and nature of pheochromocytomas are mainly rested upon the pathologic examination.

Background: There is limited description in the literature of the initial diagnosis of adrenal histoplasmosis by fine needle aspiration cytology (FNAC)., Objective: To study the aetiology, clinical features and morphological differential diagnosis of adrenal histoplasmosis diagnosed initially by FNAC., Methods: Five cases diagnosed by ultrasound-guided FNAC from 2008 to 2010 were found in the laboratory records and reviewed. Clinical features and cytomorphological features were correlated., Results: All the patients were male and were aged 45-83 years (mean 60 years). Three patients had a history of contact with birds, one was diabetic and all were negative for human immunodeficiency virus (HIV). The commonest symptom was weight loss. On cytological smears, uniform round to oval (about 2-4 μm in diameter) budding yeasts were seen intracellularly (within histiocytes) as well as extracellularly. In three cases, an inflammatory response in the form of epithelioid cell granulomas along with multinucleated giant cells was seen., Conclusions: Adrenal histoplasmosis does occur in immunocompetent persons living in areas not endemic for the disease. Although it is not common, it needs to be considered in the differential diagnosis of bilateral adrenal enlargement. FNAC is a simple, safe technique to establish the initial diagnosis. Correct diagnosis and treatment leads to a favourable outcome., (© 2010 Blackwell Publishing Ltd.)

Adrenal histoplasmosis is an uncommon mycotic disease typically caused by Histoplasma capsulatum. The objective was to determine the clinicopathological findings in adrenal histoplasmosis. Pathological records were searched from the database at the Department of Pathology, Faculty of Medicine Ramathibodi Hospital, Mahidol University from 1993 to 2008 for cases of adrenal histoplasmosis. The keywords were "histoplasmosis" and "adrenal gland". Adrenal histoplasmosis was diagnosed by histopathology and Gomori-Grocott methenamine silver staining. Histoplasma capsulatum was confirmed by tissue culture and/or serology. The authors report seven cases of adrenal histoplasmosis in immunocompetent patients. The mean age at diagnosis was 67 years. All patients presented as chronic fatigue syndrome. The onset of symptoms ranged from one to three months. Addison's disease was found in adrenal histoplasmosis in one case (14.3%). The computed tomography revealed adrenal nodules measuring 1.2 to 7.8 cm in diameter. The histopathology showed granulomatous inflammation with caseous necrosis. Culture of adrenal tissue from two patients revealed Histoplasma capsulatum. Serum Histoplasma antibodies were positive in four cases. A cure was accomplished in 6 out of 7 cases (85.7%). The patients were followed up for 2.5 to 16.5 years.

A 5.5-y-old spayed female ferret (Mustela putorius furo) with a history of adrenal disease, respiratory disease, and chronic obesity was evaluated for progressive lethargy and ataxia, diminished appetite, and possible polyuria and polydipsia. Physical examination revealed obesity, lethargy, tachypnea, dyspnea, a pendulous abdomen, significant weakness and ataxia of the hindlimbs, prolonged skin tenting, and mild tail-tip alopecia. Clinicopathologic analysis revealed severe hyperglycemia, azotemia, an increased anion gap, glucosuria, ketonuria, proteinuria, and hematuria. Abdominal ultrasonography showed hyperechoic hepatomegaly, bilateral adrenomegaly, splenic nodules, mild peritoneal effusion, and thickened and mildly hypoechoic limbs of the pancreas with surrounding hyperechoic mesentery. Fine-needle aspirates of the liver were highly suggestive of hepatic lipidosis. In light of a diagnosis of concurrent diabetic ketoacidosis and pancreatitis, the ferret was treated with fluid therapy, regular and long-acting insulin administration, and pain medication. However, electrolyte derangements, metabolic acidosis, dyspnea, and the clinical appearance of the ferret progressively worsened despite treatment, and euthanasia was elected. Necropsy revealed severe hepatic lipidosis, severe suppurative pancreatitis and vacuolar degeneration of pancreatic islet cells, a pancreatic β islet cell tumor, bilateral adrenal cortical adenomas, and myocardial fibrosis. To our knowledge, this case represents the first report of concurrent diabetes mellitus, pancreatitis, pancreatic β islet cell tumor (insulinoma), and adrenal disease in a domestic ferret. The simultaneous existence of 3 endocrine diseases, pancreatitis, and their associated complications is a unique and clinically challenging situation., (Copyright 2011 by the American Association for Laboratory Animal Science)

Objective: To assess the additional value of adrenal venous sampling (AVS) to diagnose primary aldosteronism sub-types in patients who have a unilateral nodule detected by computed tomography (CT scan) and who should undergo an adrenalectomy., Methods: A retrospective study to assess consecutive patients with primary aldosteronism undergoing an adrenal CT scan and AVS. Criterion for selective cannulation was an equal or higher cortisol level in the adrenal vein compared to the inferior vena cava. An adrenal-vein aldosterone-to-cortisol ratio of at least two times higher than the other side defined lateralization of aldosterone production., Results: Sixty-seven patients (mean age 52 years, 39 men) underwent a CT scan and AVS. In nine patients (13%), cannulation of the right adrenal vein led to a technical failure. Both procedures led to diagnosis of 29 patients with adenoma-producing aldosterone (APA; 50%), 23 bilateral adrenal hyperplasias (40%), and six unilateral adrenal hyperplasias (10%). Of the 45 patients with a nodule detected by CT, subsequent AVS showed bilateral secretion in 16 patients (36%). Compared to the strategy of coupling CT scans with AVS to diagnosis APA, a CT scan alone had an accuracy of 72.4% (P < 0.001). Among patients with a macronodule detected by CT, 13 (37%) had bilateral secretion as assessed by AVS. The patients with a macronodule detected by CT alone had the same risk of a discrepancy as those with a small nodule (P = 0.99)., Conclusion: AVS is essential to diagnose the unilateral hypersecretion of aldosterone, even in patients in whom a unilateral macronodule is detected by CT, to avoid unnecessary surgery.

Glucocorticoid (GC) is an adrenal steroid hormone that controls a variety of physiological processes such as metabolism, immune response, cardiovascular activity, and brain function. In addition to GC induction in response to stress, even in relatively undisturbed states its circulating level is subjected to a robust daily variation with a peak around the onset of the active period of the day. It has long been believed that the synthesis and secretion of GC are primarily regulated by the hypothalamus-pituitary-adrenal (HPA) neuroendocrine axis. However, recent chronobiological research strongly supports the idea that multiple regulatory mechanisms along with the classical HPA neuroendocrine axis underlie the diurnal rhythm of circulating GC. Most notably, recent studies demonstrate that the molecular circadian clockwork is heavily involved in the daily GC rhythm at multiple levels. The daily GC rhythm is implicated in various human diseases accompanied by abnormal GC levels. Patients with such diseases frequently show a blunted GC rhythmicity and, more importantly, circadian rhythm-related symptoms. In this review, we focus on recent advances in the understanding of the circadian regulation of adrenal GC and its implications in human health and disease., (Crown Copyright © 2010. Published by Elsevier B.V. All rights reserved.)

Despite their small size, pathologic condition of the adrenal glands is often far from insignificant. Imagers should therefore be familiar with the principles and techniques that underpin the ability of imaging to characterize most lesions. Ignorance of these techniques fails to deliver the necessary imaging value to referrers and patients alike. This article, outlines the range of possible abnormalities encountered in the adrenal gland, the imaging modalities and specialized techniques used to detect and characterize them, the principles based on which these techniques are used, and finally a working imaging algorithm that can be readily used in daily practice., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Background: Posterior retroperitoneoscopic adrenalectomy (PRA) is an excellent surgical option for adrenal gland removal. The operation requires that surgeons learn a new approach with few similarities to anterior adrenalectomy. This study reports a large series of PRAs incorporated into surgical care using a team-model approach., Study Design: The prospective endocrine surgery database was queried to identify patients who underwent PRA during a recent 4-year period. Demographic, operative, and pathologic data were recorded. The authors' initial experiences with PRA (group 1) are compared with our contemporary experience (group 2)., Results: One hundred and eighteen PRAs were successfully performed (100 unilateral and 9 bilateral). Indications were pheochromocytoma in 21 patients, Cushing's syndrome or Cushing's disease in 22 patients, aldosteronoma in 22 patients, virilizing tumor in 3 patients, isolated metastasis in 28 patients, and nonfunctional mass in 19 patients. Forty-eight percent of patients had undergone earlier abdominal surgery. Forty-eight percent were obese (body mass index [calculated as kg/m(2)] ≥30). No significant differences were found in operative time (110 versus 118 minutes, p = 0.30), tumor size (2.59 versus 2.85 cm, p = 0.44), or body mass index (29.63 versus 29.93, p = 0.82) between groups 1 and 2. Both complications (15.9% versus 7.7%, p = 0.29) and conversion rates (9.5% versus 1.9%, p = 0.19) were lower in group 2, although this was not statistically significant., Conclusions: PRA is a technique safely performed for a variety of adrenal lesions, is ideal for patients who have undergone earlier abdominal surgery, and is feasible in obese patients. Proficiency can be obtained during a short period, leading to low conversion and complication rates. This technique should be incorporated into the armamentarium of the endocrine surgeon. A team approach to learning new surgical techniques is effective., (Copyright © 2011 American College of Surgeons. Published by Elsevier Inc. All rights reserved.)

Adrenal masses are common incidental findings on cross-sectional imaging. Most of these masses are benign, and adenomas are the most common entity. Several imaging studies allow accurate diagnosis of these masses, separating inconsequential benign masses from the lesions that require treatment. This article discusses contemporary adrenal imaging and the optimal algorithm for the workup of incidentally detected adrenal masses., (Copyright © 2011 Elsevier Inc. All rights reserved.)

22 children with tumors or other surgical diseases of the adrenal glands were operated on laparoscopically. Children aged from 5 months to 17 years. Operation lengths varied from 45 to 190 minutes. There were no lethal outcomes, conversions and serious complications of the procedure. Authors consider the laparoscopic adrenalectomy to be the safe and widely appropriate method for the use in juvenile cathegory of patients.

Adrenal cysts are rare and are usually discovered incidentally during diagnostic imaging, surgery, or autopsy. Most cystic lesions of the adrenal gland are nonfunctioning and become symptomatic when complicated by rupture, hemorrhage, or infection. A 40-year-old woman presented with a history of gradual-onset pain in her left flank region at 20 weeks' gestation. Ultrasound showed a 20-cm cystic mass in her left abdominal cavity. Pertinent laboratory tests were within normal limits. The patient underwent exploratory laparotomy, which revealed a 20 × 15-cm left adrenal cyst; thus, we performed left adrenalectomy with complete excision of the cyst. Histological examination confirmed a hemorrhagic adrenal pseudocyst. The patient had an uneventful postoperative course, and subsequent routine obstetric ultrasound examinations showed normal fetal activity and development until the pregnancy terminated with a stillbirth caused by pre-eclampsia at 34 weeks' gestation. To the best of our knowledge, this is only the 12th reported case of adrenal pseudocyst discovered during pregnancy. We analyze the clinicopathologic findings and discuss the possible association of pregnancy, with special reference to etiopathogenesis, presentation, diagnosis, and treatment.

Objective: This study aimed to assess stathmin expression in benign and malignant phaeochromocytomas and paragangliomas., Methods: Tissue specimens from 37 patients with malignant phaeochromocytomas (n = 5), malignant paragangliomas (n = 3), benign phaeochromocytomas (n = 24), and benign paragangliomas (n = 5) were analysed for stathmin expression by western blotting and immunohistochemical staining with polyclonal antibodies. Malignancy was defined by metastases in nonchromaffin tissues., Results: There were higher stathmin expression levels in phaeochromocytomas compared to normal adrenal tissue, by western blotting and reverse-transcriptase polymerase chain reaction, whereas immunohistochemical staining showed stathmin immunoreactivity in the cytoplasm of tumour cells. Malignant neoplasms had significantly higher stathmin expression compared to benign neoplasms by western blotting and immunohistochemical staining., Conclusion: Malignant phaeochromocytomas and paragangliomas have significantly higher levels of stathmin expression compared to benign tumours. The potential value of stathmin in predicting malignancies requires further studies., (Copyright © 2011 Asian Surgical Association. Published by Elsevier B.V. All rights reserved.)

Objective: Aberrant adrenal expression of various hormone receptors has been identified in ACTH-independent macronodular adrenal hyperplasia (AIMAH) causing cortisol hypersecretion regulated by hormones other than ACTH. We aimed to determine aberrant expression of multiple hormone receptors in vivo and in vitro in adrenal tissue of a patient with AIMAH., Design: The design of the study includes clinical case description, and biochemical and immunohistochemical analysis to demonstrate aberrant expression of multiple hormone receptors in AIMAH., Methods: The subject of the study is a male diagnosed with Cushing's syndrome because of AIMAH. Directly after laparoscopic removal of the adrenals, adrenal tissue was incubated with and without test substances (ACTH, forskolin, arginine vasopressin (AVP), desmopressin, epinephrine, norepinephrine, purified human chorionic gonadotropin (hCG), metoclopramide and the combinations of AVP with ACTH, epinephrine and metoclopramide). LH/hCG-receptor (hCG-R) immunohistochemistry and RT-PCR analyses were performed to demonstrate aberrant expression of LH/hCG-R and V(1-3)-AVPR., Results: AIMAH was characterized by in vivo cortisol responsiveness to AVP and in vitro cortisol responses to AVP, hCG, epinephrine, and norepinephrine suggesting aberrant adrenal expression of the receptors for AVP (the V(1-3)-AVPRs), catecholamines (the beta-AR), and LH (the LH/hCG-R). Incubation with combinations of AVP and ACTH and of AVP with epinephrine induced a stronger cortisol response compared with incubation with the individual agents. Moreover, we demonstrated adrenal V(1-3)-AVPR and LH/hCG-R expression., Conclusions: AIMAH tissue may simultaneously express multiple aberrant hormone receptors, and individual ligands may potentiate each other regarding cell proliferation and cortisol production.

We report a 54-year-old man who presented with abdominal pain and severe hyponatremia a few days after a left lower lobectomy for lung cancer. An abdominal computed tomography scan without contrast showed a bilateral adrenal hemorrhagic infarction, mainly on the right side. Serum sodium level was 113 mmol/L, and cortisol level was 0 microg/L. Anticardiolipin-type antibodies (immunoglobulin G isotype) level was 75 GPL/mL (normal value < 10). With hydrocortisone supplementation and curative doses of low-molecular-weight heparin, the patient recovered progressively and was discharged on postoperative day 17. Final diagnosis was bilateral adrenal gland hemorrhagic necrosis leading to adrenal insufficiency, associated with antiphospholipid syndrome. We discuss the mechanism and the role of the operation in the occurrence of this particularly rare and potentially life-threatening complication. Recommendations to prevent thrombosis in surgical patients who have antiphospholipid antibodies are lacking., (Copyright 2010 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

The increasing use of cross-sectional imaging has led to an increase in the incidental discovery of adrenal masses (adrenal incidentalomas). Although most of these lesions are benign, they often present a diagnostic dilemma. Before creating a management plan, the physician should determine if the lesion is benign or malignant and if the lesion is functioning or nonfunctioning. Incidentally discovered adrenal masses usually are benign adenomas; however, myelolipomas, cysts, hemorrhage, pheochromocytomas, metastases, and adrenocortical carcinomas are also possible. Unenhanced computed tomography and chemical shift magnetic resonance imaging can characterize most adenomas because the lesions have high lipid content. Contrast-enhanced computed tomography can further characterize the adenomas because of the washout characteristics with iodinated intravenous contrast media. Fluorodeoxyglucose- positron emission tomography can be helpful in characterizing some lesions, and biopsy is rarely required. This article summarizes the American College of Radiology Appropriateness Criteria for the use of imaging modalities and biopsy to characterize incidentally discovered adrenal masses.

Introduction: Laparoscopic subtotal adrenalectomy (LSA) has emerged as a treatment option in the management of certain adrenal pathologies. After LSA, a satisfactory blood supply to the adrenal remnant is vital to preserve steroid function. The aim of this study was to review the adrenal blood supply with relevance to LSA., Materials and Methods: The adrenal blood supply was examined in three ways: 1) a review of six previous cadaver studies looking at adrenal blood supply; 2) a review of our 164 consecutive laparoscopic adrenalectomies (LAs) (January 1999-January 2009); the details of the main adrenal veins and variants had been prospectively recorded; and 3) dissection of 4 cadaver specimens (macroscopic and histologic examination was performed)., Results: The six cadaver studies showed duplication or triplication of the main adrenal vein with variable frequency. Four of six studies described smaller peripheral veins surrounding the adrenal gland. The arterial supply appeared constant, with each adrenal gland supplied by up to 60 small arteries. In our series of LA, the main adrenal vein was constant. In 6 cases, the main vein was duplicated. Our cadaver dissection showed the large number of small peripheral veins and arteries surrounding the adrenal gland. Histology confirmed that these peripheral veins drained the adrenal gland., Conclusions: After LSA, a nonfunctioning adrenal remnant is unlikely due to an inadequate arterial supply. Division of the main adrenal vein appears safe, but excessive mobilization of the adrenal remnant should be avoided to prevent destruction of the peripheral veins.

Bone morphogenetic proteins (BMPs) are members of the transforming growth factor-beta superfamily of ligands that impact on a multitude of biological processes including cell type specification, differentiation and organogenesis. Furthermore, a large body of evidence points towards important BMP-dependent mechanisms in tumorigenesis. In accordance with their diverse actions, BMPs have been demonstrated to serve as auto-, para- and endocrine modulators also in a number of hormonal systems. In this review, we highlight novel aspects of BMP-dependent regulatory networks that pertain to adrenal physiology and disease, which have been uncovered during recent years. These aspects include the role of BMP-dependent mechanism during adrenal development, modulating effects on catecholamine synthesis and steroidogenesis and dysregulation of BMP signalling in adrenal tumorigenesis. Furthermore, we summarize potential therapeutic approaches that are based on reconstitution of BMP signalling in adrenocortical tumour cells.

Background: EUS-guided FNA of the left and right adrenals has been described, but data are very limited., Objectives: Our primary objective was to determine the impact of the diagnostic utility of EUS-guided FNA of adrenal glands on patient management. Our secondary objective was to determine predictors of malignant adrenal involvement., Study Design: Observational study., Setting: Tertiary referral center., Patients: Patients with enlarged adrenal(s) on abdominal imaging underwent EUS-guided FNA. The left adrenal (n = 54) was sampled via the transgastric approach and the right adrenal (n = 5) via a transduodenal approach., Results: Fifty-nine patients (63% men, median age 65 years) were evaluated. The median adrenal gland size was 25 x 17 mm. Adrenal tissue adequate for interpretation was obtained in all of the patients. EUS-guided FNA confirmed malignancy in 22 (37%) patients. Based on size (> or =30 mm) alone, EUS had an accuracy of 68%. Patients with malignant cytology had higher standard uptake value scores on positron-emission tomography compared with patients with benign adrenal masses (P < .001). Malignant masses were more likely to have an altered adrenal gland shape compared with benign masses (crude odds ratio [OR] 12.0; P < .001). On multivariable analysis, altered adrenal gland shape was a significant predictor of malignancy (adjusted OR 7.94; P = .015), whereas a size of 30 mm or larger (adjusted OR 1.30; P = .774) and hypoechoic nature (adjusted OR 12.05; P = .148) were not. All patients except 2 with malignant cytology were treated with systemic therapy without the need for additional invasive biopsies or surgery. No immediate complications were encountered., Limitations: Lack of surgical criterion standard; 1 experienced endosonographer., Conclusions: EUS-guided FNA of the adrenal glands is a minimally invasive and safe approach that documents or excludes malignant involvement. EUS-guided FNA should be the first next test to evaluate enlarged adrenal glands because it directs therapy and affects patient management., (Copyright 2010 American Society for Gastrointestinal Endoscopy. Published by Mosby, Inc. All rights reserved.)

The diagnosis of primary retroperitoneal cystic tumors is very infrequent in surgical pathology. We report the case of a 51-year-old woman presenting with an incidental left-sided retroperitoneal mass (32 x 24 mm in diameter) suspected of being an adrenal tumor. Intraoperatively the tumor was identified as a cystic lesion filled with mucous secretion and laparoscopically completely resected. The diagnosis was histopathologically confirmed as a bronchogenic cyst. In this article the laparoscopic removal of such a rare benign congenital aberration resulting from an abnormal budding of the tracheobronchial tree is presented.