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The adrenal glands in children can be affected by a variety of benign lesions. The diagnosis of adrenal lesions can be challenging, but assessment of morphologic changes in correlation with the clinical presentation can lead to an accurate diagnosis. These lesions can be classified by their cause: congenital (eg, discoid adrenal gland, horseshoe adrenal gland, and epithelial cysts), vascular and/or traumatic (eg, adrenal hemorrhage), infectious (eg, granulomatous diseases), enzyme deficiency disorders (eg, congenital adrenal hyperplasia [CAH] and Wolman disease), benign neoplasms (eg, pheochromocytomas, ganglioneuromas, adrenal adenomas, and myelolipomas), and adrenal mass mimics (eg, extralobar sequestration and extramedullary hematopoiesis). Multimodality cross-sectional imaging helps to define the origin, extent, and relationship of these lesions to adjacent structures, as well as to guide treatment management. The anatomic and functional imaging modalities used to evaluate pediatric adrenal lesions include ultrasonography, computed tomography (CT), magnetic resonance imaging, and iodine 123 metaiodobenzylguanidine scintigraphy. Identifying the imaging features of nonmalignant adrenal lesions is helpful to distinguish these lesions from malignant adrenal neoplasms. Identifying characteristic imaging findings (eg, enlarged adrenal glands, with cerebriform surface, and stippled echogenicity in CAH; a T2-hyperintense mass with avid contrast enhancement in pheochromocytoma; low CT attenuation [<10 HU] and signal intensity drop on opposed-phase chemical shift images in adenoma; and enhancing suprarenal mass supplied by a systemic feeding artery in extralobar sequestration) can aid in making the correct diagnosis. In addition, clinical features (eg, ambiguous genitalia in CAH and hypertension in pheochromocytoma) can also guide the radiologist toward the correct diagnosis. © RSNA, 2017.

Epithelial- lined (true) cysts are rare lesions and until now the only information we had about their histogenesis was based on the analysis of a few cases. We retrospectively reviewed 8 cases of cysts with a true epithelial lining (confirmed immunohistochemically). The pathological findings and immunohistochemical analysis of the epithelial linings allowed for categorization of the cysts into 3 groups. Five cysts had pure mesothelial lining, which was flattened to cuboidal, and demonstrated a positive reaction for mesothelial markers (eg. calretinin, WT1), and a negative reaction for EpCAM, EMA, PAX8 and ER. Two cysts had cuboidal to flattened lining, the cells of which were diffusely or focally positive for mesothelial markers, for some epithelial markers (eg. EpCAM and EMA) and despite a lack of müllerian-type epithelium demonstrated a positive reaction for PAX8 and focally for ER. A cyst derived from adreno-hepatic fusion (AHF)-related intra-adrenal bile ductules was diagnosed in a right adrenal gland which was directly adherent to the liver, microscopically features of AHF were visible with intermingling of adrenal and liver parenchymal cells. The immunoreactivity pattern was similar among the preserved cells of the cyst-lining, the intra-adrenal bile ductules and the normal bile ductules in the adjoining liver parenchyma. On the basis of this case series from a single institution (8 presented now and 1 reported before) we propose a new histogenetic categorization of adrenal epithelial cysts into: 1. pure mesothelial cysts (the most common type), 2. mesothelial cysts with incomplete or complete müllerian metaplasia 3. AHF-related cysts., (Copyright © 2017 Elsevier GmbH. All rights reserved.)

Primary adrenal disorders contribute 20%â€"30% of patients with endogenous Cushing's syndrome. Most of the primary adrenal diseases are unilateral and include adenoma and adrenocortical carcinoma, whereas bilateral adrenal lesions are uncommon and include primary pigmented nodular adrenocortical disease, primary bilateral macronodular adrenocortical hyperplasia, isolated micronodular adrenocortical disease, bilateral adenomas or carcinomas, and rarely pituitary adrenocorticotropic hormone-dependent adrenal nodular disease. Cyclic adenosine monophosphate-dependent protein kinase A signalling is the major activator of cortisol secretion in primary adrenal nodular disorders. We report two cases of bilateral adrenal nodular disease with endogenous Cushing's syndrome, including one each of primary pigmented nodular adrenocortical disease and primary bilateral macronodular adrenocortical hyperplasia., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

A 70-year-old man with T-cell deficiency experienced continuous right waist pain and a low degree of fever. Abdominal CT found a large right adrenal mass. FDG PET/CT imaging was acquired to identify the nature of this likely malignant mass and search for potential metastases. The images revealed increased FDG activity in the mass. The patient received right adrenalectomy. Postsurgical pathological and microbial examination demonstrated adrenal cryptococcosis.

Introduction: The correct differentiation between unilateral and bilateral adrenal involvement in patients with primary aldosteronism (PA) is of utmost importance to justify surgical treatment. The aim of this study was to determine the accuracy of adrenal imaging compared to adrenal venous sampling (AVS), histopathology and postoperative outcome., Methods: The data of all patients with unequivocal AVS who underwent unilateral laparoscopic adrenalectomy for primary aldosteronism between May 2004 and April 2015 were entered in this retrospective study. We compared computed tomography (CT) and magnetic resonance imaging (MRI) results with corresponding AVS data, histopathology findings and postoperative outcome., Results: A total of 175 patients underwent unilateral laparoscopic adrenalectomy for primary aldosteronism. AVS was successful in 152 patients and postoperative outcome available in 148 patients. Despite unilateral disease according to AVS results, bilateral normal glands were seen in 15 MRI (17·2%) and 7 CT scans (8·5%), respectively. Unilateral enlargement of the nonhypersecreting adrenal gland was found in three MRI (3·5%) and 10 CT scans (12·2%) of patients who showed aldosterone hypersecretion deriving from the contralateral gland. Fifteen MRI (17·2%) and 18 CT scans (22·0%) revealed bilateral adrenal pathology despite unilateral aldosterone hypersecretion., Conclusion: The accuracy of CT and magnetic resonance imaging in predicting unilateral disease is poor. AVS appears to be an essential diagnostic step to identify those patients who may benefit from unilateral adrenalectomy., (© 2017 Stichting European Society for Clinical Investigation Journal Foundation.)

In the normal human adrenal gland, serotonin (5-HT) stimulates aldosterone secretion through the 5-HT 4 receptor (5-HT4R). However, the physiological role of the serotonergic control of adrenocortical function is not known. In the present study, we have investigated the ability of l-Lysine, which has been shown to act as a 5-HT 4 receptor antagonist, to counteract in vitro and in vivo the stimulatory effect of 5-HT4R agonists on aldosterone production. l-Lysine was found to inhibit aldosterone production induced by 5-HT and the 5-HT4R agonists BIMU8 from cultured human adrenocortical cells. The action of l-Lysine (4.95 g/day orally) on the adrenal cortex was also evaluated in 20 healthy volunteers in a double blind, cross-over, placebo controlled study. l-Lysine had no significant influence on basal plasma aldosterone levels and the aldosterone responses to upright posture, tetracosactide, and low sodium diet (10 mmol/day for 3 days). Conversely, l-Lysine significantly reduced the surge of plasma aldosterone induced by metoclopramide indicating that l-Lysine is able to efficiently antagonize the adrenal 5-HT 4 receptors in vivo. These results suggest that l-Lysine supplementation may represent a new treatment of primary adrenal diseases in which corticosteroid hypersecretion is driven by overexpressed 5-HT 4 receptors., (© Georg Thieme Verlag KG Stuttgart · New York.)

Purpose: Most knowledge regarding outcome after adrenal surgery stems from retrospective studies reported by highly specialized centres. The aim of this study was to report a national experience of adrenalectomy with particular attention to predictive factors for postoperative complications, conversion from endoscopic to open surgery and length of hospital stay., Methods: Adrenalectomies reported in the Scandinavian Quality Register for Thyroid, Parathyroid and Adrenal Surgery (SQRTPA) 2009-2014 were included. Risk factors for complications, conversion and hospital stay >3 days were assessed using univariable and multivariable logistic regression analysis., Results: There were 659 operations. Endoscopic adrenalectomy was performed in 513 (77.8%) operations and almost half of these were robotic assisted. The median length of hospital stay was 3 (range 1-30) days. There was no 30-day mortality. In 43 (6.6%) patients, at least one complication was registered. The only factor associated with complications in multivariable analysis was conversion to open surgery odds ratio (OR) 3.61 (95% confidence interval 1.07 to 12.12). The risk for conversion was associated with tumour size OR 1.03 (1.00 to 1.06) and with malignancy on histopathology OR 8.33 (2.12 to 32.07). Length of hospital stay increased in patients with operation of bilateral tumours OR 3.13, left-sided tumours OR 1.98, hyper secretion of catecholamines OR 2.32, conversion to open surgery OR 42.05 and open surgery OR 115.18., Conclusions: The present study shows that endoscopic surgery is widely used. Complications were associated with conversion and the risk for conversion was associated with tumour size and malignant tumour. Hospital stay was short.

Background: Extrahepatic manifestations of Echinococcus multilocularis are very rare, especially in the adrenal glands. To the best of our knowledge, only seven cases of adrenal alveolar echinococcosis have been reported, all from the Far East. All of these occurred exclusively in the right adrenal gland., Case Presentation: We report a rare case of an extrahepatic alveolar echinococcosis in an asymptomatic 78-year-old white man with an incidentaloma of his right adrenal gland. After surgical resection and medical treatment with albendazole no recurrence of the disease appeared at 1-year follow-up., Conclusions: As the occurrence of Echinococcus multilocularis in Europe increases, alveolar echinococcosis should be included in the differential diagnosis of cystic adrenal incidentalomas.

Objective: To perform a systematic review of published literature on adrenal biopsy and to assess its performance in diagnosing adrenal malignancy., Methods: Medline In-Process and Other Non-Indexed Citations, MEDLINE, EMBASE, and Cochrane Central Register of Controlled Trial were searched from inception to February 2016. Reviewers extracted data and assessed methodological quality in duplicate., Results: We included 32 observational studies reporting on 2174 patients (39.4% women, mean age 59.8 years) undergoing 2190 adrenal mass biopsy procedures. Pathology was described in 1621/2190 adrenal lesions (689 metastases, 68 adrenocortical carcinomas, 64 other malignancies, 464 adenomas, 226 other benign, 36 pheochromocytomas, and 74 others). The pooled non-diagnostic rate (30 studies, 2013 adrenal biopsies) was 8.7% (95%CI: 6-11%). The pooled complication rate (25 studies, 1339 biopsies) was 2.5% (95%CI: 1.5-3.4%). Studies were at a moderate risk for bias. Most limitations related to patient selection, assessment of outcome, and adequacy of follow-up. Only eight studies (240 patients) could be included in the diagnostic performance analysis with a sensitivity and specificity of 87 and 100% for malignancy, 70 and 98% for adrenocortical carcinoma, and 87 and 96% for metastasis respectively., Conclusions: Evidence based on small sample size and moderate risk of bias suggests that adrenal biopsy appears to be most useful in the diagnosis of adrenal metastasis in patients with a history of extra-adrenal malignancy. Adrenal biopsy should only be performed if the expected findings are likely to alter the management of the individual patient and after biochemical exclusion of catecholamine-producing tumors to help prevent potentially life-threatening complications., (© 2016 European Society of Endocrinology.)

Primary hyperaldosteronism is one of the most common causes of secondary hypertension but clear differentiation between its various subtypes can be a clinical challenge. We report the case of a 37-year-old African-American woman with refractory hypertension who was admitted to our hospital for palpitations, shortness of breath and headache. Her laboratory results showed hypokalaemia and an elevated aldosterone/renin ratio. An abdominal CT scan showed a nodule in the left adrenal gland but adrenal venous sampling showed elevated aldosterone/renin ratio from the right adrenal vein. The patient began a new medical regimen but declined any surgical options. We recommend clinicians to maintain a high level of suspicion to consider the less common subtypes of primary hyperaldosteronism, especially given the fact that the management greatly varies., (2016 BMJ Publishing Group Ltd.)

We have noted an increasing number of cases of enlarged adrenal glands where the underlying diagnosis was masked by a diffusely hemorrhagic process. We identified from our database 59 cases (32 consults, 27 routine) of adrenal glands with diffuse (>25%) hemorrhage received between 2000 and 2014. Fifty-three adrenalectomies and 6 biopsies were identified. The diagnoses after central review were 41 adrenocortical adenomas, 1 nodular adrenocortical hyperplasia with associated myelolipoma, 1 benign adrenocortical cyst, and 10 nonneoplastic adrenal glands with hemorrhage. A definitive diagnosis for the 6 biopsies was precluded by the sample size. The adrenocortical adenomas (size, 1-13 cm; 25%-95% hemorrhage) showed clear cell change in the neoplastic area (10%-80% of the tumor), 19 showed focal calcification (1 with ossification), 11 showed areas of papillary endothelial hyperplasia, 10 showed scattered lymphoplasmacytic inflammation, 6 showed benign cortical tissue extending beyond the adrenal capsule into soft tissue, 1 showed necrosis in the form of ghost cells, 2 showed lipomatous change, and 6 were associated with incidental benign lesions (1 cortical cyst, 1 schwannoma, and 4 myelolipomas). Twenty-four of the adrenocortical adenomas were consults where the referring pathologist had trouble classifying the lesion. Of the 10 nonneoplastic adrenals (4.5-22 cm; 40%-80% hemorrhage), 2 were consults. In summary, pathologists have difficulties recognizing adrenocortical adenomas in the setting of a massively enlarged and hemorrhagic adrenal gland. Although there is a correlation between adrenocortical malignancy and size, hemorrhage into nonmalignant adrenal glands can result in markedly enlarged adrenals., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Inflammation of the adrenal glands is caused by autoimmunopathies or infections and can induce adrenal insufficiency. Autoimmune lymphocytic adrenalitis is often combined with other autoimmune diseases and the most frequent cause of Addison's disease; however, it only becomes clinically apparent when more than 90 % of the adrenal cortex has been destroyed. Histological features are characterized by lymphoplasmacytic inflammation leading to an increased destruction of adrenocortical tissue but less severe courses can also occur. The second most frequent form of adrenalitis is adrenal tuberculosis, showing typical granulomatous findings that are nearly always caused by spreading from a tuberculous pulmonary focus. Other bacterial as well as viral infections, such as Epstein-Barr virus (EBV), cytomegalovirus (CMV) and others, generally affect the adrenal glands only in patients with immunodeficiency disorders. In these infections, the adrenal cortex and medulla are frequently involved to roughly the same extent. Although surgical specimens from inflammatory adrenal lesions are extremely rare, the various forms of adrenalitis play an important role in the post-mortem examination of the adrenal glands for clarification of unclear causes of death (e.g. death during an Addisonian crisis).

Objective: Our objective was to compare the perioperative parameters of needle-assisted and conventional laparoendoscopic single-site adrenalectomy (LESS-A)., Methods: We compared 23 patients undergoing needle-assisted LESS-A with 29 patients undergoing conventional LESS-A at Hiroshima University Hospital between November 2009 and February 2014. Needle-assisted LESS-A was performed using a MiniLap instrument (Stryker, San Jose, CA, USA). We used this instrument to protectively retract the liver at the right side of the tumor and the spleen at the left side by grasping with a Securea endoscopic surgical spacer (Hogy Medical Co., Ltd., Tokyo, Japan). Various parameters including insufflation time, estimated blood loss, pain scale, resumption of oral intake, transfusion rate, and complications were analyzed using the Mann-Whitney U test., Results: In all cases, LESS-A was completed successfully with no major intraoperative complications. Patients in both treatment groups had similar age, body mass index, sex, and laterality. Significantly, needle-assisted LESS-A was performed using the transumbilical approach rather than the subcostal approach. The insufflation time of the needle-assisted LESS-A was shorter than that of the conventional LESS-A (p = 0.0335). No patients required intraoperative or postoperative blood transfusions. Retrospective design and the small sample size are main limitations of this study., Conclusion: Needle-assisted LESS-A was performed safely and in a manner that mitigated many of the difficulties of LESS surgery., (Copyright © 2015. Published by Elsevier Taiwan.)

Objective: Histoplasmosis is an infectious disease caused by the dimorphic fungus Histoplasma capsulatum, endemic in central and eastern states of United States, South America and Africa. India is considered to be non-endemic area for histoplasmosis. Disseminated histoplasmosis may affect almost all systems. Disseminated histoplasmosis with asymptomatic adrenal involvement has been described in immunocompromised patients; whereas isolated adrenal involvement with adrenal insufficiency as the presenting manifestation of the disease is rare., Material and Method: Twelve patients from a non-endemic area with adrenal histoplasmosis, who were immunocompetent and diagnosed as adrenal histoplasmosis by cytology/histopathology between January 2012 to December 2014 were studied. 18F-FDG PET/CT (fluorodeoxyglucose positron emission tomography/computed tomography) was used to assess the extent of involvement., Results: There were a total of 12 immunocompetent males (mean age: 56.9 years). Ten patients had bilateral adrenal involvement and two had a unilateral left adrenal mass. All the patients had histopathologically/cytologically proven adrenal histoplasmosis. Two patients had simultaneous histoplasmosis of other sites, one in the epiglottis and the other in the alveolus. 18F-FDG PET/CT was performed in 10 patients showing high FDG uptake in the adrenals. All these patients received Amphotericin B and/or Itraconazole treatment that led to symptomatic improvement., Conclusion: A diagnosis of invasive fungal infection requires a high index of suspicion, especially in immunocompetent patients who present with nonspecific symptoms, clinical signs, laboratory and radiological features that can resemble adrenal neoplasms. Clinical specimens must be sent for cytopathology/histopathology together with fungal culture for a definite diagnosis and appropriate management.

Post-transplant lymphoproliferative disorder (PTLD) is one of the life-threatening complications after hematopoietic stem cell transplantation (HSCT) and solid organ transplantation (SOT), and it is associated almost exclusively with Epstein-Barr virus (EBV). We herein report 2 cases of EBV-associated PTLD after allogeneic HSCT localized in the adrenal gland. Both patients developed adrenal tumor within 3 months after HSCT and were successfully treated with rituximab or tapering immunosuppressive agents. Both remained alive without recurrence. A literature review revealed 12 reported cases of PTLD involving the adrenal gland, but the adrenal gland was involved as one of the lesions of advanced-stage PTLD after SOT. To the best of our knowledge, this is the first report to show cases of isolated EBV-associated adrenal PTLD after HSCT. PTLD should be recognized as one of the causes of isolated adrenal tumor after HSCT., (© 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Catheter ablation is established as a first-line therapy for most patients with recurrent supraventricular tachycardia (SVT), with high success rates and very low complication rates. A 60-year-old woman developed severe right flank pain following straightforward catheter ablation for SVT. This was caused by a spontaneous right adrenal haemorrhage, which, after much delay, was eventually recognised as the cause of her symptoms. Adrenal haematomas are rare and, to the best of our knowledge, this is the first reported case of spontaneous adrenal haemorrhage occurring after any interventional cardiac procedure. Clinicians should be aware of this rare but potentially serious complication and consider it as a differential diagnosis in any patient with severe flank pain following interventional cardiac procedures, to prevent delays in diagnosis., (2015 BMJ Publishing Group Ltd.)

We report a rare case of xanthogranulomatous adrenalitis in a 55-year-old man. The patient presented to the hospital with fever, nausea, and right flank pain. His medical history was significant for diabetes and an adrenal mass that was detected 6 years prior to presentation during a computed tomography (CT) scan for trauma secondary to a motor vehicle collision. The mass was thought to be a myelolipoma. Magnetic resonance imaging (MRI) revealed a 12-cm right adrenal mass that was considered suspicious for carcinoma, which was surgically excised and cultured intraoperatively. The cultures subsequently grew methicillin-resistant Staphylococcus aureus (MRSA). Grossly, the adrenal mass was an encapsulated, necrotic lesion with surrounding areas of fat necrosis. On histologic examination, the tissue showed sheets of histiocytes, lymphocytes, and plasma cells diffusely involving the adrenal gland along with bright yellow lipofuscin crystals in a background of necrosis and fibrosis.

Objectives: Computed tomography (CT) and magnetic resonance imaging (MRI) are the main imaging modalities used for analysis of adrenal lesions. We compared the ability of CT and MRI to detect and characterize benign adrenal lesions., Patients and Methods: Unenhanced abdominal CT and MRI were performed in 16 patients (age range 39-77), and reviewed by a radiologist with 6years of experience in abdominal imaging. The presence, number, size and structure of each mass were analyzed and compared between the two modalities., Results: There were 18 adrenal masses in 11 patients, four patients had adrenal hyperplasia (AH), whereas one patient had left-sided AH and right-sided adenoma. Ten masses were≥2cm in diameter, and were perfectly depicted using CT and all MRI techniques. There were nine masses with diameter<2cm detected by CT, three of them were missed using MRI. AH was detected in five patients using CT, but its mild form was missed in one patient using MRI. Four masses with attenuation values of>10Hounsfield units could not be characterized using unenhanced CT, but three of them were characterized using MRI., Conclusion: CT has higher sensitivity for detection of small adrenal tumours and adrenal hyperplasia than MRI. MRI is an important tool in characterization of adrenal masses that could not be characterized using unenhanced CT., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)

A northern Gulf of Mexico (GoM) cetacean unusual mortality event (UME) involving primarily bottlenose dolphins (Tursiops truncatus) in Louisiana, Mississippi, and Alabama began in February 2010 and continued into 2014. Overlapping in time and space with this UME was the Deepwater Horizon (DWH) oil spill, which was proposed as a contributing cause of adrenal disease, lung disease, and poor health in live dolphins examined during 2011 in Barataria Bay, Louisiana. To assess potential contributing factors and causes of deaths for stranded UME dolphins from June 2010 through December 2012, lung and adrenal gland tissues were histologically evaluated from 46 fresh dead non-perinatal carcasses that stranded in Louisiana (including 22 from Barataria Bay), Mississippi, and Alabama. UME dolphins were tested for evidence of biotoxicosis, morbillivirus infection, and brucellosis. Results were compared to up to 106 fresh dead stranded dolphins from outside the UME area or prior to the DWH spill. UME dolphins were more likely to have primary bacterial pneumonia (22% compared to 2% in non-UME dolphins, P = .003) and thin adrenal cortices (33% compared to 7% in non-UME dolphins, P = .003). In 70% of UME dolphins with primary bacterial pneumonia, the condition either caused or contributed significantly to death. Brucellosis and morbillivirus infections were detected in 7% and 11% of UME dolphins, respectively, and biotoxin levels were low or below the detection limit, indicating that these were not primary causes of the current UME. The rare, life-threatening, and chronic adrenal gland and lung diseases identified in stranded UME dolphins are consistent with exposure to petroleum compounds as seen in other mammals. Exposure of dolphins to elevated petroleum compounds present in coastal GoM waters during and after the DWH oil spill is proposed as a cause of adrenal and lung disease and as a contributor to increased dolphin deaths.

PET/CT with (18)F-fluorodeoxyglucose (FDG) or using different radiocompounds has proven accuracy for detection of adrenal metastases in patients undergoing cancer staging. It can assist the diagnostic work-up in oncology patients by identifying distant metastases to the adrenal(s) and defining oligometastatic disease that may benefit from targeted intervention. In patients with incidentally discovered adrenal nodules, so-called adrenal "incidentaloma" FDG PET/CT is emerging as a useful test to distinguish benign from malignant etiology. Current published evidence suggests a role for FDG PET/CT in assessing the malignant potential of an adrenal lesion that has been 'indeterminately' categorized with unenhanced CT, adrenal protocol contrast-enhanced CT, or chemical-shift MRI. FDG PET/CT could be used to stratify patients with higher risk of malignancy for surgical intervention, while recommending surveillance for adrenal masses with low malignant potential. There are caveats for interpretation of the metabolic activity of an adrenal nodule on PET/CT that may lead to false-positive and false-negative interpretation. Adrenal lesions represent a wide spectrum of etiologies, and the typical appearances on PET/CT are still being described, therefore our goal was to summarize the current diagnostic strategies for evaluation of adrenal lesions and present metabolic and anatomic appearances of common and uncommon adrenal lesions. In spite of the emerging role of PET/CT to differentiate benign from malignant adrenal mass, especially in difficult cases, it should be emphasized that PET/CT is not needed for most patients and that many diagnostic problems can be resolved by CT and/or MR imaging.

Foci of ectopic thyroid tissue are uncommon. Most sites of thyroid ectopia are confined to the neck region. The presence of ectopic thyroid tissue outside the migration pathway of the primitive thyroid in other locations is exceptional. Given that any disease of the thyroid gland may also affect ectopic thyroid tissue, pathologists has to recognize benign or malignant conditions that may develop in the ectopic focus. We present the case of a 32-year-old woman with ectopic thyroid parenchyma in the adrenal gland. Clinically, postoperative thyroid ultrasound echography and computed tomography scans did not reveal any thyroid tumor. The ectopic tissue was a cyst bordered by mature follicular thyroid structures and was histologically benign, without the molecular alterations associated with malignant tumors of follicular cell derivation (BRAFV600E, N-RAS, H-RAS, K-RAS). Review of the literature reveals that adrenal ectopic thyroid tissue is nearly always cystic and has distinctive pathologic features., (© The Author(s) 2014.)

Purpose: The guidelines on adrenal hemorrhage has not established in Japan. In this article, we discuss the management of adrenal hemorrhage., Objects and Methods: We experienced 6 patients from November 2004 to September 2013 in The University of Tokyo Hospital and The Fraternity Memorial Hospital, and we searched 57 cases already reported in Japan by using Japan Medical Abstracts Society (http://search.jamas.or.jp/). So we analyzed total 63 adrenal hemorrhage cases in Japan., Results: In 63 cases, 5 cases were performed TAE, 3 cases were performed emergent surgeries, 13 cases were managed conservatively and elective surgeries were performed in the other cases. 5 cases were fulfilled criteria for Hb < 10 g/dl and the maximum diameter of the hematoma > 10 cm. Of 5 cases, 4 cases were performed emergent hemostasis., Conclusions: Adrenal hemorrhages caused by metastatic tumor tend to be serious anemia. In addition, the most patients with adrenal hemorrhages, who had Hb < 10 g/dl and the maximum diameter of the hematoma > 10 cm, required immediate medical treatment, e.g. TAE or surgical hemostasis.

Unlabelled: Adrenal cystic lesions are uncommon but due to the improved radiologic imaging techniques their appearance seems to increase., Material and Methods: We analyzed the clinical and radiological findings of 10 patients with adrenal cysts and the pathological features of the operative material. Standard dissection procedure and paraffin embedded tissue sections were made, stained by HE and immunohistochemically with CD34, CD 31, Factor 8, Podoplanin, CKWS and AE1/AE3 RESULTS: The mean age of the patients was 40.6 years; female to male ratio was 2.3:1. All the cysts were diagnosed as cystic lesions radiologically except one. The most present clinical symptom was abdominal pain. The diameter of the cysts measured from 2 to 7 cm. Four of the cysts were diagnosed as pseudocysts and six as endothelial. Six cysts were lined by CD34(+) and CD31(+) cells, four were lined by Factor 8(+) and podoplanin(+) cells and four had no lining., Conclusion: Endothelial cysts were more common cysts in our study and the immunohistochemical results suggested common vascular origin to all endothelial cysts and supported additional separation of angiomatous and lymphangiomathous adrenal vascular cysts.

Computed tomography (CT) performed for a 75-year-old man as a follow-up examination for deep vein thrombosis in October 2010 revealed a left adrenal mass (diameter, 8 mm). In December 2012, the adrenal mass increased to 28 mm in diameter, and he was referred to our department. Several blood examinations revealed that the adrenal mass was non-functioning, and only peripheral lesions were observed to be enhanced by using CT in the arterial phase. Malignancy was suspected due to the irregular shape and growth of the mass, and left adrenalectomy was performed in February 2013. The histopathological diagnosis was adrenal mycobacteriosis, and clinical diagnosis was adrenal tuberculosis. No other tuberculosis infection-related lesion was detected, and the patient was treated with multidrug antituberculous chemotherapy.

Armadillo-containing proteins (ACPs) are a large family of evolutionary conserved proteins, characterized by the tandem repeat copy of a 42 amino acids motif, which forms a 3 dimensional protein-protein interaction domain. This permits ACPs to interact with plenty of partners and consequently, most of these proteins have several independent cellular roles. Perhaps the most well-known protein of this family is β-catenin, which is crucial in the regulation of development and adult tissue homeostasis through its 2 independent functions, acting in cellular adhesion in addition to being a transcriptional co-activator. APCs have important functions in many tissues, but here we summarize the adrenocortical role of 2 well-described ACPs, β-catenin (CTNNB1), Adenomatous Polyposis Coli (APC), and discuss the possible role in the adrenal cortex of the most recently discovered, Armadillo-repeat containing 5 (ARMC5)., (© Georg Thieme Verlag KG Stuttgart · New York.)

Opportunistic infections cause significant morbidity and mortality in patients infected with the human immunodeficiency virus (HIV). Multiple opportunistic infections can occur in a patient in the setting of severe immunodeficiency and can have atypical clinicoradiological presentation. Cardiac involvement has also been observed on autopsy in HIV-infected patients in the form of myocarditis, dilated cardiomyopathy, endocarditis, neoplasms, and drug-related cardiotoxicity. Mostly, the cardiac opportunistic infections are clinically asymptomatic, and sudden death due to these is extremely rare. We report a case of 44-year-old gentleman who presented with cough, pleuritic chest pain, and breathlessness and died of refractory shock due to myocarditis. At autopsy, he was found to have Toxoplasma myocarditis, Pneumocystis jirovecii pneumonia, and cytomegalovirus adrenalitis., (Copyright © 2014 Elsevier Inc. All rights reserved.)

An incidental, bilateral, retained fetal adrenal cortex was detected in a male cynomolgus macaque (age, approximately 2.4 y) used in a 4-week toxicology study. Microscopic examination of the adrenal gland cortex zone revealed the presence of additional solid sheets and columns of cells supported by vascular capillary bed and composed of large polyhedral cells with abundant eosinophilic, slightly finely vacuolated cytoplasm that surrounded the entire circumference of the medulla. Nuclei were vesicular, round to oval with prominent small nucleoli. There was no evidence for inflammation or cellular degeneration. Based on the microscopic examination, a diagnosis of retained fetal cortex of the adrenal gland was made. This morphologic change resembles fetal cortex in human infants. To our knowledge, this case description is the first report of a cynomolgus macaque with the rare entity of retained fetal cortex, which should not be misinterpreted as a test article-related change., (Copyright © 2014 Elsevier GmbH. All rights reserved.)

Objective: To analyze surgical methods and evaluate treatment efficacy and safety for managing adrenal cystic lesions., Materials and Methods: All patients presenting with adrenal lesions of the West China Hospital were reviewed retrospectively from January 2003 to April 2013 and 47 were diagnosed as adrenal cysts. Basic information, clinical history, physical examination, laboratory investigations, abdominal ultrasound and enhanced computed tomography were detailed noted. Cysts with different surgical management were analyzed and surgery option, operative time, postoperative complications and after-surgery hospital stay were all noted. The final diagnosis was judged by histopathology. Patients were followed from 3 month to 10 years., Results: All the 47 patients with a mean age of 43.8 years were managed by surgical intervention. Compared laparoscopic technology with open technology, the laparoscopic has the advantage of a shorter operation time, shorter hospital stay after surgery and enhanced cosmesis. The histopathologic result was: 23 (50%) were endothelial cysts and 16 (35%) were pseudocysts. One patient had evidence to recurrence at the followed-up stage., Conclusion: Adrenal cysts are rare and with the development of imaging techniques many of these are diagnosed incidentally. CT has advantages in detecting the cysts with haemorrhage, intracystic debris, calcification and mixed adrenal mass. Minimally invasive surgery offers equivalent efficacy to traditional open procedures, while providing a shorter operation time, shorter convalescence and improved cosmesis. Patients after surgical resection should be followed up closely especially if functional cysts and histopathology of cystic tumor are present.

Hydatid disease is endemic in parts of India, yet genitourinary involvement is rare. Laparoscopic management of such cases is uncommonly reported. We present a case of an adrenal hydatid and its management by laparoscopic aspiration, instillation of scolicidal solution, and partial excision of the cyst.

MR imaging has proven to be a versatile modality in evaluation of the kidneys, collecting system, and adrenal glands. By performing a comprehensive MR examination, it is not only possible to accurately characterize cystic and solid lesions of the kidneys, as well as urothelial masses, but also to provide important preoperative information to the surgeon. In addition, MR imaging can characterize many adrenal lesions and can frequently obviate biopsy. 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., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Objectives: To investigate the diagnostic performance of 15-min delayed contrast-enhanced computed tomography (15-DECT) compared with that of chemical shift magnetic resonance (CSMR) imaging in differentiating hyperattenuating adrenal masses and to perform subgroup analysis in underlying malignancy and non-malignancy., Methods: This study included 478 adrenal masses in 453 patients examined with 15-DECT and 235 masses in 217 patients examined with CSMR. Relative percentage washout (RPW) and absolute percentage washout (APW) on 15-DECT, and signal intensity index (SII) and adrenal-to-spleen ratio (ASR) on CSMR were measured. Sensitivity, specificity and accuracy of 15-DECT and CSMR were analysed for characterisation of adrenal adenoma. Subgroup analyses were performed in patients with and without underlying malignancy. Attenuation and size of the masses on unenhanced CT correlated with the risk of non-adenoma., Results: RPW calculated from 15-DECT showed the highest diagnostic performance for characterising hyperattenuating adrenal masses regardless of underlying malignancy, and the sensitivity, specificity and accuracy were 91.7 %, 74.8 % and 88.1 %, respectively in all patients. The risk of non-adenoma increased approximately threefold as mass size increased 1 cm or as its attenuation value increased by 10 Hounsfield units., Conclusions: 15-DECT was more accurate than CSMR in characterising hyperattenuating adrenal masses regardless of underlying malignancy., Key Points: Delayed contrast-enhanced CT and chemical shift magnetic resonance (CSMR) characterise adrenal lesions. 15-min DECT is more accurate than CSMR in characterising hyperattenuating adrenal masses. Sensitivity of CSMR decreases as the CT attenuation of adenomas increases. Risk of non-adenoma is increased 2.9-fold as size increased by 1 cm. Risk of non-adenoma is increased 2.9-fold as attenuation increased by 10 HU.

Advanced imaging often reveals adrenal tumors and tumor-like conditions in both symptomatic and asymptomatic patients. When adrenal disease is clinically suspected, cross-sectional imaging can be helpful in evaluating the etiology of the patient's symptoms. When adrenal disease is incidentally identified, what the clinician and patient really want to know is whether the findings are benign or malignant, as this ultimately will affect their next step in management. Using radiologic-pathologic correlation, we broadly classify common, uncommon, and rare tumors and tumor-like conditions that can occur in the adrenal as benign or malignant. This classification follows predominant trends in observed biologic behavior while acknowledging those tumors that may behave in the minority in an unpredictable manner. We review the clinical background and presentation of functional adrenal tumors including Conn syndrome, Cushing syndrome, and catecholamine-secreting tumors, as well as their relationship with adrenal anatomy. We discuss a variety of benign tumors, including adrenal cortical adenoma (including oncocytoma) and pheochromocytoma, as well as uncommonly and rarely encountered tumors such as myelolipoma, hemangioma, lymphangioma, schwannoma, ganglioneuroma, and adenomatoid tumor. A variety of tumefactive but nonneoplastic lesions are addressed, including adrenal cortical hyperplasia, adrenal hemorrhage, adrenal cysts, and infections. Malignant tumors discussed include adrenal cortical carcinoma, the rare malignant pheochromocytoma, lymphoma, metastases, and sarcomas. For each tumor and tumor-like lesion, the clinical presentation, epidemiology, key imaging findings, diagnostic differential considerations, and management options are briefly addressed. Finally, an approach to the workup of suspected or incidentally discovered tumors is presented based on a selected literature survey and our clinical experience. Radiologists play an important role in identification and diagnosis of adrenal tumors and tumor-like conditions in both symptomatic and asymptomatic patients., (RSNA, 2014)

The objective of this study was to analyze morphological changes and parasite loads in the adrenal gland from 45 dogs with visceral leishmaniasis (VL). The animals were from the Zoonosis Control Center of Araçatuba, state of São Paulo, which is an endemic region for the disease. These animals were euthanized due to positive diagnoses of VL. The dogs were classified into asymptomatic, oligosymptomatic and symptomatic groups. The parasite load was determined by immunohistochemistry, using VL-positive dog hyperimmune serum. Nine dogs showed an inflammatory infiltrate composed, predominantly, of plasma cells and macrophages. However, only eight dogs showed macrophages with amastigote forms of the parasite, immunolabeled in the cytoplasm. The medullary and reticular layers were the most affected areas, possibly due to a favorable microenvironment created by hormones in these regions. The density of parasites in the glandular tissue was not associated with clinical signs of VL (P > 0.05). However, the presence of the parasite was always associated with the presence of a granulomatous inflammatory infiltrate. This gland may not be an ideal place for the parasite's multiplication, but the presence of injuries to the glandular tissue could influence the dog's immune system, thus favoring the parasite's survival in the host's different organs.

Background: Posterior retroperitoneoscopic adrenalectomy (PRA) was popularized by Walz and colleagues as an alternative approach to minimally invasive adrenalectomy, offering less postoperative pain and faster return to normal activity compared with laparoscopic transperitoneal adrenalectomy (LA). The authors have recently changed from LA to PRA in suitable patients and audited their outcomes., Methods: Data were prospectively collected for 10 patients who underwent PRA, and a chart review and telephone interviews were conducted with 13 consecutive patients who underwent LA by the same surgeon. Patient demographics, tumor characteristics, analgesia use, operative and anesthetic time, length of stay, and complications were recorded., Results: Data were collected for 13 LAs and 10 PRAs. Patients' baseline characteristics, including age, BMI, and tumor size, were similar between the 2 groups. There were no conversions to open surgery, transfusions, or deaths. Operative time was similar between the 2 groups. PRA patients required less, inpatient postoperative opioid analgesia compared with LA patients (median 1.25 vs. 23 mg of intravenous morphine equivalent, P=0.003), and had a shorter length of stay (median 1 vs. 2 d, P<0.001). The median total days on opioids were lower for PRA patients compared with LA patients (0.5 vs. 9 d, P<0.001)., Conclusion: Our initial results supports previously published findings that PRA is a safe procedure, with a relatively short learning curve, resulting in reduced postoperative analgesia use, and reduced length of hospital stay when compared with the laparoscopic transperitoneal approach.

Introduction: The aim of this study was to evaluate the feasibility and usefulness of supersonic shear wave elastography (SSWE) in the diagnosis of nonmalignant adrenal masses., Material and Methods: 13 patients with a total number of 16 adrenal masses were enrolled in the study. In each case, both conventional ultrasound imaging and SSWE for stiffness assessment were performed. The final diagnosis was based on CT, MRI, biochemical studies, surgery or more than one year of follow up., Results: The final diagnosis: nodular hyperplasia in six masses, six adenomas, three cysts, and one myelolipoma. All solid adrenal masses presented the elastography signal in contrast to cystic lesions that were devoid of it, as shear waves do not propagate through fluids., Conclusions: SSWE is a feasible technique that can be applied during ultrasound of the abdomen and retroperitoneum. SSWE presents potential for the differentiation of solid and cystic adrenal lesions. Further large scale studies evaluating the possibility of differentiation of adrenal and other retroperitoneal masses with SSWE are warranted.

Objectives: The incidence of unilateral adrenal histoplasmosis in nonendemic areas is rare. This is even rarer if the host is immunocompetent., Methods: We report a patient who was referred for unexplained weight loss who underwent a computed tomography scan of abdomen revealing a large unilateral adrenal nodule measuring 7.6 cm in size., Results: Hormonal workup was negative including urine 24-hour free metanephrines, and the patient underwent unilateral adrenalectomy. The pathology showed budding yeast that stained with Gomori's methenamine silver (GMS) revealed the diagnosis of adrenal histoplasmosis., Conclusion: We encourage clinicians to include infection by Histoplasma capsulatum as well as other granulomatous diseases and tumors in the differential diagnosis of unilateral adrenal enlargement even in immunocompetent hosts living in nonendemic areas.

Background: Ectopic thyroid tissue is usually found anywhere along the embryonic descent pathway of the medial thyroid anlage from the tongue to the trachea (Wölfler area). However, ectopic thyroid tissue in the adrenal gland (ETTAG) is not easy to understand on the basis of thyroid embryology; because it is so rare, the possibility of metastasis should first be considered. Here, we describe two cases of ETTAG with pathogenetic implications and review the associated literature., Patient Findings: Two cases of ETTAG presented as incidental cystic adrenal masses in adult females, one having a congenital hernia of Morgagni. The ETTAG was histologically indistinguishable from normal orthotopic thyroid tissue, and its follicular nature was confirmed by immunohistochemical positivity for thyroglobulin, thyroperoxidase, thyroid transcription factor-1 (TTF-1/Titf-1/Nkx2.1), cytokeratin AE1/AE3, cytokeratin 7, pendrin, human sodium iodide symporter, paired box gene 8, and forkhead box E1 (TTF-2), as well as positivity for the messenger RNA of the thyroglobulin gene by in situ hybridization analysis. No C cells (negativity for calcitonin, chromogranin, and synaptophysin) were present. Neither BRAF nor KRAS mutations were detected with real-time polymerase chain reaction analysis. Further work-up did not show evidence of thyroid malignancy., Summary: ETTAG is a rare finding, with only seven cases reported; women are much more frequently affected than men (8:1), and it usually presents in the fifth decade (mean age 54, range 38-67) as a cystic adrenal mass incidentally discovered on abdominal ultrasonography and/or in computed tomography images. ETTAG is composed of normal follicular cells without C cells. The expression of some transcription factors (TTF-1, paired box gene 8, and FOXE1) involved in development and/or migration of the medial thyroid anlage is preserved. Coexistence of a congenital hernia of Morgagni in one patient suggests an overdescent of medial thyroid anlage-derived cells in its pathogenesis., Conclusion: Although ETTAG pathogenesis remains unknown, the lack of C cells together with the coexistence of a congenital defect of the anterior diaphragm (hernia of Morgagni) in one of our patients could suggest an overdescent of medial thyroid anlage-derived cells in the origin of this heterotopia.

Objective: The purpose of this article is to determine the natural history of adrenal cysts on the basis of long-term imaging follow-up., Materials and Methods: This retrospective study included patients with adrenal cysts who had at least 12 months of imaging follow-up (1993-2010). Medical records were reviewed. Two radiologists reviewed imaging examinations in consensus and recorded wall thickness (thin, ≤ 3 mm; thick, > 3 mm), septations, and calcification. CT attenuation value, MRI signal intensity, the presence or absence of enhancement, and typical sonographic features were used to confirm fluid content of the lesions. Cyst wall enhancement was recorded (thin, ≤ 3 mm and smooth; thick, > 3 mm). Cyst diameter on the initial and most remote follow-up examinations was compared. The Wilcoxon matched-pairs signed rank test was applied to assess statistically significant differences in size and CT attenuation on follow-up examinations., Results: Twenty patients with unilateral adrenal cysts (seven male and 13 female patients; mean age, 44 years; range, 10-75 years) had a mean imaging follow-up period of 64 months (range, 12-198 months). CT, MRI, and ultrasound examinations were obtained in 19, 11, and 13 patients, respectively. Cysts were diagnosed by lack of enhancement on CT or MRI in 12 patients, typical sonographic features in three patients, and combination of CT and sonographic or MRI features in five patients. Signal intensities typical for fluid were found on all MRI examinations, attenuations of less than 20 HU on 17 of 19 (89%) CT examinations, and features of either simple or mildly complicated cysts on all sonograms. Thin walls, wall calcifications, and thin septations were found in 20 (100%), 12 (60%), and four (20%) lesions, respectively. During the follow-up of 20 lesions, the median cyst diameter increased by 26.0% (interquartile range, 6.8-68.4%) in 12 (60%) patients, decreased by 32.9% (interquartile range, 7.1-42.8%) in six (30%) patients, and was unchanged in two (10%) patients. The median baseline CT attenuation values did not significantly change on follow-up CT examinations (p = 0.72). No patient developed a complication of adrenal cyst. Four patients had histologically confirmed benign adrenal cysts., Conclusion: Interval enlargement of an adrenal cyst is frequent and as an isolated finding does not indicate malignancy or presence of a complication. However, some adrenal cysts may decrease or remain stable in size over time.

Adrenal cysts are rare. Most are benign but some may contain malignancy. There are no established guidelines for their surgical management. The purpose of this study was to determine the pathological findings and likelihood of malignancy in hormonally inactive adrenal cysts after adrenalectomy. Using the pathology registries at two centers, we identified patients who underwent excision of an adrenal cyst between 1994 and 2009. Hormonally active cysts including pheochromocytomas were excluded. Charts were reviewed for patient demographics, presentation, surgical management, and postoperative course. Of 551 adrenalectomy specimens, 15 (2.7%) contained an inactive adrenal cyst or cystic component. Cysts were more likely to be in women (67%) and right-sided (73%). Three patients (20%) were symptomatic from their lesion. Laparoscopic adrenalectomy was performed in nine patients (60%). Pathology revealed eight hemorrhagic cysts, four lymphangiomas, one hemangioma, one epithelial cyst, and one metastatic pulmonary adenocarcinoma. Laboratory and radiographic workup are essential in determining whether adrenal cysts have hormonal function or a solid tissue component before adrenalectomy. Although nonfunctional adrenal cysts may contain malignancy, most are benign. It is reasonable to observe asymptomatic, nonfunctioning, benign-appearing adrenal cysts in patients in whom follow-up can be ensured.

Introduction: Adrenal pseudocysts are rare cystic masses that arise from the adrenal gland and which are usually non-functional and asymptomatic. We report a rare case of a giant hemorrhagic adrenal pseudocyst presenting with abdominal pain and we discussed the radiological features., Presentation of Case: A 75 year old man was admitted with acute abdominal pain post mild-trauma. Computed tomography (CT) of abdomen revealed a hemorrhagic mass measuring 18 cm located in the right suprarenal region, displacing the right kidney and liver. He subsequently underwent to contrast enhancement ultrasound (CEUS), which showed features suggestive for hemorrhagic adrenal pseudocyst. A complete endocrine working didn't show any hormonal hypersecretion. The patient underwent laparotomy and right adrenal mass was excised. Histological examination revealed giant hemorrhagic adrenal pseudocyst. The abdominal pain resolved after surgery., Conclusions: to the best our knowledge, this is the first case studied with CEUS reported in the literature.