A novel model using leukocytes to differentiating mild autonomous cortisol secretion and non-functioning adrenal adenoma.

BACKGROUND: Mild autonomous cortisol secretion (MACS) accounts for a significant proportion of adrenal incidentaloma. Current endocrinological screening tests for MACS are complex, particularly in areas with limited medical resources. This study aimed to develop a diagnostic tool based on leukocyte-related parameters to differentiate between MACS and non-functioning adrenal adenoma (NFA). METHODS: Inthis retrospective case-control study, propensity score-matching was used to select 567 patients from a cohort of 1108 patients (201 MACS, 907 NFA). External validation cohort included 52MACS and 48 NFA from two hospitals, which did not overlap with the modeling cohort patients. Leukocyte-related parameters were evaluated, and the diagnostic efficacy of each parameter was assessed by calculating Youden's J index (J) and the area under the curve (AUC). The study population was divided into training and testing samples using a 10-fold cross-validation method. Machine learning (ML) and classification and regression tree (CART) model were established. RESULTS: After propensity score matching, 567 patients were enrolled, including 197 MACS and 370 NFA. With the exception of basophil percentage, all other parameters differed significantly between the two groups. Lymphocyte count, lymphocyte percentage, eosinophils count, eosinophils percentage, and basophil percentage were lower in the MACS group compared to the NFA group. Eosinophils percentage demonstrated the highest AUC (0.650), with a sensitivity of 51.3% and specificity of 73.2%. The ML model, based on multiple parameters,exhibited better performance in diagnosing MACS (sensitivity 76%, specificity 77.4%, and AUC 0.818). A clinically usable CART model achieved an AUC of 0.872, with a sensitivity of 95% and a specificity of 75.7%.  In the validation cohort, the prediction accuracy of the ML model and the CART model were 0.784 and 0.798, respectively. CONCLUSION: TheCART diagnostic model, constructed based on leukocyte-related parameters, could assist clinicians in distinguishing between MACS and NFA.

Recent guidelines for diagnostic and therapeutic management of accidentally detected adrenal tumours (incidentaloma) in adults.

At the end of 2023, the European Journal of Endocrinology published the latest guidelines of the European Society of Endocrinology (ESE) on the management of accidentally detected adrenal tumours (incidentalomas) in adults. Comparing them with the previous version of the ESE recommendations from 2016, a tendency towards far-reaching individualisation of the therapeutic and diagnostic approach in patients with adrenal incidentaloma can be seen - it is reflected by changes in the initial assessment of the malignancy of the lesion, in the scope of the proposed hormonal assessment, and qualification for surgery. The latest version of the guidelines includes 9 completely new recommendations, and 5 more recommendations have been significantly changed. Among the most important changes, the introduction of the term "mild autonomous cortisol secretion" (MACS) instead of the previously used term "autonomous cortisol secretion (ACS)" along with more precise recommendations regarding its management should be emphasised. An important novelty is also the modification of the criteria for benign adrenal adenoma, which does not require further imaging observation - due to the results of recent clinical studies, the authors have removed the criteria of size below 4 cm from the definition. Among others, the guidelines also encourage more proactive surgical treatment of indeterminate adrenal incidentaloma in people < 40 years of age and pregnant women. The authors of the recommendations repeatedly accentuate the importance of a multidisciplinary approach in making decisions regarding further management of patients with an unspecified adrenal tumour. Despite a few significant differences compared to the previous version of guidelines, the authors emphasise the presence of gaps in the current scientific evidence, which would not allow for the formulation of more unambiguous recommendations. The need to optimise ordered diagnostic tests, which generate additional socio-economic burdens without negative impact on patients' health, is also an important aspect of the latest guidelines.

Complex Presentation of Pheochromocytoma: Hypertensive Encephalopathy and Takotsubo-Like Cardiomyopathy in a Young Female.

BACKGROUND Pheochromocytoma, a rare catecholamine-secreting tumor, often presents with paroxysmal or sustained hypertension, tachycardia, headache, and diaphoresis. Timely diagnosis is essential to prevent adverse complications. Less common presentations include pheochromocytoma crisis, with severe neurological and cardiac complications. CASE REPORT We report a unique case of a 25-year-old woman who initially presented with pheochromocytoma-induced hypertensive encephalopathy and acute coronary syndrome. Echocardiography revealed takotsubo-like cardiomyopathy, and magnetic resonance imaging of the brain revealed posterior reversible encephalopathy syndrome. Initial treatment focused on controlling her blood pressure and supporting cardiac function. Due to her recovering from immediate crisis and absence of further symptoms, the patient refused further follow-up. However, she eventually experienced another episode of hypertensive crisis 2 years later. Subsequent investigations with 24-h urine tests revealed elevated vanillylmandelic acid levels (7.93 mg/24 h), normetanephrine (2638.72 µg/24 h), and nor-metanephrine to creatinine ratio (3546.67) and normal urine metanephrine levels (195.92 µg/24 h) and metanephrine to creatinine ratio (263.33). Contrast-enhanced computed tomography of the abdomen revealed a 4.3×3.1×4-cm mass in the right adrenal gland. A DOTATATE positron emission tomography scan revealed a 3.9×4.3×2.7-cm localized right adrenal pheochromocytoma. Biochemical testing and adrenal imaging revealed a previously undiagnosed pheochromocytoma. Following targeted medical therapy and right adrenalectomy, the patient achieved complete resolution of her hypertension and associated symptoms. CONCLUSIONS Our case is a unique simultaneous presentation of posterior reversible encephalopathy syndrome and takotsubo-like cardiomyopathy, highlighting the importance to consider pheochromocytoma in acute neurological and cardiac presentations, even in the absence of typical symptoms.

Reference intervals for urinary metanephrines to creatinine ratios in dogs in Japan.

The urinary normetanephrine to creatinine ratio (uNMN/Cr) and urinary metanephrine to creatinine ratio (uMN/Cr) are commonly evaluated for the diagnosis of pheochromocytomas (PCC) in dogs. This study aimed to establish reference intervals for uNMN/Cr and uMN/Cr in 56 healthy dogs in Japan and to investigate the effect of urine collection methods on these measurements in 15 non-PCC dogs. The upper limits of reference intervals for uNMN/Cr and uMN/Cr were 124.4 nmol/mmol (90% confidence interval [CI] 107.7-137.0) and 121.1 nmol/mmol (90% CI 102.8-145.1), respectively. Both uNMN/Cr and uMN/Cr were significantly higher when urine was collected in the hospital compared to at home. Several factors, including the method of urine collection, should be considered when utilizing these reference intervals.

Paraganglioma-induced pheochromocytoma crisis successfully treated by veno-arterial extracorporeal membrane oxygenation: a case report.

Pheochromocytoma crisis is rare but potentially fatal if not recognized early and properly managed. Here, a woman in her 20s with a paraganglioma-induced pheochromocytoma crisis, who was successfully treated by veno-arterial extracorporeal membrane oxygenation (VA-ECMO) and interval tumor resection, is described. In July 2022, the patient was brought to hospital with a complaint of sudden-onset of palpitations with vomiting. The patient developed cardiorespiratory failure with hypoxia. Computed tomography scan showed pulmonary oedema and a mass anterior to the inferior vena cava. She was transferred to the intensive care unit and treated with VA-ECMO. The patient's ECMO was withdrawn after 6 days without any complications. After hemodynamic stabilization, the patient underwent tumor resection 4 months later. The postoperative course was uneventful and she was discharged on postoperative day 7. Histopathological analysis confirmed a paraganglioma. VA-ECMO may play a significant role in saving lives and providing time for accurate diagnosis and specific treatment of a patient with pheochromocytoma crisis. Appropriate individual management can help avoid the occurrence of ECMO complications.

Pheochromocytoma/Paraganglioma Syndrome Type 1 Presenting with Atypical Symptoms and a Novel Pathogenic Variant in the SDHD Gene: A Case Report.

This case report presents a 10-year-old patient diagnosed with pheochromocytoma/paraganglioma syndrome type 1 (PPGL1), underlined by a novel heterozygous pathogenic variant (c.154_161del, p.ser52Profster14) in the SDHD gene. Initially, the patient manifested symptoms unusual for pheochromocytoma, including polyuria and polydipsia; however, further diagnostic investigations revealed a pheochromocytoma (PCC) tumor in the adrenal gland. Subsequently, whole exome sequencing (WES) test identified a pathogenic frameshift variant in the SDHD gene, strongly suggestive of PPGL1. This study highlights the importance of considering atypical symptoms in diagnosing rare pediatric pheochromocytoma/paraganglioma tumors and underscores the value of genetic testing in identifying underlying genetic causes, thereby facilitating personalized management of the condition.

Unveiling Pheochromocytoma: A Puzzling Prelude of Nausea, Vomiting, and Abdominal Pain.

BACKGROUND Pheochromocytomas, rare tumors arising from the adrenal medulla, can present with highly variable symptoms; therefore, pheochromocytomas frequently remain undiagnosed, leaving the potential for physiological complications. Acutely, these complications include pheochromocytoma crisis, in which high levels of catecholamines are released and cause a life-threatening hypertensive emergency. Over time, undiagnosed pheochromocytomas can lead to cardiovascular damage and end-organ disease related to chronic exposure to elevated blood pressure. CASE REPORT We share a case of pheochromocytoma in a 45-year-old woman who presented with gastrointestinal symptoms of intractable nausea, vomiting, and abdominal pain. Imaging revealed an adrenal mass that had radiographic features that were most consistent with myelolipoma. Before exposing the patient to anesthesia and endoscopy for further diagnostic workup of her gastrointestinal symptoms, which can trigger a catecholamine surge in individuals with a pheochromocytoma, further biochemical testing was performed. Testing of plasma and urine confirmed pheochromocytoma, and surgical resection was performed for definitive treatment. Ultimately, the patient had resolution of her symptoms following the removal of the tumor. CONCLUSIONS The resolution of symptoms following surgical resection suggests that symptoms may have been related to the mass effect of the tumor or as an atypical manifestation of increased catecholamine levels. Additionally, by screening for pheochromocytoma, the patient was able to avoid potential complications that can result from common gastroenterological diagnostic procedures. This case report highlights the potential benefit for screening for pheochromocytoma when faced with symptoms that may be non-specific or related to mass effect upon surrounding organs.

Effectiveness of Robot-assisted Adrenalectomy for a Giant Adrenal Hemangioma: A Case Report.

BACKGROUND: Adrenal hemangiomas are extremely rare benign tumors that often need to be distinguished from malignancies. Adrenal tumors >4 cm in size are treated surgically because the possibility of malignancy cannot be ruled out. Traditionally, open surgery has been the mainstay of treatment; however, in recent years, robot-assisted surgery has been increasingly used for tumors of larger size and suspected malignancy. Here, we report a case of robot-assisted adrenalectomy for an 11 cm adrenal hemangioma. CASE REPORT: A 62-year-old male with lateral abdominal pain was referred to our hospital for further examination and treatment. His medical history was significant for hypertension, diabetes, and dyslipidemia. Computed tomography revealed an 11 cm left adrenal tumor, and all endocrinological screening tests were negative. Because the possibility of malignancy could not be ruled out, a robot-assisted adrenalectomy was performed. The operation time was 129 min, and the estimated blood loss was 7 ml. Pathological findings revealed an adrenal hemangioma. The postoperative course was uneventful, and patient's condition subsequently improved postoperatively. CONCLUSION: Robot-assisted adrenalectomy was performed for a giant adrenal hemangioma without any complications. Robotic surgery is useful for resecting adrenal hemangiomas even exceeding 11 cm in diameter.

Demographic and Clinical Characteristics of Patients with Adrenal Incidentaloma from a Single Center in South India: A Retrospective Study.

CONTEXT: Adrenal incidentalomas (AIs) are relatively uncommon neoplasms in 2% of apparently healthy individuals requiring evaluation for functionality and malignancy. AIM: We aimed to study the clinical, biochemical, and radiological profiles of patients presenting with AI and histopathological outcomes of those undergoing adrenalectomy. Materials and Methods: This retrospective study enrolled 62 AI patients attending a tertiary care center in South India between January 2016 and October 2023. Demographic details, radiological features, functionality, and histopathological data were analyzed. RESULTS: Out of 62 patients, total masses evaluated were 65 indicating bilaterality in 3 patients. The female: male ratio was 1.69, with a median age of 55 years (interquartile range: 44-64 years). 45.1% of individuals were >60 years. The most common indication for imaging was pain abdomen in 43 (69.4%). The median size was 3.2 cm. Fifty-five (88.7%) were assessed for functionality and 27 (49.1%) were functional. Among the 62 individuals, 14 (20.2%) had hypercortisolism, 11 (15.9%) had pheochromocytoma, 5 (7.24%) had primary hyperaldosteronism (PA), and 4 (5.7%) had hyperandrogenism including plurihormonal in 7. A mass size of 3.2 cm was of great value in distinguishing functional tumors with a sensitivity of 72% and specificity of 66% with an area under the curve of 0.682. A total of 34 (54.8%) patients underwent adrenalectomy. On histopathological examination, Adenoma (44.1%) was the most common followed pheochromocytoma (26.5%), adrenal cysts (8.8%), and Myelolipoma (5.9%). Two (5.9%) incidentalomas were adrenocortical carcinoma (ACC). Eight (53.3%) adenomas were functional with 6 having hypercortisolism (including 1 with hyperandrogenism) and 2 with PA. CONCLUSION: In our experience, the incidence of pheochromocytoma was second most common after adenoma. Since most functional tumors (60%) and all ACCs were ≥4 cm, a thorough biochemical evaluation for hormonal excess and evaluation for malignancy followed by surgery should be considered for lesions, especially ≥4 cm. Thus, we report the baseline demographic and clinical characteristics of patients with AI from a single center in South India.

Résumé Contexte:Les incidents surrénaliens (AIS) sont des néoplasmes relativement rares chez 2% des individus apparemment en bonne santé nécessitant une évaluation de la fonctionnalité et de la malignité. Objectif: Nous visions à étudier les profils cliniques, biochimiques et radiologiques des patients présentant IA et résultats histopathologiques de ceux qui subissent une surrénalectomie.Matériaux et méthodes:Cette étude rétrospective a inscrit 62 patients AI fréquentant un centre de soins tertiaires dans le sud de l'Inde entre janvier 2016 et octobre 2023. Les détails démographiques, les caractéristiques radiologiques, les fonctionnalités et les données histopathologiques ont été analysés.Résultats:Sur 62 patients, les masses totales évaluées étaient 65 indiquant la bilatéralité chez 3 patients. Le ratio féminin: mâle était de 1,69, avec un âge médian de 55 ans (intervalle interquartile: 44­64 ans). 45,1% des individus étaient> 60 ans. L'indication la plus courante pour l'imagerie était l'abdomen de la douleur dans 43 (69,4%). La taille médiane était de 3,2 cm. Cinquante-cinq (88,7%) ont été évaluées pour les fonctionnalités et 27 (49,1%) étaient fonctionnelles. Parmi les 62 individus, 14 (20,2%) avaient un hypercortisolisme, 11 (15,9%) Le phéochromocytome, 5 (7,24%) avait une hyperaldostéronisme primaire (PA), et 4 (5,7%) avaient l'hyperandrogénisme, y compris le plurihormonal dans 7. Une taille de masse de 3,2 cm était d'une grande valeur dans la distinction des tumeurs fonctionnelles avec une sensibilité de 72% et une spécificité distinctive de 66% avec une zone sous la courbe de 0,682. Au total, 34 (54,8%) patients ont subi une surrénalectomie. À l'examen histopathologique, l'adénome (44,1%) était le phéochromocytome suivi le plus courant (26,5%), les kystes surrénaliens (8,8%) et le myélolipome (5,9%). Deux incidentsalomes (5,9%) étaient un carcinome surrénocortical (ACC). Huit (53,3%) adénomes étaient fonctionnels avec 6 souffrant d'hypercortisolisme (dont 1 avec l'hyperandrogénisme) et 2 avec PA.Conclusion:D'après notre expérience, l'incidence du phéochromocytome était la deuxième plus courante après l'adénome. Étant donné que la plupart des tumeurs fonctionnelles (60%) et tous les ACC étaient ≥ 4 cm, une évaluation biochimique approfondie pour l'excès hormonal et l'évaluation de la malignité suivie d'une chirurgie doivent être envisagées pour les lésions, en particulier ≥4 cm. Ainsi, nous rapportons les caractéristiques démographiques et cliniques de base des patients atteints d'IA d'un seul centre du sud de l'Inde.

Acute onset of hypertensive encephalopathy in a dog with right adrenal pheochromocytoma and neoplastic invasion of the caudal vena cava: Case report and review of the literature.

Background: Canine pheochromocytomas (PCCs) are rare tumors of the adrenal medulla. Clinical signs are often vague, resulting in intermittent catecholamine over secretion or neoplastic invasion of adjacent structures. Case Description: A 12-year-old Epagneul Breton dog with a 1-year history of chronic kidney disease, was examined for acute onset of severe neurological signs. Based on clinical and instrumental data, hypertensive encephalopathy was suspected. Cardiac and abdominal ultrasound were performed. Severe hypertensive cardiopathy and a right adrenal gland mass with invasion of the caudal vena cava were diagnosed. Computed tomography imaging confirmed the suspect of invasive malignant neoplasia. Emergency pharmacological therapy was started to reduce systemic pressure, improve clinical signs, and stabilize the dog in view of surgical resolution. After initial improvement, patient conditions abruptly worsened, and euthanasia was elected. Histology examination confirmed a right adrenal PCC, with caval invasion. Conclusion: To the authors' conclusions, acute hypertensive encephalopathy is a peculiar manifestation of PCCs. Ultrasound is a useful, and rapid test to suspect PCC as it can detect adrenal alterations, caval invasion, metastasis, and cardiac sequelae consistent with the condition. PCC can mimic multiple affections, and be misinterpreted, especially when a concurrent disease has already been diagnosed. Veterinarians need to be aware that comorbidities could mask clinical signs and delay diagnosis. Furthermore, this clinical case reminds us to include PCC also in the differential diagnosis of dogs with an acute onset of severe neurological signs.

Predictors of malignancy for treatment direction in patients with nonfunctioning adrenal incidentaloma.

BACKGROUND: Adrenal incidentalomas (AI) are predominantly nonfunctional and benign, and their detection and differential diagnosis are aided by computed tomography (CT). A nonfunctioning adrenal incidentaloma (NFAI) usually requires regular follow-up; however, adrenalectomy may be necessary in certain patients. This study aimed to evaluate prognostic predictors to guide the treatment approach for AIs. METHODS: This retrospective, single-center study involved patients diagnosed with NFAI from January 2000 to December 2020. Patients were divided into surgery and observation groups. A subgroup analysis compared malignant and benign adenoma within the surgery group. RESULTS: A total of 307 patients were included, with 127 in the surgery group and 180 in the observation group. The surgery group displayed distinct morphological and malignant potential features in CT scans more frequently than the observational group did. The malignant subgroup exhibited more irregular borders on CT, and a higher number of patients with absolute washout under 60 % and relative washout under 40 % compared with the benign adenoma subgroup. Interestingly, within the surgery group, the mean tumor size was <4 cm for the both malignant and benign adenoma subgroups. CONCLUSIONS: Characterizing NFAI is important for appropriate treatment, as not all AIs have a favorable prognosis. CT findings associated with malignant potential, such as Hounsfield unit and washout values, were useful in determining the need for surgical treatment. However, the conventional criterion of a 4-cm size threshold for surgery was not a reliable malignancy predictor. Surgical resection should be considered for specific patient groups to ensure proper treatment over mere observation.

Clinical Review: The Approach to the Evaluation and Management of Bilateral Adrenal Masses.

OBJECTIVE: This white paper provides practical guidance for clinicians encountering bilateral adrenal masses. METHODS: A case-based approach to the evaluation and management of bilateral adrenal masses. Specific clinical scenarios presented here include cases of bilateral adrenal adenomas, hemorrhage, pheochromocytomas, metastatic disease, myelolipomas, as well as primary bilateral macronodular adrenal hyperplasia. RESULTS: Bilateral adrenal masses represent approximately 10% to 20% of incidentally discovered adrenal masses. The general approach to the evaluation and management of bilateral adrenal masses follows the same protocol as the evaluation of unilateral adrenal masses, determined based on the patient's clinical history and examination as well as the imaging characteristics of each lesion, whether the lesions could represent a malignancy, demonstrate hormone excess, or possibly represent a familial syndrome. Furthermore, there are features unique to bilateral adrenal masses that must be considered, including the differential diagnosis, the evaluation, and the management depending on the etiology. Therefore, considerations for the optimal imaging modality, treatment (medical vs surgical therapy), and surveillance are included. These recommendations were developed through careful examination of existing published studies as well as expert clinical opinion consensus. CONCLUSION: The evaluation and management of bilateral adrenal masses require a comprehensive systematic approach which includes the assessment and interpretation of the patient's clinical history, physical examination, dynamic hormone evaluation, and imaging modalities to determine the key radiographic features of each adrenal nodule. In addition, familial syndromes should be considered. Any final treatment options and approaches should always be considered individually.

Characteristics of Adrenal Hemorrhage: A Single Clinic's Experience.

<b>Introduction:</b> Adrenal hemorrhage (AH) is a very rare and potentially life-threatening disease which may be secondary to trauma or of non-traumatic etiology.<b>Aim:</b> The aim of the study was to present the characteristics and management of adrenal hemorrhage and show that adrenal hemorrhage is more common than expected and that the clinical symptoms are not specific.<b>Materials and methods:</b> This retrospective study involved 199 patients with postoperative diagnosis of adrenal hemorrhage.<b>Discussion:</b> The factors identified as potential causes of adrenal hemorrhage are adrenocortical carcinoma, pheochromocytoma, and adrenal adenoma. The study group included 199 patients with postoperative diagnosis of AH. It showed that all patients with postoperative diagnosis had pheochromocytoma (n = 54), adrenal adenoma (n = 68), or adenocarcinoma (n = 17). If we look more careful at the results, we can find only 30% of patients (n = 39) with preoperative diagnosis of AH. This group of 39 patients was prepared for expedited surgery. In this group of patients, the preoperative diagnosis of AH was pheochromocytoma 28% (n = 11), adenocarcinoma (n = 4), and adrenal adenoma (n = 9).<b>Conclusions:</b> Bleeding into adrenal tumors is still an insufficiently understood topic due to its unpredictability and, as can be seen in our material, of varying severity. Out of 199 patients, only 30% (n = 39) were prepared for surgery with a preoperative diagnosis of AH; most of them had pheochromocytoma. We suggest that is very important to prepare patients for surgery with a preoperative diagnosis of AH using α-adrenoreceptor antagonists. Prolongation of the diagnostic process (time between the imaging examination and the surgery) may result in the disease progressing and adrenal bleeding.

[Diagnosis and management of patients with pheochromocytoma/paraganglioma: Consensus of experts of the Russian Medical Society for Arterial Hypertension and the Multidisciplinary Group for the Diagnosis and Treatment of Neuroendocrine Tumors].

The understanding of the nature of catecholamine-secreting tumors has changed significantly in recent years, affecting terminology and classification. Phaeochromocytoma/paraganglioma (PCC/PG) is a rare neuroendocrine tumor from chromaffin tissue that produces and secretes catecholamines. The incidence of PCC/PG is relatively low, with 2-8 cases per 1 million population per year; among patients with arterial hypertension, their prevalence is 0.2-0.6%. However, delayed diagnosis of PCC/PG is associated with a high risk of cardiovascular complications and a high mortality rate. The consensus presents the clinical manifestations of the disease with an emphasis on the course of arterial hypertension as the most common symptom in PCC/PG; modern ideas about the features of diagnosis, aspects of preoperative preparation, treatment, and follow-up of patients with PCC/PG are considered.

Acute respiratory distress syndrome associated with a pheochromocytoma in an adult dog.

A 16-year-old castrated male shih tzu dog was brought to the emergency service because of an acute (24 h) history of gagging, coughing, and lethargy. Physical examination revealed dyspnea concurrent with diffuse, bilateral, pulmonary crackles and wheezes. Oxygen saturation, measured with a pulse oximeter, was < 95%. Thoracic radiographs revealed a multifocal alveolar pattern superimposed over a diffuse interstitial pattern with normal heart size. The dog continued to be dyspneic despite oxygen therapy. In accordance with the radiographic findings, further supportive care was recommended. Due to the unknown prognosis and financial constraints, the owner elected humane euthanasia. Necropsy confirmed a pheochromocytoma and lung changes without evidence of congestive heart failure. Findings were consistent with diffuse alveolar damage. These findings correlated with the dog's clinical diagnosis of acute respiratory distress syndrome (ARDS). No other disease processes associated with ARDS were identified. The purpose of this case report is to describe an unusual presentation of ARDS likely associated with a pheochromocytoma and confirmed by necropsy. Acute respiratory distress syndrome associated with a pheochromocytoma has been described in medical literature but has never been reported in veterinary medicine. Key clinical message: Pheochromocytomas should be added to the list of risk factors associated with ARDS in dogs. Dogs with a suspected diagnosis of pheochromocytoma whose owners elect against surgical removal should be closely monitored for an acute onset of respiratory distress, which could suggest the development of ARDS.

Syndrome de détresse respiratoire aiguë associé à un phéochromocytome chez un chien adulteUn chien shih tzu mâle castré de 16 ans a été amené aux services d'urgence en raison d'antécédents aigus (24 heures) de haut-le-coeur, de toux et de léthargie. L'examen physique a révélé une dyspnée concomitante à des crépitements et des respirations sifflantes diffuses, bilatérales et pulmonaires. La saturation en oxygène, mesurée avec un oxymètre de pouls, était < 95 %. Les radiographies thoraciques ont révélé un motif alvéolaire multifocal superposé à un motif interstitiel diffus avec une taille cardiaque normale. Le chien est resté dyspnéique malgré l'oxygénothérapie. Conformément aux résultats radiographiques, des soins de soutien supplémentaires ont été recommandés. En raison du pronostic inconnu et des contraintes financières, le propriétaire a opté pour l'euthanasie sans cruauté. La nécropsie a confirmé un phéochromocytome et des modifications pulmonaires sans signe d'insuffisance cardiaque congestive. Les résultats concordaient avec des lésions alvéolaires diffuses. Ces résultats étaient en corrélation avec le diagnostic clinique du syndrome de détresse respiratoire aiguë (ARDS) du chien. Aucun autre processus pathologique associé à l'ARDS n'a été identifié. Le but de ce rapport de cas est de décrire une présentation inhabituelle de l'ARDS probablement associée à un phéochromocytome et confirmée par la nécropsie. Le syndrome de détresse respiratoire aiguë associé à un phéochromocytome a été décrit dans la littérature médicale mais n'a jamais été rapporté en médecine vétérinaire.Message clinique clé:Les phéochromocytomes doivent être ajoutés à la liste des facteurs de risque associés à l'ARDS chez le chien. Les chiens avec un diagnostic suspecté de phéochromocytome dont les propriétaires choisissent de ne pas procéder à l'ablation chirurgicale doivent être étroitement surveillés pour détecter l'apparition aiguë d'une détresse respiratoire, ce qui pourrait suggérer le développement d'un ARDS.(Traduit par Dr Serge Messier).

A rare association of pheochromocytoma, paraganglioma, and pituitary adenoma (3PA): A case report and literature review.

RATIONALE: 3P association (3PA) is a rare condition with co-occurrence of pituitary adenoma and pheochromocytoma/paraganglioma. There have been less than a hundred documented cases of 3PA, which can be sporadic or related to genetic mutations. The present case report describes the first Iranian patient with 3PA and a 90th case of 3PA in the available literature. PATIENT CONCERNS AND INTERVENTIONS: A 36-year-old Caucasian male was admitted with headache and sudden increase in blood pressure. An abdominal CT scan revealed a retroperitoneal mass posterior to the inferior vena cava, later removed and diagnosed as a pheochromocytoma. Four years later, he noticed occasional mild headaches and a painless mass on the right side of his neck. The ultrasonography evaluations suggested a carotid body tumor, which was surgically removed. About a month after his second surgery, the severity of the patient's headaches worsened, and he developed right homonymous hemianopia. A brain MRI showed a mass in favor of macroadenoma, craniopharyngioma, or meningioma, and elevated prolactin level led to the diagnosis of macroprolactinoma. DIAGNOSES: Based on the provided history, this patient was diagnosed with 3PA, and a genetic study identified a positive succinate-dehydrogenase-complex subunit b mutation, possibly linked to his family history of carotid body tumor. OUTCOMES: He has remained symptom-free during his visits every 3 months. LESSONS: The number of cases diagnosed with 3PA worldwide is increasing. Using clinical and genetic assessments, we can timely diagnose and adequately monitor individuals with or at risk of 3PA.

[The value of serum dehydroepiandrosterone sulfate in the functional evaluation of adrenal space-occupying lesions in adults].

Objective: To evaluate the function of serum dehydroepiandrosterone sulfate (DHEAS) in adult adrenal space-occupying lesions. Methods: In this cross-sectional study, 395 patients with adrenal space-occupying lesions who had their DHEAS levels measured were collected from the First Medical Center of Chinese PLA General Hospital from January 2010 to June 2021. They were divided into the adrenal Cushing syndrome (ACS) group (n=100) and non-ACS group (n=295). The former was divided into the cortisol-producing adrenal adenoma (CPA) group (n=67) and primary bilateral macronodular adrenal hyperplasia (PBMAH) group (n=33). Clinical data of each group were collected and compared among groups by independent samples t-test, chi-square test, and Mann-Whitney U test. Serum DHEAS ratio corrected for age and sex was further constructed to identify the receiver operating characteristic curve and the optimal tangent point value for different adrenal occupation. Results: Patients in the ACS group were younger (44.9±13.7 vs. 49.9±12.5, P=0.001); had a larger proportion of women (79/100 vs.139/295, P=0.001); and had higher cortisol levels [8∶00Am, 497.31 (343.52, 606.50) vs. 353.11 (267.50, 487.91) nmol/L, P<0.001] than those in the non-ACS group. The serum DHEAS level and ratio in the ACS group were significantly lower than those in the non-ACS group [0.50 (0.40, 1.21) vs. 2.68 (1.56, 4.32) µmol/L, 1.00 (0.43, 1.68) vs. 3.17 (2.21, 4.54), both P<0.001]. When the serum DHEAS ratio cut-off point was 1.29, the sensitivity and specificity for differential diagnosis of ACS and non-ACS were 72.0% and 91.5% respectively. The ratio of DHEAS in the CPA group was lower [0.58 (0.27, 1.05) vs. 1.14 (1.04, 2.40), P<0.001] than that in the PBMAH group. When the serum DHEAS ratio cut-off point was 0.99, the sensitivity and specificity for differential diagnosis of CPA and PBMAH were 64.2% and 81.2% respectively. Conclusion: Corrected age-sex DHEAS ratio can assist in the functional assessment of adrenal space-occupying lesions.

Germline Mutations and Phenotypic Associations in Korean Patients With Pheochromocytoma and Paraganglioma: A Multicenter Study and Literature Review.

Genetic testing is recommended for all patients with pheochromocytomas and paragangliomas (PPGL) to establish genotype-phenotype associations. We investigated germline mutations in 59 patients with PPGL at six Korean university hospitals using next-generation sequencing (NGS) targeting 38 PPGL-associated genes, including those recommended by the Korean PPGL Task Force. Germline mutations were identified in 13 patients (22%), and affected four genes: RET, NF1, VHL, and SDHD. Germline mutations were significantly associated with a family history of PPGL, smaller tumor size, and the presence of other types of tumors. Using 95 Korean PPGL cases with germline mutations identified through a literature review and 13 cases from our cohort, we characterized genotype-phenotype correlations. Mutation hotspots were identified in specific codons of RET (codons 631 and 634), VHL (157 and 167), and SDHB (131 and 253). NF1 mutations varied, indicating the absence of common hotspots. These findings highlight the efficacy of the recommended NGS panel for Korean patients with PPGL and the importance of genetic testing in establishing clinical management and personalized therapeutic strategies.

Management of a giant adrenal schwannoma.

In this case report, we present a man in his 60s who presented with an incidentally discovered right adrenal mass, which turned out to be an adrenal schwannoma. This is a very rare tumour that originates from Schwann cells and involves the peripheral nerves. The tumour was removed by open adrenalectomy, and this 15-cm adrenal schwannoma is one of the largest reported in the literature, with none >16 cm having ever been reported. This case highlights the importance of keeping an open mind about the cause of an incidentally discovered adrenal mass, which is an increasingly common way for adrenal tumours to present given the increased access to cross-sectional imaging. As well as presenting the case and the pathological basis behind adrenal schwannomas, we include a review of the literature and a general discussion about incidentally discovered adrenal masses.