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Apart from neuroblastomas, adrenal tumors are rarely seen in children. The most common adrenal tumors are adrenocortical carcinoma and pheochromocytoma. Adrenocortical carcinoma is usually a large heterogeneous, well-marginated mass with solid/cystic areas and calcifications, with poor prognosis. Most of the pheochromocytomas are benign tumors and usually show intense contrast enhancement, the pattern of which may be diffuse, mottled, or peripheral on computed tomography and magnetic resonance imaging. The purpose of this article is to evaluate primary nonneurogenic adrenal tumors.

Schwannoma of the adrenal gland is very rare, comprising 0.2% of adrenal tumors, presenting usually as non-specific abdominal pain. We report such a rare case in a 50-year-old female. Her only complaint was right lumbar pain. Ultrasonography (USG) and computed tomography (CT) both showed a supra renal cystic mass most likely of adrenal origin. Excision of mass was performed. Post-operative course was uneventful. Histopathology showed a benign tumor of Schwann cell origin. Usually, pre-operative imaging including CT and magnetic resonance imaging (MRI) are inconclusive and diagnosis has to be confirmed on histopathology.

The aim of this study was to evaluate the diagnostic accuracy of the 1 mg dexamethasone suppression test (DST) for the prediction of autonomous cortisol secretion (ACS)-related comorbidities in patients with adrenal incidentalomas (AIs). This was a retrospective multicenter study. We recruited patients with AI/s ≥ 1 cm, excluding those who, during the study, were found during the extension study of an extra-adrenal cancer, with a known diagnosis of hereditary syndromes characterized by adrenal tumors, those presenting with overt hormonal excess syndromes, and those in whom the DST results were missing. A total of 823 patients met the inclusion criteria. Based on the 1.8, 3.0, and 5.0 µg/dl post-DST cortisol thresholds, the prevalence of ACS was 33.5%, 13.7%, and 5.6%, respectively. The prevalence of hypertension (OR = 1.8, 95% CI = 1.3–2.4), diabetes (OR = 1.6, 95% CI = 1.2–2.2), and dyslipidemia (OR = 1.4, 95% CI = 1.0–1.9) was higher with cortisol post-DST ≥ 1.8 µg/dl; the prevalence of hypertension (OR = 2.1, 95% CI = 1.4–3.3) and diabetes (OR = 1.7, 95% CI = 1.1–2.6) was higher with values ≥ 3.0 µg/dl; and the prevalence of hypertension (OR = 2.0, 95% CI = 1.0–3.7) was higher with levels ≥ 5.0 µg/dl. However, the diagnostic accuracy of the DST for the prediction of cardiometabolic comorbidities in patients with AIs was poor, with areas under the ROC curve

Background Laparoscopic adrenalectomy is widely used for treating various adrenal tumors. Posterior retroperitoneoscopic adrenalectomy was introduced after transperitoneal laparoscopic adrenalectomy. The comparability and superiority of posterior retroperitoneoscopic adrenalectomy have been widely investigated. We aimed to compare the outcomes of posterior retroperitoneoscopic adrenalectomy and transperitoneal laparoscopic adrenalectomy using 10 years' data. The changes in outcomes over time were also analyzed. Methods This was a retrospective observational study. A total of 505 patients who underwent laparoscopic adrenalectomy between 2009 and 2018 were included. The patients were divided into two groups: transperitoneal (n=114) and retroperitoneal (n=391) groups. Patients who underwent posterior retroperitoneoscopic adrenalectomy were further classified into sub-groups (sub-group 1: 2009-2012; sub-group 2: 2013-2015; and sub-group 3: 2016-2018) based on time periods. Clinicopathological factors and postoperative outcomes were retrospectively reviewed and analyzed. Results The retroperitoneal group showed significantly shorter operation time, diet initiation time, and hospital stay compared to the transperitoneal group (P=0.000, 0.000 and 0.000, respectively). Analgesics were used less frequently in the retroperitoneal group than in the transperitoneal group (P=0.048). In the retroperitoneal group, the time to postoperative diet initiation shortened over time (P=0.000). Conclusions Posterior retroperitoneoscopic adrenalectomy is a favorable surgical method that results in patient outcomes that are comparable to those of conventional transperitoneal laparoscopic adrenalectomy, without compromising patient safety.

Adrenal tumors are common tumors in urology and they can be further divided into functioning and nonfunctioning tumors according to whether there is uncommon endocrine function. In clinical practice, the early identification and accurate assessment of adrenal tumors are essential for the guidance of subsequent treatment. However, a nonfunctioning adrenal tumor often lacks obvious clinical symptoms, making it difficult to be timely and precisely diagnosed by conventional examinations. Therefore, a rapid and accurate method for identifying the functioning and nonfunctioning adrenal tumors is urgently required to achieve precise treatment of adrenal tumors. In this study, surface-enhanced Raman spectroscopy was investigated as a diagnostic tool to identify the blood serum samples from healthy volunteers as well as the patients with functioning and nonfunctioning adrenal tumors. Based on the SERS peak analysis, abnormal glycolysis, DNA/RNA, and amino acid metabolites were found to be potential biomarkers for identifying patients with adrenal tumors, while metabolites related to disordered protein catabolism and excessive hormone secretion were expected to further differentiate functioning adrenal tumors from nonfunctioning adrenal tumors. In addition, principal component analysis followed by support vector machine (PCA-SVM) was further applied on those serum SERS measurements, and the classification accuracies of 96.8% and 84.5% were achieved for differentiating healthy group versus adrenal tumor group and functioning adrenal tumor group versus nonfunctioning adrenal tumor group, respectively. The results have demonstrated the prodigious potential of precise adrenal tumor diagnosis by using the blood serum surface-enhanced Raman spectroscopy technique.

Background Adrenal hemangioma is a rare benign adrenal tumor that is usually misdiagnosed preoperatively. We here present a case of adrenal cavernous hemangioma that was successfully treated with retroperitoneal laparoscopic adrenalectomy. Case presentation A 67-year-old man with dull right back pain attended our clinic for examination of a mass on the right adrenal gland for 1 week. Pheochromocytoma was considered according to the preoperative computed tomography angiography + computed tomography urography findings and was subsequently corrected to adrenal gland hemangioma according to postoperative pathological findings. The patient showed no recurrence of adrenal hemangioma during the 1-year follow-up period after surgery. Conclusion Adrenal gland hemangioma is rare with a high rate of misdiagnosis, and it should be considered in imaging findings of adrenal tumors with typical hemangioma. Surgery is an effective treatment method.

Introduction. In the literature, the insufficient attention is paid to preoperative planning of access to adrenal masses using modern computed tomographic navigation capabilities. The purpose. To demonstrate the possibilities of designing a safe access for adrenalectomy with the appliation of three-dimensional printed models based on the integral assessment of preoperative computed tomographic data.Materials and methods. The possibilities of preoperative design of access for adrenalectomy were studied in 362 patients with adrenal tumors, for whom computed tomography was performed on an Aquillion 64 (Toshiba, Japan).Results. Reliable anthropometric (BMI, body shape) and CT criteria for designing surgical access to the right and left NP were determined. Three patients with a borderline number of risk criteria for the development of vascular complications associated with technical difficulties of adrenalectomy (for the right AP, ≥4, for the left AP, ≥3) underwent CT-segmentation of images followed by the creation of three-dimensional plates — a model of the AP tumor with adjacent organs and vessels.Conclusion. Preoperative computed tomographic access design, taking into account the criteria of the risk of complications and the application of three-dimensional printed models, make it possible to reasonably use endoscopic and open adrenalectomy options, significantly improving the immediate results of patient treatment.

Background Some authors consider adrenal lesions size of less than 4 cm as a positive cut-off limit to set the indications for minimally invasive surgery due to a lower risk of malignancy. Aim of this study is to report the risk of cancer for adrenal lesions measuring 4 cm or more in diameter, assessed as benign at preoperative workup (primary outcome), and to evaluate the feasibility and safety of laparoscopic adrenalectomy (LA) in these cases (secondary outcome). Methods From January 1994 to February 2019, 579 patients underwent adrenalectomy. Fifty patients with a preoperative diagnosis of primary adrenal cancer or metastases were excluded. The remaining 529 patients were included and divided in five subgroups based on adrenal lesion size at definitive histology: group A, 4–5.9 cm (137 patients); group B, 6–7.9 cm (64 patients); group C, 8–9.9 cm (13 patients); group D, ≥ 10 cm (11 patients); group E, Results Four (2.9%) malignant lesions were observed in group A, 5 (7.8%) in group B, 2 (15.4%) in Groups C and D (18.2%) and 13 (4.3%) in Group E. Comparing the cancer risk among the groups, no statistically significant differences were observed. Operative time increased with increasing lesion size. However, no statistically significant differences were observed between benign and malignant lesions in each group comparing operative time, conversion and complication rates, postoperative hospital stay and mortality rate. Conclusions Adrenal lesions measuring 4 cm or more in diameter are not a contraindication for LA neither in terms of cancer risk nor of conversion and morbidity rates, even if the operative time increases with increasing adrenal lesion diameter. Further prospective studies with a larger number of patients are required to draw definitive conclusions.

Background: We aimed to compare the results of patients who underwent laparoscopic adrenalectomy (LA) for masses ≥6 cm versus

To determine the accuracy of cystic appearance within adrenal masses on contrast-enhanced CT in distinguishing pheochromocytomas and malignant adrenal tumors from adenomas. We performed a retrospective review of adult patients with pathologically proven adrenal tumors who underwent contrast-enhanced abdominal CT. There were 92 patients (mean age 64.7 years, 52 men) with 22 pheochromocytomas, 34 malignant masses, and 36 adenomas. Two abdominal radiologists independently reviewed CT images to determine the presence of cystic appearance within the adrenal masses, defined as focal regions of low attenuation within the tumor that subjectively had fluid density. Cystic appearance was present in 12/22 (55%, 95% CI 32–76%) pheochromocytomas (mean size 5.3 cm), 15/34 (44%, 95% CI 27–62%) malignant masses (mean size 5.8 cm), and 2/36 (5.6%, 95% CI 0.7–9%) adenomas (mean size 3.2 cm). Sensitivity and specificity of cystic appearance for distinguishing pheochromocytoma or malignant masses from adenomas were 48.2% (95% CI 34.7–62.0%) and 94.4% (95% CI 81.3–99.3%), respectively. Cystic appearance was a significant predictor of tumor type (p = 0.015) even after controlling for tumor size. Reader agreement for cystic appearance was almost perfect with a kappa of 0.85. Cystic appearance in adrenal tumors on contrast-enhanced CT has high specificity and low sensitivity for distinguishing pheochromocytoma and malignant adrenal masses from adenomas.

Although safe and feasible, partial adrenalectomy is not a widespread procedure. Endorsement of robotic technologies and fluorescence techniques in adrenal surgery might help develop partial adrenalectomy and could avoid unnecessary total adrenalectomies. When performed in selected cases, partial adrenalectomy is associated with good postoperative outcomes comparable with those reported after total adrenalectomy. It has been hypothesized that one of the advantages of the robotic approach in adrenal-sparing surgery is to reduce manipulation of the gland allowing preservation of the vascularization of the residual adrenal, overcoming some limits when performing a laparoscopic conventional approach. A major drawback of the robotic surgery is its cost, but the overcost due to the use of the robotic system could be balanced by the execution of a high number of partial adrenalectomies leading to fewer life-long replacement steroid treatment. Partial adrenalectomy could become the recommended management for small benign and hormonal active adrenal tumors. Indocyanine green fluorescence (IGF) also seems to be a useful technique to help surgeons identify the adrenal gland and to locate small tumors from the normal adrenal tissue in difficult patients. It is likely that the use of a robotic approach associated with IGF may extend indications of partial adrenalectomy in the years to come.

Background/Aim. Incidentalomas of the adrenal gland are adrenal masses commonly discovered by chance on imaging not performed for suspected adrenal disease. The aim o f t his study was to analyze clinical, hormonal and histopathological characteristics of adrenal incidentalomas. Methods. This retrospective study included 85 patients (32 men and 53 women) examined for adrenal incidentalomas at the Clinic for Endocrinology, Military Medical Academy in Belgrade, from January 2013 to December 2017. The age of the patients, gender, size, and localization of adrenal tumors, functional activity, as well as the presence of comorbidities were analyzed. Adrenalectomy was performed in 35 patients due to its size and functional activity, and histological findings were analyzed. Results. The largest number (56.4%) of the adrenal tumors, was detected by ultrasound examination of the abdomen, 23 (27.2%) by abdominal computed tomography (CT) scan, 13 (15.2%) by chest CT scan and 1 (1.2%) by magnetic resonance (MR) imaging of the abdomen. The average tumor size was 3.8 ? 2.3 cm (range from 1 to 1 5 c m). Adrenal tumors were bilateral in 20 (23%) patients, in 39 (46%) patients, the tumors were localized in the right adrenal gland, and in 26 (31%) in the left gland. Subclinical hypercortisolism, defined as insufficient cortisol suppression during overnight dexamethasone test (1 mg), was observed in 34 (40%) patients, while the absence of cortisol suppression (autonomous cortisol secretion) was found in 4 (4.7%) patients. In the remaining 47 (55.3%) patients, complete overnight suppression of cortisol secretion was achieved. Thirty-five (41%) patients underwent adrenalectomy; among them, in 4 (11.4%) cases, adrenocortical carcinoma was found, 15 (42.9%) were adenomas, pheochromocytoma was found in 4 (11.4%) cases, nodular hyperplasia in 5 (14.3%) cases, distant metastasis in one (2.8%) case and the remaining were different benign masses. Conclusion. For patients with adrenal incidentalomas, two fundamental questions on determining the functionality of the tumor and/or the presence of malignancy need to be clarified. All patients with adrenal incidentaloma should undergo hormonal evaluation for autonomous or possible autonomous cortisol secretion, as well as for autonomous, adrenergic, and mineralocorticoid excess. In patients with autonomous adrenal secretion, surgery is indicated even if the typical clinical manifestation is absent.

Segmental caudal vena cava (CVC) aplasia is a rare congenital vascular anomaly in dogs. Two dogs were diagnosed by CT imaging to have right adrenal tumors with concomitant segmental CVC aplasia. During surgery, a firm connection between the right adrenal gland and CVC was observed in both cases. The adrenal glands were found ventral to the CVC and the adrenal tumor was resected including the vascular wall. CVC venectomy for tumor removal will be required if the right adrenal gland is displaced in dogs with segmental CVC aplasia, even if there is no intravascular invasion.

The presence of prostatic tissue, in addition to uterus and adrenal tumors, is possible in 46XX patients with CAH. Lesions of these organs are usually benign. However, complications including prostate and adrenal cancer were also reported.

Delivery of breath-hold MR-guided SABR is time-consuming, and the use of real-time tumor-tracking in a sagittal plane may fail to detect out-of-plane displacements of organs-at-risk. Analysis of daily MR-scans performed pre- and post-SABR revealed frequent decreases in stomach volumes, and in the planned stomach doses.

Image-guided radiofrequency ablation (RFA) has been accepted as a minimally invasive treatment for adrenal tumors in patients who are unable to undergo adrenalectomy. Accordingly, this treatment has become more readily available for treating functioning or non-functioning adrenal masses. Thus, endocrinologists need a better understanding of percutaneous RFA of adrenal tumors. The purpose of this review is to briefly describe the basic mechanism of RFA, indications and contraindications, patient preparation prior to RFA, type of complications, how to avoid complications, RFA procedures, and treatment outcomes.

Summary Introduction. - The objective of this publication is to recall the initial oncological management of adrenal incidentalomas. Material & Methods. - The multidisciplinary working group updated french urological guidelines established by the CCAFU in 2018, based on an exhaustive literature review carried out on PubMed. Results. - Although the majority of the adrenal masses are benign and non-functional, it is important to investigate them, as a percentage of these can cause serious endocrine diseases or be cancers. Malignant adrenal tumors are mainly represented by Adrenocortical Carcinomas (ACC), malignant pheochromocytomas (MPC) and adrenal metastases (AM). The malignancy assessment of an adrenal incident includes a complete history, a physical examination, a biochemical / hormonal assessment to look for subclinical hormonal secretion. Diagnostic hypotheses are sometimes available at this stage, but it is the morphological and functional imaging and the histological analysis which will make it possible to close the malignancy assessment and make the oncological diagnosis. Conclusions. - AC and MPC are mainly sporadic but a hereditary origin is always possible. ACC is suspected preoperatively but the diagnosis of certainty is histological. The diagnosis of MPC is more delicate and is based on clinic, biology and imagery. The diagnosis of certainty of AM requires a percutaneous biopsy. At the end, the files must be discussed within the COMETE – adrenal cancer network (Appendix 1).

Highlights • Adrenal incidentalomas are tumors found accidentally by imaging. • Hemangiomas are quite rare and in certain cases their surgical intervention should never be overlooked. • Embolization is primarily implied but one should never neglect the failure rate and the need for surgical intervention. • There are 4 types of AH: cavernous hemangioma, venous hemangioma, capillary type hemangioma, and mixed hemangioma. • To our knowledge, our case has the largest size of an AH being reported in the literature with a 17 cm., Introduction Adrenal incidentalomas are tumors found accidentally by imaging. Among the incidentalomas, hemangiomas are quite rare and in certain cases their surgical intervention should never be overlooked. Presentation of case We present a case of 70 years old Lebanese female with an adrenal tumor presented as syncope found to have anemia on presentation and a bleeding 17 cm adrenal tumor on imaging. Patient had workup to rule out functioning adrenal tumors and decision to excise the tumor was taken after failure of embolization. Pathology report denied malignancy despite of the huge size and was in favor of hemangioma. Conclusion Adrenal hemangiomas are rare and they rarely present as syncope. Attention to such a life-threatening condition should be sustained. Embolization is primarily implied but one should never neglect the failure rate and the need for surgical intervention.

Highlights • A chronic expanding hematoma of the adrenal gland is extremely rare. • Chronic expanding hematomas mimic sarcomatous lesions. • Gallium scintigraphy may help the differential diagnosis between chronic expanding hematomas and sarcomatous lesions. • Complete resection of chronic expanding hematomas is the gold standard. • Preoperative arterial embolization may reduce intraoperative bleeding on complete tumor removal., Introduction A chronic expanding hematoma in the retroperitoneal space is a rare disease with poorly understood pathology, and preoperative diagnosis of such hematomas using conventional methods is sometimes difficult. Presentation of case A 68-year-old man with a history of slowly progressive abdominal distention was referred to our department for further evaluation. Contrast-enhanced CT revealed a large retroperitoneal tumor of the adrenal gland. MRI revealed that the tumor was iso-intense to hyperintense on T2-weighted imaging, with heterogeneous signal intensity on T1-weighted imaging without fat components. Angiography of the left adrenal artery confirmed many extravasations into the tumor. However, gallium scintigraphy showed no accumulation in the tumor. These findings were suggestive of a chronic expanding hematoma of left adrenal gland. This patient underwent complete tumor resection. Postoperative histopathological findings revealed a chronic expanding hematoma. Discussion Chronic expanding hematomas are slowly expanding, space-occupying masses as a result of trauma, surgery, or bleeding disorders. Chronic expanding hematomas mimic malignant tumors such as sarcomatous lesions. Although CT and MRI are used to obtain the diagnosis, the diagnosis is sometimes difficult. Gallium scintigraphs play a pivotal role in the differential diagnosis between them. Conclusion Gallium scintigraphs, magnetic resonance imaging and computed tomography, are useful tools to differentiate chronic expanding hematomas from sarcomatous lesions.

Purpose To explore the laparoscopic technique with the retroperitoneal approach for complex adrenal tumors. Patients and methods The clinical data of 11 patients with complex adrenal tumors from July 2017 to July 2018 were analyzed retrospectively. Among them, there were 4 males and 7 females, 4 with adrenal myelolipomas, 3 with adrenal pheochromocytomas, 2 with adrenal cysts, 1 with adrenocortical adenoma and 1 with adrenal ganglioneuroma. The average tumor diameter was 6.5 ± 1.2 cm, and the average age of the patients was 48 ± 13 years. Results All the operations were successfully completed. The average operation time was 95 ± 15 min, the average amount of blood loss was 50 ± 15 mL, and the average postoperative hospital stay was 2.6 ± 1.3 days. No tumor recurrence was found after 1 year of follow-up. Conclusion Retroperitoneal laparoscopic surgery is effective for the treatment of complex adrenal tumors, but it requires good surgical skills. Surgeons skilled in laparoscopic technology can safely carry out retroperitoneal laparoscopic surgery for complex adrenal tumors.

Minimally invasive techniques such as Image guided thermal ablation are now widely used in the treatment of tumors. Microwave ablation (MWA) is one of the newer modality of thermal ablation and has proven its safety and efficacy in the management of the tumors amenable for ablation for primary and metastatic diseases. It is used in the treatment of primary and secondary liver malignancies, primary and secondary lung malignancies, renal and adrenal tumors and bone metastases. We wanted to share our initial experience with this newer modality. In this article we will describe the mechanism and technique of MWA, comparison done with RFA, advantages and disadvantages of MWA along with pre procedure workup, post procedure follow-up and review of literature.

Recently, more and more attention has been directed to the role of adipose tissue and adipocytokines in the pathogenesis of metabolic and inflammatory disorders in humans. Excess fat tissue has also been associated with a higher risk of malignancies. Advances in the research on the role of adipokines in adrenal tumors may elucidate the relationship between various types of adipose tissue (visceral, subcutaneous, and periadrenal) and metabolic disorders observed in hormonally active adrenal tumors, as well as associations with adrenal cortex cancer. In patients with active or cured Cushing syndrome, increased leptin and resistin concentrations as well as release of pro-inflammatory cytokines can be associated with cardiovascular risk. Also, the renin-angiotensin-aldosterone system in patients with primary hyperaldosteronism may affect the metabolic activity of the adipose tissue. Elevated resistin concentrations in this group of patients are associated with morphological changes of the myocardium independently of the effects of the metabolic syndrome. Further, it has been suggested that hypoadiponectinemia comprises an additional factor in the pathogenesis of carbohydrate metabolism disorders and the risk of cardiovascular complications in pheochromocytoma patients. Understanding the mechanisms of action of adipokines may be important in developing prophylactic and therapeutic strategies in hormonally active and malignant tumors of the adrenal glands.

Currently, laparoscopic adrenalectomy is considered as the preferred technique to manage adrenal tumors. However, there are no prospective randomized studies evaluating this strategy. With the recent advances in surgical equipment and the widespread of robotic technology, a robotic approach is considered as an interesting option in some medical centers. This approach seems to be feasible and safe but high-level evidence of its benefits is still lacking. This review summarizes indications, advantages and drawbacks of robotic adrenalectomy and describes its surgical technique.

Aim: We aim to demonstrate that a surface-enhanced Raman spectroscopy (SERS) probe can be effectively used for protein detection in adrenal tumors. Materials & methods: The SERS probe method, which uses Au@Ag core-shell nanoparticles conjugated with a CgA antibody and a SERS reporter, was applied to detect CgA in adrenal tumors. Results: Our data reveal that the results of the CgA-SERS probe method were almost identical to those of western blot and superior to those of traditional immunohistochemistry. Conclusion: This study offers a novel strategy to detect CgA in adrenal tumors and provides more reliable protein test results than traditional immunohistochemistry analysis for adrenal pathologists, meaning that it might be a better clinical reference for the diagnosis of pheochromocytoma.

The increased incidence of accidental revealing adrenal tumors in radiation studies requires further improvement of laboratory diagnostic methods for the timely detection of their latent hormonal activity and malignancy potential, as well as the determination of strategic therapeutic approaches. The aim of the work was to evaluate the capabilities of modern laboratory diagnostics in verification of latent forms of hormone-active adrenal tumors to determine further treatment tactics. The study included 207 (14.9%) of 1390 patients in whom the hormones precursors of steroidogenesis and catecholamine metabolites were studied in blood plasma by liquid chromatography, as well as tumor markers. These patients were divided into a group of subclinical forms of hormone-active NP tumors (n = 173) and a group of truly hormone-inactive formations (n=34). It has been established that pre-aldosteroma is characterized with an increase of corticosterone (8.1±3.4 ng/ml), 11-deoxycorticosterone (12.3±3.0 ng/ml), 18-hydroxycorticosterone (5.4±1,4 ng/ml) in blood. For pre-corticosteroma: increased cortisol (119.2±16.1 ng/ml), 11-deoxycortisol (11.5±1.9 ng/ml), 11-deoxycorticosterone (12.8±2.1 ng/ml), the ratio of cortisol/cortisone (9.1±1.6 ng/ml) in blood. The presence of «silent» pheochromocytoma was confirmed by an increase of blood free methanephrine (105.0±27.1 ng/ml) and normetanephrine (196.0±43.6 ng/ml), as well as chromogranin A (223.3±15.3 pg/ml). The latent forms of adrenocortical cancer were characterized by an increase in blood dehydroepiandrosterone sulfate (45.2±3.9 μmol/L), cancer embryo antigen (9.8±1.2 ng/ml), vascular endothelial growth factor (1122.0±24.5 ng/ml), IL-6 (95.4±1.8 pg/ml), 11-deoxycortisol (21.8±4.5 ng/ml), 11-deoxycorticosterone (4.2±3.2 ng/ml). All 173 patients with hormone-active NP tumors, as well as 30 (88.2%) with large (6.5±2.0 cm) hormone-inactive formations with the presence of compression syndrome, underwent adrenalectomy. Determination of the precursors of steroidogenesis and catecholamine metabolites in blood plasma by liquid chromatography can reliably identify the functional activity of adrenal tumors and determine their malignant potential, as well as substantiate indications for timely surgical treatment.

In the largest retrospective study of adrenal incidentalomas (AIs) in Japan between 1999 and 2004, adrenal tumors detected during secondary hypertension (HT) screening were included. The characteristics of patients with adrenal tumors detected during HT screening may differ from those of patients with AIs. This study aimed to compare the characteristics of patients with AIs with those of patients with adrenal tumors detected during HT screening. We retrospectively analyzed patients referred to our division for detailed examination of adrenal tumors between April 2009 and April 2017. When the purposes of imaging tests included HT screening, we defined adrenal tumors as HT associated, otherwise as strictly defined AIs. We reviewed data on age, sex, purpose and modality of imaging, location of tumor, tumor diameter, and hormonal evaluation. We identified 104 patients with HT-associated adrenal tumors and 413 with AIs. Patients with HT-associated adrenal tumors were younger (54.2 years vs. 61.7 years, p < 0.001) and had smaller tumor diameters (1.3 cm vs. 1.9 cm, p < 0.001), lower prevalence of nonfunctioning tumors (24.0% vs. 67.6%, p < 0.001), and higher prevalence of primary aldosteronism (58.7% vs. 4.8%, p < 0.001) than those with AIs. There were no differences in terms of tumor location and prevalence of subclinical Cushing's syndrome, Cushing's syndrome, and pheochromocytoma (18.3% vs. 16.0%, 7.7% vs. 8.0%, and 2.9% vs. 4.6%, respectively). In conclusion, patients with HT-associated tumors were younger and had a smaller tumor with higher prevalence of primary aldosteronism than those with AIs.

Chronically sustained systemic hypertension in dogs can damage the kidneys, eye, brain, heart, and vessels. In human medicine, systemic hypertension has been implicated as the most common risk factor for aorta dilation, which can progress to an aneurysm. Abdominal ultrasound has been commonly used to monitor the size of the abdominal aorta in people with systemic hypertension. In this retrospective cross-sectional abdominal ultrasound study, evaluation of the size of the abdominal aorta relative to the caudal vena cava was performed in 18 control dogs and 128 dogs with confirmed systemic hypertension. Preexisting conditions contributing to systemic hypertension in these dogs were renal disease, hyperadrenocorticism, diabetes mellitus, adrenal tumors, and previous administration of phenylpropanolamine or palladia. The abdominal aorta and caudal vena cava were assessed from longitudinal images cranial to the trifurcation with measurements made from outer border to outer border of the walls, being careful not to compress the caudal vena cava that would alter its size. Our hypothesis was the ratio of the diameter of the abdominal aorta to caudal vena cava would be higher in dogs with systemic hypertension compared to dogs with normal blood pressure. The mean abdominal aorta-caudal vena cava ratio was 1.028 in control dogs with a normal blood pressure and 1.515 in dogs with systemic hypertension. In dogs with confirmed systemic hypertension, the abdominal aorta was dilated compared to the caudal vena cava in the caudal abdomen. An increase in the abdominal aorta-caudal vena cava ratio in a dog should raise suspicion for the presence of systemic hypertension and prompt evaluation of blood pressure.

Many years of experience in identifying patients with various pathologies of the adrenal glands using multispiral computed tomography are presented. Multispiral CT is a highly informative method of choice in the diagnosis of adrenal tumors. Multispiral CT in the perfusion version of the tumor of the adrenal gland increases the accuracy of diagnosis, which optimizes the tactics of treatment. Improving the algorithm for assessing perfusion indices of the adrenal gland incidentalomas using multispiral CT will allow to evaluate the histological nature of the pathological process. It is justified to conduct perfusion MSCT studies in patients with volumetric adrenal masses with the purpose of differential diagnosis of hyperplastic and tumor processes in them. An attempt was made to develop an algorithm for MSCT diagnostics of the adrenal glands and determine its effectiveness. The radiological characteristic of the adrenal glands incidentalomas is a reflection of their morphological structure. There are statistically significant differences in aldosterone-producing and cortisol-producing adenomas in size and CT density. Morphological signs of adrenal tumor degeneration correlate with functional activity. Surgical treatment is absolutely indicated for patients with incidentalomas corresponding to the radiological phenotype of malignant tumors, as well as for patients who have revealed hormonal activity during a comprehensive examination. Dynamic observation is indicatedfor patients with hormonal-inactive incidental small-sized (< 4 cm) in the absence ofradiological signs of malignancy. In patients with an extremely high risk of surgical treatment, endovascular interventions aimed at suppressing adrenal hyperfunction are advisable. The main prognostic factors for adrenal glands incidentalomas are: radiological phenotype, hormonal activity, duration of the disease, the choice of adequate treatment tactics. In surgical treatment, the prognosis depends on the morphological characteristics of adrenal gland incidentalomas.

Abdominal tumors (AT) in children account for approximately 17% of all pediatric solid tumor cases, and frequently exhibit embryonal histological features that differentiate them from adult cancers. Current molecular approaches have greatly improved the understanding of the distinctive pathology of each tumor type and enabled the characterization of novel tumor biomarkers. As seen in abdominal adult tumors, microRNAs (miRNAs) have been increasingly implicated in either the initiation or progression of childhood cancer. Moreover, besides predicting patient prognosis, they represent valuable diagnostic tools that may also assist the surveillance of tumor behavior and treatment response, as well as the identification of the primary metastatic sites. Thus, the present study was undertaken to compile up-to-date information regarding the role of dysregulated miRNAs in the most common histological variants of AT, including neuroblastoma, nephroblastoma, hepatoblastoma, hepatocarcinoma, and adrenal tumors. Additionally, the clinical implications of dysregulated miRNAs as potential diagnostic tools or indicators of prognosis were evaluated.

PurposeHormonal assessment (HA) and contrast-enhanced CT (ceCT) show insufficient sensitivity and specificity when staging unilateral adrenal lesions (ALs). We aimed at: 1) developing an imaging-based, i.e. ceCT and FDG-PET, diagnostic score able to discriminate adrenal tumors entailing adrenalectomy from those who need clinical monitoring, and 2) identifying a diagnostic flow-chart that allows clinicians to avoid unneeded diagnostic procedures and to address patients to the optimal management.MethodsRetrospective single-center study assessing patients with unilateral AL and the following inclusion criteria: a) a four-phase ceCT; b) FDG-PET within one month of the ceCT; c) histopathology or a clinical follow-up of at least 24 months. Firstly, multivariate logistic regression analysis was employed to identify the predictors of adrenal tumors to treat surgically (AL-to-treat) and regression-based coefficients were used to develop a diagnostic score. Secondly, using cut-offs of predictor variables, a diagnostic flow-chart was defined.ResultsForty-eight patients were enrolled (mean age 61 years), of whom 21/48 (44%) had AL-to-treat. The remaining 27/48 (56%) presented with AL to follow-up only (i.e. benign). Maximum and minimum lesion diameter, Hounsfield units (HU) before contrast media injection and HU at the delayed phase (HUdelayed), relative and absolute washout, AL SUVmax, AL SUVmean, ratio SUVmax AL/SUVmax liver (adrenal-liver ratio) were associated with the presence of AL-to-treat (all pConclusionWe developed a two-parameter imaging-based score that may be applied to predict adrenal tumors requiring adrenalectomy. Furthermore, a diagnostic flow-chart to stratify patients on the basis of the optimal management was proposed, thus guiding undefined unilateral adrenal lesions to FDG-PET imaging.

Background: The chemokine receptor CCR7 is crucial for an intact immune function, but its expression is also associated with clinical outcome in several malignancies. No data exist on the expression of CCR7 in adrenocortical tumors. Methods: CCR7 expression was investigated by qRT-PCR and immunohistochemistry in 4 normal adrenal glands, 59 adrenocortical adenomas, and 181 adrenocortical carcinoma (ACC) samples. Results: CCR7 is highly expressed in the outer adrenocortical zones and medulla. Aldosterone-producing adenomas showed lower CCR7 protein levels (H-score 1.3 ± 1.0) compared to non-functioning (2.4 ± 0.5) and cortisol-producing adenomas (2.3 ± 0.6), whereas protein expression was variable in ACC (1.8 ± 0.8). In ACC, CCR7 protein expression was significantly higher in lymph node metastases (2.5 ± 0.5) compared to primary tumors (1.8±0.8) or distant metastases (2.0 ± 0.4, p &lt, 0.01). mRNA levels of CCR7 were not significantly different between ACCs, normal adrenals, and adrenocortical adenomas. In contrast to other tumor entities, neither CCR7 protein nor mRNA expression significantly impacted patients’ survival. Conclusion: We show that CCR7 is expressed on mRNA and protein level across normal adrenals, benign adrenocortical tumors, as well as ACCs. Given that CCR7 did not influence survival in ACC, it is probably not involved in tumor progression, but it could play a role in adrenocortical homeostasis.

Barbara Choroma&nacute;ska,1 Piotr My&sacute;liwiec,1 Tomasz Koz&lstrok;owski,1 Magdalena &Lstrok;uba,1 Piotr Wojskowicz,1 Jacek Dadan,1 Hanna My&sacute;liwiec,2 Katarzyna Choroma&nacute;ska,3 Katarzyna Makarewicz,4 Anna Zalewska,5 Mateusz Maciejczyk6 1 1st Department of General and Endocrine Surgery, Medical University of Bialystok, Bialystok, Poland; 2Department of Dermatology and Venereology, Medical University of Bialystok, Bialystok, Poland; 3Department of Oral Surgery, Medical University of Gdansk, Gdansk, Poland; 4Regional Centre for Transfusion Medicine in Bialystok, Bialystok, Poland; 5Experimental Dentistry Laboratory, Medical University of Bialystok, Bialystok, Poland; 6Department of Hygiene, Epidemiology and Ergonomics, Medical University of Bialystok, Bialystok, PolandCorrespondence: Mateusz Maciejczyk Email mat.maciejczyk@gmail.comBackground: Adrenal masses are the most common of all human tumors. The role of nitrosative stress and inflammation in cancer development has already been demonstrated. However, it is not known whether they are involved in the pathogenesis of adrenal tumors. The aim of the study was to investigate a cross-talk between nitrosative stress, inflammation and hypoxia-inducible factor (HIF-1α) in 75 patients with different types of adrenal masses (non-functional incidentaloma, pheochromocytoma and Cushing’s/Conn’s adenoma).Methods: The plasma concentrations of total nitric oxide (NO), S-nitrosothiols, peroxynitrite nitrotyrosine and the activity of serum myeloperoxidase (MPO) were measured spectrophotometrically, whereas concentrations of interleukin 1 beta (IL-1β), tumor necrosis factor α (TNF-α) and hypoxia-inducible factor 1 alpha (HIF-1α) were measured using commercial ELISA kits. The control group consisted of 50 healthy people matched by age and sex to the study group. The number of subjects was determined a priori based on our previous experiment (power of the test = 0.9; α = 0.05).Results: We found significantly higher nitrosative stress (↑nitric oxide, ↑peroxynitrite, ↑S-nitrosothiols and ↑nitrotyrosine) in the plasma of patients with adrenal tumors, which was accompanied by increased inflammatory (↑myeloperoxidase, ↑interleukin 1 beta and ↑tumor necrosis factor α) and hypoxia (HIF-1α) biomarkers. Peroxynitrite and nitrotyrosine were positively correlated with aldosterone level. Nitrosative stress was also associated with inflammation and HIF-1α. Interestingly, plasma nitrotyrosine and serum MPO differentiated patients with adrenal tumor from healthy individuals with high sensitivity and specificity. Moreover, using multivariate regression analysis, we showed that ONOO− and IL-1β depended on cortisol level, while ONOO−, nitrotyrosine and HIF-1α were associated with aldosterone. Unfortunately, none of the assessed biomarkers differentiated between tumor types studied, suggesting that the severity of nitrosative damage and inflammation are similar in patients with incidentaloma, pheochromocytoma, and Cushing’s or Conn’s adenoma.Conclusion: Adrenal tumors are associated with increased protein nitration/S-nitrosylation and inflammation.Keywords: adrenal tumors, nitrosative stress, nitric oxide, peroxynitrite, S-nitrosothiols, nitrotyrosine, myeloperoxidase, interleukin 1 beta, tumor necrosis factor, α hypoxia-inducible factor

Cystic adrenal tumors are rare with an incidence of approximately 0.06% in the general population. Four main histological types are distinguished: Endothelial cysts of lymphangiomatous or angiomatous origin, pseudocysts, epithelial cysts and cysts of parasitic origin. Surgery is recommended for signs of complications, suspicion of malignancy and large size. In other cases, simple surveillance can be proposed. We report here a case of a lymphangiomatous endothelial cyst of the adrenal gland. The objective is to recall the clinical characteristics and to specify the diagnostic contribution of imaging as well as the therapeutic modalities of this entity.

The NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for Neuroendocrine and Adrenal Gland Tumors focus on the diagnosis, treatment, and management of patients with neuroendocrine tumors (NETs), adrenal tumors, pheochromocytomas, paragangliomas, and multiple endocrine neoplasia. NETs are generally subclassified by site of origin, stage, and histologic characteristics. Appropriate diagnosis and treatment of NETs often involves collaboration between specialists in multiple disciplines, using specific biochemical, radiologic, and surgical methods. Specialists include pathologists, endocrinologists, radiologists (including nuclear medicine specialists), and medical, radiation, and surgical oncologists. These guidelines discuss the diagnosis and management of both sporadic and hereditary neuroendocrine and adrenal tumors and are intended to assist with clinical decision-making. This article is focused on the 2021 NCCN Guidelines principles of genetic risk assessment and counseling and recommendations for well-differentiated grade 3 NETs, poorly differentiated neuroendocrine carcinomas, adrenal tumors, pheochromocytomas, and paragangliomas.

Simple Summary Adrenal cortical carcinoma (ACC) is an extremely rare disease with a variable prognosis. Current prognostic markers have limitations in identifying patients with a poor prognosis and who require adjuvant therapy. We developed the prognostic biomarker candidates of ACC using mass-spectrometry-based proteomics and machine learning algorithm. We further validated them in The Cancer Genome Atlas data and performed the survival analysis according to the expression levels of each protein. In addition, HNRNPA1, the protein identified as a prognostic marker of ACC based on proteomics, was validated in the immunohistochemistry staining. The prognostic protein biomarkers of ACC found in this study are expected to help determine the appropriate treatment plan for patients. Abstract Adrenal cortical carcinoma (ACC) is an extremely rare disease with a variable prognosis. Current prognostic markers have limitations in identifying patients with a poor prognosis. Herein, we aimed to investigate the prognostic protein biomarkers of ACC using mass-spectrometry-based proteomics. We performed the liquid chromatography–tandem mass spectrometry (LC–MS/MS) using formalin-fixed paraffin-embedded (FFPE) tissues of 45 adrenal tumors. Then, we selected 117 differentially expressed proteins (DEPs) among tumors with different stages using the machine learning algorithm. Next, we conducted a survival analysis to assess whether the levels of DEPs were related to survival. Among 117 DEPs, HNRNPA1, C8A, CHMP6, LTBP4, SPR, NCEH1, MRPS23, POLDIP2, and WBSCR16 were significantly correlated with the survival of ACC. In age- and stage-adjusted Cox proportional hazard regression models, only HNRNPA1, LTBP4, MRPS23, POLDIP2, and WBSCR16 expression remained significant. These five proteins were also validated in TCGA data as the prognostic biomarkers. In this study, we found that HNRNPA1, LTBP4, MRPS23, POLDIP2, and WBSCR16 were protein biomarkers for predicting the prognosis of ACC.

This review article discusses the diagnoses and treatment of patients with multiple endocrine neoplasia type 1 (MEN 1). The most common tumors associated with MEN 1 are located in the pancreas, pituitary, and parathyroid glands. Less common tumors include neuroendocrine tumors of the lung and thymus, adrenal tumors, and cutaneous lesions. This article describes the diagnosis, clinical manifestations, treatment, and surveillance of tumors associated with patients who are diagnosed with MEN 1.

Total antioxidant capacity better characterizes the redox status of the biological system than the determination of individual antioxidants separately. This study is the first to assess the total antioxidant/oxidant status in the plasma and urine of patients with adrenal tumors. The study group consisted of 60 patients (31 women and 29 men) with adrenal masses, classified into three subgroups: non-functional incidentaloma, pheochromocytoma and Cushing’s/Conn’s adenoma. The number of patients was set a priori based on our previous experiment (α = 0.05, test power = 0.9) Plasma total antioxidant capacity (TAC) was increased in incidentaloma patients, whereas in pheochromocytoma group was decreased. Plasma and urine total oxidant status (TOS) and oxidative stress index (OSI) were significantly higher in patients with adrenal tumors. Ferric reducing antioxidant potential (FRAP) was decreased in plasma and urine, while DPPH (2,2-diphenyl-1-picrylhydrazyl) antiradical activity only in plasma of patients with adrenal masses. In pheochromocytoma patients, plasma and urine TAC, as well as plasma DPPH and FRAP correlated positively with methanephrine and normethanephrine. Reduced levels of TAC, DPPH and FRAP clearly indicate a reduced ability to scavenge free radicals and thus a lack of effective protection against oxidative stress in patients with adrenal tumors. Therefore, those patients are especially vulnerable to oxidative stress and oxidative damage, which can lead to impaired cellular metabolism. Both plasma and urine redox biomarkers can be used to assess systemic antioxidant status in adrenal tumor patients.

Cortical sparing adrenalectomy allows for resection of adrenal tumor(s) while preserving unaffected adrenal tissue to prevent adrenal insufficiency. This is especially important in patients affected with bilateral adrenal tumors, typically pheochromocytomas. Posterior retroperitoneoscopic adrenalectomy (PRA) allows for a minimally invasive approach to adrenal gland resection compared to the more traditional laparoscopic transabdominal adrenalectomy and open approaches. The PRA technique is increasingly used by high-volume endocrine surgeons throughout the world. This approach is ideal to address patients with bilateral disease and was utilized in this case of a patient presenting with bilateral pheochromocytomas in the setting of multiple endocrine neoplasia 2A syndrome.

Autonomous cortisol secretion (ACS) affects up to 50% of patients with adrenal adenomas. Despite the limited evidence, clinical guidelines recommend measurement of serum concentrations of dehydroepiandrosterone-sulfate (DHEA-S) and corticotropin (ACTH) to aid in the diagnosis of ACS. Our objective was to determine the accuracy of serum concentrations of DHEA-S and ACTH in diagnosing ACS. We conducted a retrospective single center study of adults with adrenal adenoma evaluated between 2000−2020. Main outcome measure was diagnostic accuracy of DHEA-S and ACTH. ACS was defined as post-dexamethasone cortisol &gt, 1.8 mcg/dL. Of 468 patients, ACS was diagnosed in 256 (55%) patients with a median post-DST cortisol of 3.45 mcg/dL (range, 1.9–32.7). Patients with ACS demonstrated lower serum concentrations of DHEA-S (35 vs. 87.3 mcg/dL, p &lt, 0.0001) and ACTH (8.3 vs. 16 pg/mL, p &lt, 0.0001) compared to patients with non-functioning adrenal tumors (NFAT). Serum DHEA-S concentration &lt, 40 mcg/dL diagnosed ACS with 84% specificity and 81% PPV, while serum ACTH concentration &lt, 10 pg/mL diagnosed ACS with 75% specificity and 78% PPV. The combination of serum concentrations of DHEA-S &lt, 40 mcg/dL and ACTH &lt, 10 pg/mL diagnosed ACS with the highest accuracy with 92% specificity and 87% PPV. Serum concentrations of DHEA-S and ACTH provide additional value in diagnosing ACS.

Adrenocortical carcinoma (ACC) is a rare cancer with poor survival. We sought to identify prognostic factors and assess its clinical utility in postoperative management of nonmetastatic ACC. We included 65 patients who underwent adrenalectomy and clinicopathological diagnosed as European Network for the Study of Adrenal Tumors (ENSAT) stage I–III ACC in our center from 2009 to 2017. Potential clinicopathological parameters were selected. The prognostic correlation, including overall survival (OS) and recurrence-free survival (RFS), was analyzed using Kaplan–Meier method and univariate and multivariate Cox model. The 2-year and 5-year post-surgery survival rate were 54.6% (95%CI: 42.5–70.1%) and 33.5% (95%CI: 22.3–50.6%), respectively. 46 (70.8%) cases were symptomatic ACC. Tumor-related or hormone-related symptom was identified as independent prognostic factor in OS (HR = 5.5, 95%CI: 1.87–16.16) and RFS (HR = 3.62, 95%CI: 1.28–10.24). Higher tumor grade (Weiss score > 6 or Ki67 index ≥ 20%) was independently associated with poor OS (HR = 4.73, 95%CI: 2.15–10.43). R status (R1/R2/Rx) was independently correlated with worse RFS (HR = 2.56, 95%CI:1.21–5.43). Accordingly, patients with higher GRAS (Grade, R status, age, and symptoms) score were more likely to have poor prognosis (OS: HR = 2.7, 95%CI: 1.43–5.11 and RFS: HR = 3.31, 95%CI: 1.68–6.52, respectively). Symptoms, higher tumor grade and positive/unknown R status were independent risk factors in stage I–III ACC. Comprehensive consideration of GRAS parameters may optimize the prognostic assessment for postoperative patients.