Adrenal Tumors Research Articles

Adjuvant Radiotherapy After Surgical Resection Improves Local Control in Adrenocortical Carcinoma

ABSTRACTAimsAdrenocortical carcinoma is a rare cancer known for its high recurrence rate. This study aimed to evaluate the effects of postoperative adjuvant radiotherapy on the outcomes of patients with adrenocortical carcinoma.MethodsWe examined patients with adrenocortical carcinoma who had undergone curative tumor resection. Tumor stages were classified using the European Network for the Study of Adrenal Tumors staging system. Out of 131 patients, 15 underwent adjuvant radiotherapy. Patients who underwent surgery and adjuvant radiotherapy were compared with those who underwent surgery only.ResultsBaseline characteristics were similar between the adjuvant radiotherapy (n = 15) and control groups (n = 30). Local recurrence occurred in three patients (20%) who received adjuvant radiotherapy and 18 patients (60%) in the control group (p < 0.05). The estimated 3‐year locoregional‐free survival was significantly higher in the adjuvant radiotherapy group (77%) compared to the control group (38.1%, p < 0.05). However, there were no significant differences in recurrence‐free or overall survival between the two groups.ConclusionsPostoperative adjuvant radiotherapy significantly enhances local control of adrenocortical carcinoma. It should be considered a crucial component of treatment, particularly for patients at high risk of recurrence.

Adrenocortical carcinoma with circulating tumor DNA analysis at post-operative recurrence: a case report with review of literature.

Recently, the usefulness of circulating tumor DNA (ctDNA) analysis in various malignancies has been reported. However, reports on ctDNA analysis in adrenocortical carcinoma (ACC) are few. Therefore, this study aimed to examine the detectability of genetic mutations in ctDNA and the association between ctDNA allelic ratio and disease progression in a patient with post-operative recurrence of ACC. A 77-year-old woman presented with a 5.4 cm left adrenal mass, which was clinically diagnosed as subclinical cortisol-producing ACC on close examination. She underwent left adrenalectomy and was diagnosed with stage II (T2N0M0) ACC. Post-operatively, adjuvant chemotherapy with mitotane was commenced because of histologically high-grade ACC. However, 17 months post-operatively, she had a local recurrence at the left adrenalectomy site. FoundationOne® CDx Cancer Genome Profile showed CTNNB1 G34A mutation in the resected adrenal tumor. She had heart failure and interstitial pneumonia and was treated with radiotherapy for local recurrence. Subsequently, lung and liver metastasis appeared post-operatively at 21 and 23 months, respectively. Serum dehydroepiandrosterone sulfate and computed tomography findings at 27 months post-operatively showed disease progression. We collected the peripheral blood at 23 and 27 months post-operatively and analyzed 18 genes associated with adrenal disease in plasma cell-free DNA and the resected adrenal tumor using a next-generation sequencer. At both time-points, CTNNB1 mutations consistent with the primary tumor were observed in ctDNA, with the allelic ratio increasing over time from 8% to 27%. In conclusion, monitoring the ctDNA allelic ratio may be useful for evaluating disease progression in advanced ACC.

Adrenal oncocytoma: a rare presentation of a benign 18F-fluorodeoxyglucose PET avid virilising adrenal tumour.

A woman in her 70s presented with features of hyperandrogenism including clitoral enlargement and deepening of her voice. Biochemical investigations revealed raised plasma androgens and urinary androgen metabolites and imaging findings showed a highly F-18 fluorodeoxyglucose (FDG)-PET avid left adrenal tumour initially suspected to be a malignant adrenocortical carcinoma (ACC). She subsequently underwent an uncomplicated laparoscopic adrenalectomy where complete resection of her tumour was achieved. Histopathological analysis demonstrated a benign adrenal oncocytoma with no evidence of malignancy. This case illustrates a rare presentation of a functioning virilising adrenal oncocytoma as a benign mimic of ACC.

A rare and fortunate case of unruptured splenic peliosis – case report and literature review

Splenic peliosis is an extremely rare pathological entity of unclear etiology characterized by the presence of multiple blood-filled cavities. Although numerous associations have been described in the literature, the etiology remains unclear. The present case is about a healthy 40-year-old woman who was referred to our institution due to chronic symptoms of abdominal discomfort and constipation. Despite the abdominal computed tomography (CT) scan and abdominal magnetic resonance imaging (MRI) reported an adrenal mass, reviewing the scans, the radiologist suggested a 17,5 cm, well-vascularized splenic mass. A splenectomy was performed, without any complications. We discuss the current available literature and emphasize the importance of awareness of this diagnosis.

Molecular and Clinical Features of Adrenocortical Tumors in Beckwith–Wiedemann Spectrum

Background/Objectives: Adrenocortical tumors (ACTs), including adrenocortical adenoma (ACA) and carcinoma (ACC), represent 0.3–0.4% of pediatric tumors. Beckwith–Wiedemann spectrum (BWSp) confer an increased risk of ACTs, but prognosis, management, and associated molecular characteristics are unclear. Methods: This paper combines a literature review of 54 published cases of BWSp-ACT with a report of one newly identified patient, totaling 55 cases with a confirmed BWSp clinical and/or molecular diagnosis. Results: Nineteen patients with ACA, 33 with ACC, and 3 with ACT of uncertain malignant potential (umACT) were included. Twenty patients had uniparental disomy of chromosome 11p15.5 (patUPD11), 11imprinting Center 2 Loss-of-methylation (IC2-LoM), and had 2 11p15 locus duplication. Eleven patients were diagnosed during cancer screening procedures, including two metastatic at diagnosis ACC. Conclusions: Almost half of ACC patients reached the minimum score for clinical BWSp diagnosis only after ACC onset, suggesting that the BWSp score has limited value for the early diagnosis in such a setting. Two patients with metastatic ACC had a histopathological Wieneke score ≤2, not correlating with clinical malignancy and confirming limitations of the current histopathological classification, as previously documented. Ultrasound screening failed identifying the ACC before metastasis in two cases, indicating an urgent need to develop new strategies for screening of ACTs in BWSp. Furthermore, some cases of metastatic ACC exhibited unexpectedly indolent behavior despite being malignant.

Minimally Invasive Image-Guided Percutaneous Irreversible Electroporation of Adrenal Metastases.

A single-center retrospective study was performed to evaluate the safety and efficacy of minimally invasive irreversible electroporation (IRE) to treat metastatic adrenal tumors. This single-center study, approved by the Institutional Review Board, retrospectively analyzed six patients who underwent image-guided percutaneous IRE for adrenal metastases. Pre-procedural imaging included CT, MRI and/or 18F-FDG PET-CT scans. Primary outcomes measures included technical success, efficacy and safety, while secondary outcome measures were local progression-free survival (LPFS), distant progression-free survival (DPFS) and overall survival (OS). Follow-up scans were scheduled post-procedure, and data analysis employed Excel, SPSS and R. Patients had diverse primary tumor origins including renal cell carcinoma (2/6), colorectal carcinoma (1/6), non-small cell lung carcinoma (1/6), leiomyosarcoma (1/6) and urothelial carcinoma (1/6). Adverse events were minimal, with only one grade 1 complication reported. Tumor characteristics revealed tumors close to critical structures, with a median pre-ablation size of 23mm. Technical success was achieved in all procedures. At first follow-up, one patient had complete response, one patient had partial response of the right adrenal gland and complete response of the left adrenal gland, two patients had partial response and two patients had stable disease. Local tumor progression occurred in two out of seven tumors with a median LPFS of 10.9months, and distant progression was observed in four out of six patients. Percutaneous, minimally invasive IRE shows promise as a safe treatment option for unresectable metastatic adrenal tumors, demonstrating potential effectiveness. However, further studies with larger patient cohorts are needed to confirm its safety and efficacy.

Adrenal Cortical Carcinoma Presenting as CSCR.

We describe a case of bilateral chronic central serous chorioretinopathy (CSCR) secondary to adrenal cortical carcinoma. Case report of a 70-year-old Hispanic man presenting with bilateral multifocal CSCR. Clinical findings of bilateral chronic CSCR along with 160 µm of subretinal fluid (SRF) and choroidal thickness greater than 400 µm without enhanced depth optical coherence tomography was noted in a patient presenting with distortion in vision in both eyes and weight gain of 15 pounds, weakness, and fatigue starting 8 months prior. Further endocrine testing showed an elevated 24-hour urinary free cortisol level of 137 µg with no change in serum cortisol levels following low-dose dexamethasone suppression test and undetectable serum adrenocorticotropic hormone (ACTH) levels consistent with ACTH-independent Cushing's syndrome. Imaging of the abdomen revealed heterogeneously enhancing masses, and biopsy of the adrenal tumor led to the diagnosis of adrenal cortical carcinoma. He died 3 weeks after starting hospice care, and only completed one round of chemotherapy. Adrenal tumors including adrenal cortical carcinoma may present as bilateral chronic central serous chorioretinopathy.

The Use of Near-Infrared Indocyanine Green Fluorescence Imaging to Differentiate Benign Versus Malignant Adrenal Tumors.

Despite advances in imaging modalities, a significant number of adrenal tumors are considered indeterminate and removed surgically. Currently, there is no intraoperative tool available to provide further information about the nature of indeterminate adrenal tumors. The aim of this study was to investigate whether near-infrared indocyanine green (ICG) imaging can be used in this regard. This was an institutional review board-approved study. Within 9 years, 197 patients underwent robotic adrenalectomy with ICG imaging. Adrenal tumors were characterized prospectively as fluorescent or nonfluorescent intraoperatively. ICG was administered as a 2.5 mg dose, one to three times a procedure. No patient developed an allergic reaction. Sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) were calculated. Robotic transabdominal lateral adrenalectomy was performed in 155 patients and posterior retroperitoneal adrenalectomy in 42 patients. One hundred and eighty-four patients had benign adrenal tumors, 10 patients had malignant tumors, and three patients had tumors of uncertain malignant potential. Sensitivity, specificity, PPV, and NPV to predict a benign tumor were 87%, 38.5%, 95.2%, and 17.2%. To our knowledge, this is the largest study on ICG imaging of adrenal tumors. High PPV to detect benign tumors suggests a utility of ICG to provide the surgeon with further information about the benign vs malignant nature of indeterminate adrenal tumors taken to surgery.

Perioperative management of patients with glioblastoma copresenting with pheochromocytoma: illustrative case.

Undiagnosed pheochromocytoma can present with hemodynamic instability during surgical procedures. Here, the authors discuss a 69-year-old male with isocitrate dehydrogenase (IDH)-wildtype glioblastoma copresenting with undiagnosed pheochromocytoma, which, to the authors' knowledge, is the second reported case in the literature. The patient presented to the emergency department with a 1-month history of coordination difficulties, progressive morning headache, and mild left-side weakness. Imaging showed a 5-cm peripherally enhancing intra-axial right parietal mass with surrounding vasogenic edema. Intraoperatively, the patient had significant uncontrollable hypertension up to 240/120 mm Hg, and the operation was promptly aborted. Contrast-enhanced computed tomography imaging of the chest, abdomen, and pelvis identified a 4.9-cm left adrenal mass of indeterminant etiology. Endocrinology diagnosed the incidentaloma as a pheochromocytoma, initiating alpha blockade followed by beta blockade, and the urology service performed a laparoscopic adrenalectomy after patient stabilization. The neurosurgery service removed the intra-axial brain lesion 2 days after adrenalectomy, which was diagnosed as IDH-wildtype glioblastoma. The patient was discharged home after 6 days in stable condition. This case highlights the importance of preoperative screening for pheochromocytoma in neurosurgical patients with adrenal incidentalomas, especially in incidentalomas > 4 cm, even without high clinical suspicion. https://thejns.org/doi/10.3171/CASE24374.

Partial adrenalectomy by the posterior retroperitoneoscopic approach: A single institution series of 766 consecutive procedures.

Partial adrenalectomy (PA), as an alternative to total adrenalectomy in selected cases, allows for the preservation of adrenocortical function, especially in hereditary and/or bilateral diseases. In this study, we analyze our experience in posterior retroperitoneoscopic PA (PRPA) and its impact on adrenocortical function. From January 2010 to December 2023, 709 PRPAs were performed on 766 adrenal glands in a total of 697 patients (421F; 276M; and mean age 49.3±15.3). Patients suffered from all types of adrenal tumors (239 pheochromocytoma, 225 Conn's syndrome, 102 Cushing's syndrome (CS), 66 nonfunctioning adenomas, 38 adrenal metastases, and 27 others). Mean tumor size was 2.7±1.5cm. Bilateral disease occurred in 135 patients (60 pheochromocytoma, 19 Conn's syndrome, 32 CS, 2 nonfunctioning adenomas, 17 adrenal metastases, and 5 combined diseases). Mean operating time for unilateral operation was 39.2±17.2min. Minor postoperative complications were observed in 1.5% and major complications in 0.1%. The median hospital stay was 2days. In bilateral cases, 18 of 33 (54%) Cushing patients and nine of 102 (9%) non-Cushing patients received corticosteroid replacement therapy (CRT) early postoperatively. Long-term follow-up data were collected for 242 patients (35%). After unilateral surgery, only patients with CS required CRT (8/24). Eleven (24.4%) out of 45 patients with bilateral non-Cushing disease needed CRT. Eight patients (3%) developed ipsilateral recurrent disease. PRPA is a safe procedure with a low complication and recurrence rate. This makes it the preferred operation in cases of bilateral disease and for selected patients with benign pathologies.

High specificity and sensitivity of spot urine normetanephrine-to-creatinine ratios in the diagnosis of canine pheochromocytoma.

To evaluate the specificity/sensitivity and predictive values of urine spot metanephrines-to-creatinine ratios to diagnose canine pheochromocytomas and to determine whether there is a correlation between urine catecholamine levels and tumor volume. In a retrospective study, 32 client-owned dogs with adrenal tumors were identified (13 nonpheochromocytoma and 19 pheochromocytoma). Medical records were evaluated for patients undergoing adrenalectomy between August 2020 and August 2023. Patients were divided into groupings of pheochromocytoma and other adrenal tumor diagnosis on the basis of histopathology. Urine spot normetanephrine concentrations were significantly elevated in dogs with pheochromocytomas compared to dogs with other adrenal tumors. Sensitivity was 78.9%, specificity 76.9%, positive predictive value 83.3%, and negative predictive value 71.4% for discriminating between pheochromocytoma and nonpheochromocytoma. Spot urine metanephrine concentrations were not significantly different between the 2 groups. Only 5 of 19 pheochromocytoma patients had urine normetanephrine concentrations > 4 times the upper reference limit, which is the previously established diagnostic cutoff. Median tumor volume did not correlate with normetanephrine values, nor did those values correlate to patients that experienced intraoperative complications. Twenty-one percent of cases were biochemically silent pheochromocytomas. Urine normetanephrine-to-creatinine levels but not metanephrine-to-creatinine levels can potentially differentiate a pheochromocytoma from a nonpheochromocytoma. Previously published guidelines of 4 times the upper reference limit in spot urine metanephrines testing used to diagnose pheochromocytomas will lead to a significant number of missed diagnoses.

High Diagnostic Accuracy of Arterial Phase CT in Differentiating Pheochromocytoma in Good/Poor Washout Adrenal Masses

Abstract Introduction Differentiating pheochromocytomas from other adrenal masses based on CT characteristics remains challenging, particularly in lipid-poor lesions with variable washout patterns. This study evaluated CT features for distinguishing pheochromocytomas in good and poor washout sub-cohorts. Methods We prospectively analyzed 72 patients with unilateral lipid-poor adrenal masses. CT protocol included basal, bolus-tracked arterial, early venous (45 seconds post-arterial), and delayed (15 minutes post-arterial) phases. Masses were categorized into good and poor washout groups. Histopathology provided the final diagnosis. CT characteristics and calculated indices were compared between pheochromocytomas and other masses in each washout category. Results The cohort included pheochromocytomas (n=35), adrenocortical carcinoma (ACC, n=15), lipid-poor adenomas (n=13), and metastatic infiltration/infection (n=9). In the good washout group, pheochromocytomas (n=15) showed larger diameters (6.00 vs. 3.45 cm, p=0.001), higher necrosis frequency (73.3% vs. 30%, p=0.049), and greater arterial attenuation (173.2 vs. 74.5 HU, p<0.001) compared to adenomas (n=9). Arterial attenuation provided a high discriminatory value (AUC: 0.967), with 100% sensitivity at 87.6 HU and 100% specificity at 139.9 HU. In the poor washout group, pheochromocytomas (n=20) exhibited higher arterial attenuation (99.2 vs. 59.2 HU, p<0.001; AUC: 0.844) compared to ACC (n=14), metastatic infiltration (n=9), and adenomas (n=4), with 85% sensitivity and 78% specificity at 73.3 HU. Normetanephrine-secreting/non-secretory pheochromocytomas showed higher arterial enhancement than metanephrine-secreting ones (132.0 vs. 90.5 HU, p=0.020) within the poor washout group. Conclusion Arterial phase attenuation on CT has good diagnostic accuracy for differentiating pheochromocytomas from other adrenal masses in both good and poor washout categories, potentially guiding hormonal workup.

Clinical significance of PET/CT in the diagnosis of primary adrenal malignancies

Background. According to the literature, the role of combined positron-emission and X-ray computed tomography (PET/CT) in diagnosing primary adrenal tumors (AT) remains limited due to both the frequency of these neoplasms and the availability of the method. Various research is required to assess the diagnostic resources of PET/CT with 18-fluorodeoxyglucose (18F-FDG). Aim. To evaluate the clinical role of PET/CT with 18F-FDG in the diagnosis of primary adrenal malignancies (PAM). Materials and methods. The study included 9 patients, 5 males and 4 females aged 44–76, with a median age of 59.3 years, with a morphologically confirmed diagnosis of the PAM. All patients underwent PET/CT with 18F-FDG. Visual and quantitative analysis of the obtained images was performed, including determination of the standardized maximum accumulation coefficient (SUVmax) in the tumor, liver, and spleen, the ratio of SUVmax of the primary tumor to SUVmax in the liver and spleen. Tumors were more often localized on the right (5/55.6%), and one case was bilateral. The maximum size of the adrenal mass averaged 6.8 cm (3.2–11.2) and the minimum size was 6.0 cm (2.3–9.0). The median SUVmax in AT was 10.0 (3.54–22.29), while in liver and spleen, it was 3.16 and 2.34, respectively, and the ratio of tumor SUVmax to liver and spleen SUVmax was 3.33 and 4.48, respectively. Conclusion. Hybrid PET/CT with 18F-FDG is a medical imaging method with a high diagnostic accuracy of PAM. PET/CT showed significant 18F-FDG tumor uptake, with a median SUVmax of 10.0 and a ratio of tumor SUVmax to liver and spleen SUVmax of 3.33 and 4.48, respectively. PET/CT with 18F-FDG may be a method of choice for diagnosing primary ATs.

Adrenocortical Carcinoma about One Case and Literature Review

Introduction: Adrenocortical carcinoma (ACC) is a rare malignant tumor that develops in the layers of the adrenal cortex with an incidence of 0.5–2 per million per year. Case report: A 40-year-old female patient, admitted for left flank heaviness, evaluation was in favour of a suspicious left adrenal tumour, the non-functional caracater was retained after a negative secretory workup. The patient was operated on with a histological appearance of metastatic ACC. Discussion and conclusion: The prognosis for these tumors is poor, since at the time of diagnosis the majority of cases are already metastatic, especially when the tumour is non-functional. The management of ACC is complex, representing a real diagnostic and therapeutic challenge.

Feasibility of extracellular volume fraction derived from single-phase spectral CT for differentiating between adrenal metastases and adenomas.

The extracellular matrix of adrenal metastases could be different from that of the adrenal adenomas, which may be characterized by the extracellular volume (ECV) fraction. This study aimed to assess the feasibility of ECV fraction derived from single-phase spectral CT for differentiating between adrenal metastases and adenomas. This retrospective study included 163 patients with unilateral nodules, including lipid-poor adrenal adenoma (n = 52, group A), lipid-rich adrenal adenoma (n = 65, group B) and adrenal metastasis (n = 46, group C). All patients underwent contrast-enhanced spectral CT of the adrenal gland with a 10-minute delayed phase. The iodine density of each lesion was assessed and ECV fraction was examined. Various parameters were compared among the three groups. Diagnostic performance was evaluated by quantifying the area under the receiver operating characteristic curve (AUC). The ECV fraction in group C (33.81 ± 11.94%) was significantly higher than that in group A (20.86 ± 10.22%) and group B (11.44 ± 6.26%) (p < 0.001), while iodine density in group C (0.93 ± 0.41mg/mL) was also higher than that in group A (0.61 ± 0.33mg/mL) and group B (0.33 ± 0.19mg/mL) (p < 0.001). ECV fraction showed significantly higher AUC (AUC = 0.790, 95%CI 0.691-0.869) for differentiating between group A and C than iodine density (AUC = 0.717, 95%CI 0.612-0.808; p = 0.002). Simultaneously, ECV fraction had significantly higher AUC (AUC = 0.945, 95%CI 0.858-0.986) for differentiating between group B and C than iodine density (AUC = 0.897, 95%CI 0.795-0.959, p = 0.03). Based on single 10-minute delayed phase spectral CT, ECV fraction showed high diagnostic performance in differentiating between adrenal metastases and adrenal adenomas, both for lipid-poor or lipid-rich adenomas. The ECV fraction could help recognize metastases in unilateral or bilateral adrenal masses for better treatment planning, especially in patients with a history of extra-adrenal malignancy.

Clinical and radiological features of atypical adrenal masses- a multicenter retrospective study.

The natural history and malignant potential of cases classified as atypical adrenal masses (AAMs) are unknown. To describe the radiological characteristics and clinical outcomes of AAMs. A multicenter retrospective study. Patients ≥18 years old with AAMs (diameter of 10-39 mm on first imaging study and pre-contrast attenuation of >10 Hounsfield units [HU] on computed tomography) were studied. We excluded adrenal metastasis, pheochromocytoma, sarcoma, lymphoma, infiltrative lesions, and adrenal hemorrhage, as well as patients with genetic predisposition to adrenal neoplasms. Data are presented as percentages and median values with interquartile ranges (IQRs). . We included 217 patients with 224 adrenal masses (61.3% women); the median age was 58 years (IQR, 49-65 years). The median size was 20.5 mm (IQR, 15-27 mm), with a median pre-contrast attenuation of 23.5 HU (IQR, 17-30 HU). The median AAM growth rate was 0.3 mm/year (IQR, 0-1.8 mm/year). Seventy-one masses (31.7%) underwent pathological evaluation. Adrenal adenoma (n=38; 17%) and adrenocortical carcinoma (ACC) (n=25; 11.2%) were the two most common diagnoses. For the adenomas, the growth rate was 0.3 mm/year (IQR, 0-2.3 mm/year) and for ACCs, the growth rate was 12.9 mm/year (IQR, 3.5-22 mm/year). The absolute contrast washout was >60% in 5 out of 7 (71.4%) ACC cases. The best growth rate cutoff for predicting malignancy was 2.68mm/year (AUC 0.939; sensitivity 87.5%, specificity 88.8%). AAMs carry significant malignant potential, and long-term follow-up is warranted when surgery is not pursued. Contrast washout is not reliable in predicting malignant potential of AAMs.

Gonadal changes in children and adolescents with congenital adrenal hyperplasia.

Testicular adrenal rest tumours (TARTs) are a common cause of infertility in males with congenital adrenal hyperplasia (CAH). Ovarian adrenal rest tumours (OARTs) and polycystic ovaries (PCO) can impair ovarian function in female patients with CAH. We aim to detect gonadal changes in children and adolescents with CAH. This study was conducted on 50 CAH patients (30 females and 20 males) with 21-hydroxylase deficiency (21-OHD), with a mean age of 10.35 ± 2.36 years. Testicular ultrasonography and pelvic magnetic resonance imaging (MRI) were done in males and females respectively. Glucocorticoid doses and biochemical data were obtained from the patients' medical records. TARTs were detected in 10/20 male patients (50 %). There was a significant relation between presence of TARTs, body mass index (BMI) standard deviation score (SDS), and bone age (p=0.017 and 0.023; respectively). There was no significant relation between presence of TARTs, laboratory parameters, or treatment received (p>0.05). Of those subjected to genetic analysis, 48 % had I2 splice (c.290-13A/C>G) followed by P30L (c.89C>T) (40.7 %). P30L (c.89C>T) was the most common allele among the patients with TARTs (42.9 %). There was no significant relation between presence of TARTs, the genotype, alleles, or the genotype groups (p>0.05). Only one female patient had radiological evidence of bilateral polycystic ovaries and none had OARTs. The prevalence of TARTs in our study was high (50 %). Screening for TARTs in males with CAH is crucial; however, routine ovarian imaging in CAH females is not indicated unless ovarian dysfunction is present.

Changes in WHO classification of adrenal tumors and new ideas for multi-dimensional diagnosis and treatment

In 2022, WHO updated the classification and concept of adrenal cortical and medullary tumors. In terms of adrenal cortical tumors, the WHO classification further standardizes the nomenclature of nodular adrenal cortical disease and refines the pathological classification of primary aldosteronism. In terms of adrenal medullary tumors, the WHO classification unifies the concepts of pheochromocytoma and paraganglioma, and reclassifies various concepts, including paraganglioma-like neuroendocrine tumors. The new standards not only cover the clinical manifestations of the disease, but also include other multiple aspects such as the histological origin of the disease, immunohistochemical manifestations, physiological mechanisms of the disease, hereditary susceptibility and prognostic factors. This article intends to explore how to improve the diagnostic and therapeutic level of adrenal tumors.

Bilateral Adrenal Tumors: A Visual Case Series

Adrenal incidentalomas are increasingly diagnosed with the widespread use of cross-sectional imaging modalities. Though these are often unilateral, bilateral adrenal masses uncommonly occur and can be of similar or different origins. While the majority of adrenal masses are benign, bilateral masses are more likely to be associated with hormonal disturbances. These range from syndromes of hormone excess, such as mild autonomous cortisol secretion, to primary adrenal insufficiency due to gland infiltration or destruction. Bilateral masses may herald the diagnosis of germline pathogenic variants, hereditary tumor predisposition syndromes, malignancy, or even infection. In many cases, imaging characteristics play an important role in making the diagnosis in addition to biochemical evaluation. Herein, we present various imaging findings of 10 patients with bilateral adrenal masses as evaluated with computed tomography, magnetic resonance imaging, and positron emission tomography scans.

Clinical and Genetic Mechanisms in Patients with MC2R Deficiency Presenting with Early Puberty.

Melanocortin receptor 2 (MC2R) in the adrenal cortex controls the hypothalamic-pituitary-adrenal axis. The melanocortin system, influenced by leptin, regulates GnRH neurons, crucial for puberty onset and fertility. This study evaluates early puberty in primary adrenal insufficiency (PAI) patients due to MC2R gene alterations. Seven patients with PAI (P1-P7) from five unrelated families, all presenting with early or precocious puberty, were included. MC2R deficiency diagnosis ranged from 1 day to 11 months. MKRN3, DLK1, KISS1, and KISS1R genes were analyzed using Sanger sequencing in four cases (P2,P4,P6 and P7). All clinical data were obtained retrospectively. Puberty onset mean age was 8.6 years (7.4-9.5) in boys (P1, P2, P3, P7) and 8.5 years (7.4-9.5) in girls (P4, P5, P6). Tumor markers were negative; no adrenal rest or tumors were found. GnRH analogs were used for rapid puberty in P2, P3, P6. Final height in P1 and P2 was below target (-2.6 SDS, -0.7 SDS). Menarche occurred at 11 and 11.3 years in P4 and P5. No pathogenic variants were found. Genetic causes of early puberty were not identified. Elevated ACTH may stimulate kisspeptin neurons, triggering puberty. Close monitoring of these patients for pubertal development is recommended.