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1.
Front Endocrinol (Lausanne) ; 13: 816476, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35757409

RESUMO

Background: Somatic mutations for excess aldosterone production have been frequently identified as important roles in the pathogenesis of unilateral primary hyperaldosteronism (uPA). Although CACNA1H mutation represents a minor etiology in primary aldosteronism, it plays a significant role in causing uPAs in sporadic cases. Objective: To identify novel somatic CACNA1H mutation in patients with uPA and investigate the pathophysiological, immunohistological, and clinical characteristics of the variant. Methods: We applied a customized and targeted gene panel next-generation sequencing approach to detect mutations from the uPA cohort in Taiwan Primary Aldosteronism Investigation study group. Information from pre-diagnostic to postoperative data was collected, including past history, medications, blood pressure readings, biochemical data, and image studies. The functional role of the variant was confirmed by in vitro studies, demonstrating aldosterone production in variant-transfected human adrenal cell lines. Results: We identified a novel somatic CACNA1H mutation c.5809G>A (p.Val1937Met) in a uPA case. The CACNA1H gene encodes the pore-forming alpha-1H subunit of the voltage-dependent T-type calcium channel Cav3.2. This somatic CACNA1H p.V1937M variant showed excellent clinical and biochemical outcomes after ipsilateral adrenalectomy. The functional effect of somatic CACNA1H p.V1937M variant results in increased CYP11B2 expression and aldosterone biosynthesis in HAC15 cells. A distinct heterogeneous foamy pattern of CYP11B2 and CYP17A1 expression was identified in immunohistological staining, supporting the pathological evidence of aldosterone synthesis. Conclusions: The somatic mutation of CACNA1H p.V1937M might be a pathogenic driver in aldosterone overproduction. This study provides new insight into the molecular mechanism and disease outcomes of uPA.


Assuntos
Canais de Cálcio Tipo T , Hiperaldosteronismo , Adrenalectomia/efeitos adversos , Aldosterona/metabolismo , Canais de Cálcio Tipo T/genética , Canais de Cálcio Tipo T/metabolismo , Citocromo P-450 CYP11B2/genética , Citocromo P-450 CYP11B2/metabolismo , Humanos , Hiperaldosteronismo/genética , Hiperaldosteronismo/metabolismo , Mutação
2.
Front Endocrinol (Lausanne) ; 13: 898084, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35721734

RESUMO

Objective: The best approach to patients with adrenal incidentaloma (AI) and possible autonomous cortisol secretion (PACS) is debated. The aim of this study was to assess the metabolic effect of adrenalectomy in AI patients with PACS in relation to cortisol secretion parameters, peripheral activation, and glucocorticoid sensitivity. Design: This is a multicenter randomized study (NCT number: NCT04860180). Methods: Sixty-two AI outpatients (40-75 years) with AI >1 cm and cortisol after overnight dexamethasone suppression test (F-1mgDST) between 50 and 138 nmol/L were randomized to adrenalectomy (Arm A) or a conservative approach (Arm B). Fifty-five patients completed the 6-month follow-up, 25 patients in Arm A (17 female patients, aged 62.5 ± 10.4 years) and 30 patients in Arm B (24 female patients, 66.1 ± 9.1 years). Plasma adrenocorticotroph hormone (ACTH), 24-h urinary free cortisol, 24-h urinary free cortisone, F-1mgDST, glucose, lipids, glycated hemoglobin (HbA1c) levels, blood pressure (BP), body weight, and treatment variations were assessed. The 24-h urinary free cortisol/cortisone ratio (an 11-beta hydroxysteroid dehydrogenase type 2 activity marker), BclI, and the N363S variants of glucocorticoid receptor (GR) polymorphisms were also evaluated. Results: BP control improved in 68% and 13% of the subjects in Arm A and Arm B, respectively (p = 0.001), and the glycometabolic control improved in 28% and 3.3% of the subjects in Arm A and Arm B patients, respectively (p = 0.02). Arm A subjects more rarely showed the BP and/or glycometabolic control worsening than Arm B patients (12% and 40%, respectively, p = 0.03). The surgical approach was independently associated with BP amelioration (OR 3.0, 95% CI 3.8-108.3, p < 0.001) but not with age, F-1mgDST levels, BMI, and hypertension and diabetes mellitus presence at baseline. The 24-h urinary free cortisol/cortisone ratio and the presence of sensitizing GR polymorphisms were not associated with the surgical outcome. The receiver operating characteristic (ROC) curve analysis showed that the BP control amelioration was associated with F-1mgDST [area under the curve (AUC), 0.82 ± 0.09 p = 0.012]. The F-1mgDST cutoff with the best compromise in predicting the BP amelioration was set at 75 nmol/L (sensitivity 77%, specificity 75%). Conclusions: AI patients with PACS benefit from surgery in terms of BP and glycometabolic control.


Assuntos
Adrenalectomia , Cortisona , Neoplasias das Glândulas Suprarrenais , Pressão Sanguínea , Feminino , Humanos , Hidrocortisona
3.
Horm Metab Res ; 54(6): 354-360, 2022 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-35697044

RESUMO

Aim was to explore the associations between baseline cortisol levels and surgery method of primary bilateral macronodular adrenal hyperplasia (PBMAH). We retrospectively reviewed the clinical features and management of 30 patients (18 females and 12 males) who were diagnosed with PBMAH in our center between 2005 and 2019. Based on surgery method, we divided the patients into two groups: unilateral adrenalectomy (UA) group; and bilateral adrenalectomy (BA) group. Serum cortisol rhythm and 24-hour urinary free cortisol (UFC/24 h) levels were assayed using chemiluminescence method. Associations between baseline cortisol levels and BA were assessed using logistic regression. The predictive value of baseline cortisol levels for BA was calculated using receiver operating characteristic (ROC) curves. Twenty patients (66.7%) underwent UAs and ten patients (33.3%) underwent BAs. After adjusting for age, sex, BMI, SBP, and adrenal volume, the concentrations of baseline serum cortisol (8 AM, 4 PM, and 0 AM) and UFC/24 h were associated with bilateral adrenalectomy (all p<0.05). The area under the ROC curve based on 8 AM serum cortisol level model was larger than that in models based on 4 PM, 0 AM serum cortisol levels and UFC/24 h, but the differences were non-significant (all p>0.05). According to maximum Youden index criteria, the optimal cutoffs of 8 AM serum cortisol level and UFC were 26.89 µg/dl and 406.65 µg/24 h, respectively, for BA. The baseline cortisol levels are positively associated with BA. Increased levels of baseline cortisol levels may predict higher possibility of BA, which should be confirmed by prospective studies.


Assuntos
Adrenalectomia , Hidrocortisona , Feminino , Humanos , Hiperplasia , Masculino , Estudos Prospectivos , Estudos Retrospectivos
4.
Sci Rep ; 12(1): 8892, 2022 May 25.
Artigo em Inglês | MEDLINE | ID: mdl-35614110

RESUMO

We performed the present study to investigate the role of computed tomography (CT) radiomics in differentiating nonfunctional adenoma and aldosterone-producing adenoma (APA) and outcome prediction in patients with clinically suspected primary aldosteronism (PA). This study included 60 patients diagnosed with essential hypertension (EH) with nonfunctional adenoma on CT and 91 patients with unilateral surgically proven APA. Each whole nodule on unenhanced and venous phase CT images was segmented manually and randomly split into training and test sets at a ratio of 8:2. Radiomic models for nodule discrimination and outcome prediction of APA after adrenalectomy were established separately using the training set by least absolute shrinkage and selection operator (LASSO) logistic regression, and the performance was evaluated on test sets. The model can differentiate adrenal nodules in EH and PA with a sensitivity, specificity, and accuracy of 83.3%, 78.9% and 80.6% (AUC = 0.91 [0.72, 0.97]) in unenhanced CT and 81.2%, 100% and 87.5% (AUC = 0.98 [0.77, 1.00]) in venous phase CT, respectively. In the outcome after adrenalectomy, the models showed a favorable ability to predict biochemical success (Unenhanced/venous CT: AUC = 0.67 [0.52, 0.79]/0.62 [0.46, 0.76]) and clinical success (Unenhanced/venous CT: AUC = 0.59 [0.47, 0.70]/0.64 [0.51, 0.74]). The results showed that CT-based radiomic models hold promise to discriminate APA and nonfunctional adenoma when an adrenal incidentaloma was detected on CT images of hypertensive patients in clinical practice, while the role of radiomic analysis in outcome prediction after adrenalectomy needs further investigation.


Assuntos
Adenoma , Hiperaldosteronismo , Adenoma/diagnóstico por imagem , Adenoma/cirurgia , Adrenalectomia , Aldosterona , Hipertensão Essencial/diagnóstico por imagem , Humanos , Hiperaldosteronismo/diagnóstico por imagem , Hiperaldosteronismo/cirurgia , Estudos Retrospectivos
5.
Horm Metab Res ; 54(5): 294-299, 2022 May.
Artigo em Inglês | MEDLINE | ID: mdl-35533674

RESUMO

Work up of adrenal masses includes assessment of endocrine activity and malignancy risk. There is no indication for surgical removal of nonfunctional adrenal adenomas, according to the guidelines. In the present study, we aimed at evaluating the impact of a university endocrine tumor board on the quality of the indications for adrenal surgery at our institution. One hundred consecutive patients receiving primary adrenal surgery at the University Hospital of Cologne, Germany were included. Their demographics, clinic-pathologic characteristics, treatment and outcome were analyzed. In 55 (55%) cases, indication for surgery consisted in functional benign tumors, including Conn, Cushing adenomas and pheochromocytomas. Forty (40%) tumors were referred to surgery for malignancy suspicion and 5 (5%) myelolipomas were removed due to their size. Eighty-nine percent of surgeries were performed as minimally invasive procedures. Overall morbidity included two (2%) self-limiting pancreatic fistulas after left laparoscopic adrenalectomy for pheochromocytoma. All functional tumors were confirmed benign by final histology. Only 33 (82.5%) of 40 suspicious cases turned out to be malignant. Consequently, nonfunctional benign adenomas were "unnecessarily" removed in only 7 (7%) patients, with 6 (85.7%) of them having a history of extra-adrenal cancer and all of them fulfilling criteria for surgery, according to the international guidelines. In conclusion, the endocrine tumor board provided an excellent adherence to the guidelines with most surgeries being performed either for functional or malignant tumors. In nonfunctional tumors with history of extra adrenal cancer, CT guided biopsy might be considered for obviating surgery.


Assuntos
Adenoma , Neoplasias das Glândulas Suprarrenais , Laparoscopia , Feocromocitoma , Adenoma/patologia , Neoplasias das Glândulas Suprarrenais/patologia , Neoplasias das Glândulas Suprarrenais/cirurgia , Adrenalectomia , Humanos , Laparoscopia/métodos , Feocromocitoma/patologia , Feocromocitoma/cirurgia
6.
Croat Med J ; 63(2): 197-201, 2022 Apr 30.
Artigo em Inglês | MEDLINE | ID: mdl-35505653

RESUMO

We present a case series of 12 consecutive robot-assisted adrenalectomies performed from May 2019 to March 2020 by a single surgeon experienced in laparoscopy using the novel Senhance robotic system. Eleven patients had primary aldosteronism due to an adrenal adenoma, diagnosed by means of endocrinological and radiological evaluation, and 1 had a benign adrenal cyst. The robotic adrenalectomy technique is described in detail. The mean procedure time was 165.1 minutes, with robotic docking time of 11.6 minutes and console time of 98.6 minutes. The mean estimated blood loss was 47 mL, and hospital stay duration was 4.5 days. There was 1 Clavien Dindo IIIB complication and 1 patient underwent conversion to laparoscopy. All patients with adenoma had complete biochemical remission after surgery. In conclusion, the Senhance robotic system is a safe and feasible platform for benign adrenal surgery in high-volume centers.


Assuntos
Laparoscopia , Procedimentos Cirúrgicos Robóticos , Robótica , Adrenalectomia/métodos , Humanos , Laparoscopia/métodos , Pesquisa , Procedimentos Cirúrgicos Robóticos/métodos
7.
J Coll Physicians Surg Pak ; 32(5): 565-569, 2022 May.
Artigo em Inglês | MEDLINE | ID: mdl-35546688

RESUMO

OBJECTIVE: To compare perioperative outcomes of minimally invasive surgery for ≥5 cm and <5 cm adrenal lesions. STUDY DESIGN: Retrospective cohort study. PLACE AND DURATION OF STUDY: Hacettepe University School of Medicine, Ankara, Turkey, between October 2007 and September 2019. METHODOLOGY: Data of 83 patients operated for adrenal lesions was collected retrospectively. Patients were categorized into two groups based on the size of the adrenal gland as <5 cm and ≥5 cm. The groups were compared in terms of perioperative outcomes. RESULTS: The median age of the patients was 51 (41-60) years, with a female-to-male ratio of 27/56. The median follow-up period was 27 (11.5-91) months. Of 83 adrenal masses, 60 (72.3%) were in the <5 cm group and 23 (27.7%) were in the ≥5 cm group. Fifteen (18.1%) patients underwent adrenalectomy for lung cancer metastasis, whereas three (3.6%) for renal cell carcinoma metastasis. The overall rate of post-operative complications was 10.8%. Post-operative complication rates were similar in each group (p=0.433). Operation time was found to be significantly higher in patients with large adrenal masses (p=0.003). CONCLUSION: Minimally invasive surgical techniques have the same perioperative results in the group with adrenal lesions ≥5 cm compared to <5 cm and may be safely employed in this group of patients. KEY WORDS: Laparoscopy, Robotic assisted laparoscopy, Adrenalectomy, Metastasectomy, Adrenal gland neoplasms.


Assuntos
Neoplasias das Glândulas Suprarrenais , Laparoscopia , Neoplasias das Glândulas Suprarrenais/complicações , Neoplasias das Glândulas Suprarrenais/cirurgia , Adrenalectomia/efeitos adversos , Adrenalectomia/métodos , Feminino , Humanos , Laparoscopia/efeitos adversos , Laparoscopia/métodos , Masculino , Pessoa de Meia-Idade , Procedimentos Cirúrgicos Minimamente Invasivos/efeitos adversos , Procedimentos Cirúrgicos Minimamente Invasivos/métodos , Complicações Pós-Operatórias/etiologia , Estudos Retrospectivos
8.
BMC Anesthesiol ; 22(1): 153, 2022 May 19.
Artigo em Inglês | MEDLINE | ID: mdl-35590236

RESUMO

BACKGROUND: Minimally invasive adrenalectomy is the standard of care for small adrenal tumours. Both the transperitoneal lateral approach and posterior retroperitoneal approach are widely used and have been proven to be safe and effective. However, the prevalence of chronic postsurgical pain has not been specifically investigated in previous studies. The primary goal of this study was to identify the prevalence of chronic postsurgical pain after minimally invasive adrenalectomy. METHODS: A cross-sectional study was performed among all consecutive patients who had undergone minimally invasive adrenalectomy in a single university medical centre. The primary outcome was the prevalence of chronic postsurgical pain. Secondary outcomes were the prevalence of localized hypoesthesia, risk factors for the development of chronic postsurgical pain, and the Health-Related Quality of Life. Three questionnaires were used to measure the prevalence and severity of chronic postsurgical pain, hypoesthesia, and Health-Related Quality of Life. Logistic regression analysis was performed to determine risk factors for development of chronic postsurgical pain. RESULTS: Six hundred two patients underwent minimally invasive adrenalectomy between January 2007 and September 2019, of whom 328 signed informed consent. The prevalence of chronic postsurgical pain was 14.9%. In the group of patients with chronic postsurgical pain, 33% reported hypoesthesia as well. Young age was a significant predictor for developing chronic postsurgical pain. The prevalence of localized hypoesthesia was 15.2%. In patients with chronic postsurgical pain, Health-Related Quality of Life was significantly lower, compared to patients without pain. CONCLUSIONS: The prevalence of chronic postsurgical pain following minimally invasive adrenalectomy is considerable. Furthermore, the presence of chronic postsurgical pain was correlated with a significant and clinically relevant lower Health-Related Quality of Life. These findings should be included in the preoperative counselling of the patient. In the absence of evidence for effective treatment in established chronic pain, prevention should be the key strategy and topic of future research.


Assuntos
Neoplasias das Glândulas Suprarrenais , Laparoscopia , Neoplasias das Glândulas Suprarrenais/cirurgia , Adrenalectomia/efeitos adversos , Estudos Transversais , Humanos , Hipestesia/etiologia , Hipestesia/cirurgia , Laparoscopia/efeitos adversos , Dor Pós-Operatória/epidemiologia , Dor Pós-Operatória/etiologia , Prevalência , Qualidade de Vida
9.
Front Endocrinol (Lausanne) ; 13: 861581, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35557845

RESUMO

Primary aldosteronism is the most common and modifiable form of secondary hypertension. Left untreated, primary aldosteronism leads high rates of cardiovascular, metabolic, and kidney disease. Therefore, early diagnosis and targeted therapy are crucial to improve long-term patient outcomes. In the case of unilateral primary aldosteronism, surgical adrenalectomy is the guideline-recommended treatment of choice as compared to alternative medical therapies such as mineralocorticoid receptor antagonist medications. Surgical adrenalectomy is not only highly successful in reversing the biochemical abnormalities inherent to primary aldosteronism, but also in mitigating the long-term risks associated with this disease. Indeed, as opposed to medical treatment alone, surgical adrenalectomy offers the potential for disease cure. Within this review article, we review the existing evidence highlighting the benefits of surgical over medical treatment for unilateral primary aldosteronism.


Assuntos
Hiperaldosteronismo , Hipertensão , Nefropatias , Adrenalectomia , Feminino , Humanos , Hiperaldosteronismo/complicações , Hiperaldosteronismo/tratamento farmacológico , Hiperaldosteronismo/cirurgia , Hipertensão/complicações , Masculino , Antagonistas de Receptores de Mineralocorticoides/uso terapêutico
10.
Actas urol. esp ; 46(4): 245-251, mayo 2022. ilus, tab
Artigo em Espanhol | IBECS | ID: ibc-203613

RESUMO

Introducción y objetivos: La cirugía laparoscópica se ha convertido en el abordaje estándar para el tratamiento de las glándulas suprarrenales. Debido a que la adrenalectomía bilateral sincrónica no se realiza con frecuencia, la evidencia sobre este procedimiento es limitada. Nuestro objetivo es reportar nuestra experiencia de 13 años con la adrenalectomía bilateral laparoscópica sincrónica, evaluando su viabilidad, seguridad y resultados perioperatorios.Pacientes y métodos: Un total de 23 pacientes consecutivos sometidos a adrenalectomía laparoscópica bilateral sincrónica entre 2007 y 2020 en un único centro académico fueron incluidos en el estudio. Las variables evaluadas fueron el tiempo quirúrgico, la pérdida media estimada de sangre, la conversión a cirugía abierta, las complicaciones postoperatorias, la mortalidad y la duración de la estancia postoperatoria.Resultados: El tiempo operatorio medio fue de 189,3±48,9min. La media de pérdida de sangre estimada fue de 163,0±201,3ml. No hubo conversiones a cirugía abierta. Cinco pacientes tuvieron complicaciones postoperatorias y 3 de estas fueron graves. Ningún paciente falleció durante el periodo perioperatorio. La mediana del tiempo de estancia postoperatoria fue de 3 días (rango 1-30). En el análisis patológico 15 pacientes tenían hiperplasia suprarrenal bilateral, 2 hiperplasia suprarrenal unilateral y un tumor benigno contralateral, uno hiperplasia suprarrenal unilateral y glándula contralateral normal, otro adenoma unilateral, 3 feocromocitomas bilaterales y uno mielolipoma bilateral.Conclusión: La adrenalectomía laparoscópica bilateral sincrónica es una técnica factible y segura. Se requiere un equipo multidisciplinar y experimentado que incluya anestesistas y endocrinólogos. (AU)


Introduction and objectives: Laparoscopic surgery is the standard approach for the treatment of adrenal glands. Bilateral synchronous adrenalectomy is rarely performed, and evidence about this procedure is limited. Our objective is to report our 13-year experience with synchronous laparoscopic bilateral adrenalectomy, evaluating its feasibility, safety, and perioperative outcomes.Patients and methods: A total of 23 consecutive patients undergoing synchronous bilateral laparoscopic adrenalectomy between 2007 and 2020 in a single academic center were included. Variables evaluated were operative time, estimated blood loss, conversion to open surgery, postoperative complications, mortality, and postoperative length of stay.Results: Mean operative time was 189.3±48.9min. Mean estimated blood loss was 163.0±201.3ml. There were no conversions to open surgery. Five patients had postoperative complications, three of those were major. No patient died in the perioperative period. Median postoperative length of stay was three days (range 1-30). At pathology analysis, 15 patients had bilateral adrenal hyperplasia, 2 unilateral adrenal hyperplasia and a contralateral benign tumor, 1 unilateral adrenal hyperplasia and a normal contralateral gland, 1 unilateral adenoma, 3 bilateral pheochromocytomas and 1 bilateral myelolipoma.Conclusion: Synchronous bilateral laparoscopic adrenalectomy is a feasible and safe technique. A multidisciplinary and experienced team involving anesthesiologists and endocrinologists is required. (AU)


Assuntos
Humanos , Masculino , Feminino , Adolescente , Adulto Jovem , Adulto , Pessoa de Meia-Idade , Idoso , Idoso de 80 Anos ou mais , Neoplasias das Glândulas Suprarrenais/cirurgia , Adrenalectomia/métodos , Laparoscopia/métodos , Adrenalectomia/efeitos adversos , Complicações Pós-Operatórias , Resultado do Tratamento
11.
Am J Case Rep ; 23: e936138, 2022 May 28.
Artigo em Inglês | MEDLINE | ID: mdl-35633028

RESUMO

BACKGROUND Ganglioneuromas are differentiated tumors originating from the neural crest. Although their occurrence is rare, they usually involve the posterior mediastinum and retroperitoneum. However, they rarely occur in the adrenal gland. Adrenal ganglioneuromas (AGNs) are hormonally inactive tumors that are mostly discovered incidentally during abdominal imaging performed for unrelated reasons. As preoperative diagnosis is challenging owing to their heterogeneous nature, adrenalectomy is the most effective method to ascertain an AGN diagnosis. We report a case of left adrenal ganglioneuroma treated by laparoscopic adrenalectomy. In addition, we have presented a relevant literature review to provide further information about this rare tumor. CASE REPORT A 41-year-old woman presented to the Emergency Department with left flank pain associated with dysuria. She was diagnosed with renal colic, which was confirmed by computed tomography of the kidneys, ureter, and bladder. Additionally, an incidental solid lesion in the left adrenal gland was discovered. She was treated conservatively for her acute condition at the Emergency Department and discharged in a good condition. Further work-up including magnetic resonance imaging revealed a left large triangular suprarenal mixed soft tissue mass. She underwent laparoscopic left adrenalectomy. The final histopathology showed an AGN. CONCLUSIONS We present a case of a large AGN in a patient with systemic lupus erythematosus. Because it is a rare tumor with a heterogeneous presentation, its preoperative diagnosis is challenging. Thus, adrenalectomy is required to confirm the diagnosis. The prognosis is excellent and recurrence is extremely rare after tumor resection.


Assuntos
Neoplasias das Glândulas Suprarrenais , Ganglioneuroma , Laparoscopia , Neoplasias das Glândulas Suprarrenais/diagnóstico por imagem , Neoplasias das Glândulas Suprarrenais/cirurgia , Glândulas Suprarrenais , Adrenalectomia/métodos , Adulto , Feminino , Ganglioneuroma/diagnóstico por imagem , Ganglioneuroma/cirurgia , Humanos
12.
BMC Urol ; 22(1): 73, 2022 Apr 30.
Artigo em Inglês | MEDLINE | ID: mdl-35501776

RESUMO

INTRODUCTION/BACKGROUND: Adrenal incidentalomas (AIs) are masses > 1 cm found incidentally during radiographic imaging. They are present in up to 4.4% of patients undergoing CT scan, and incidence is increasing with usage and sensitivity of cross-sectional imaging. Most result in diagnosis of adrenal cortical adenoma, questioning guidelines recommending removal of all AIs with negative functional workup. This retrospective study analyzes histological outcome based on size of non-functional adrenal masses. MATERIAL AND METHODS: 10 years of data was analyzed from two academic institutions. Exclusion criteria included patients with positive functional workups, those who underwent adrenalectomy during nephrectomy, < 18 years, and incomplete records. AI radiologic and histologic size, histologic outcome, laterality, imaging modality, gender, and age were collected. T-test was used for comparison of continuous variables, and the two-sided Fisher's exact or chi-square test were used to determine differences for categorical variables. Univariate analysis of each independent variable was performed using simple logistic regression. RESULTS: 73 adrenalectomies met the above inclusion criteria. 60 were detected on CT scan, 12 on MRI, and one on ultrasound. Eight of 73 cases resulted in malignant pathology, 3 of which were adrenocortical carcinoma (ACC). Each ACC measured > 6 cm, with mean radiologic and pathologic sizes of 11.2 cm and 11.3 cm. Both radiologic and pathologic size were significant predictors of malignancy (p = 0.008 and 0.011). CONCLUSIONS: Our results question the generally-accepted 4 cm cutoff for excision of metabolically-silent AIs. They suggest a 6 cm threshold would suffice to avoid removal of benign lesions while maintaining sensitivity for ACC.


Assuntos
Neoplasias do Córtex Suprarrenal , Neoplasias das Glândulas Suprarrenais , Carcinoma Adrenocortical , Neoplasias do Córtex Suprarrenal/diagnóstico , Neoplasias das Glândulas Suprarrenais/patologia , Adrenalectomia , Carcinoma Adrenocortical/cirurgia , Humanos , Estudos Retrospectivos
13.
Endocrine ; 77(1): 160-167, 2022 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-35579782

RESUMO

PURPOSE: The objective of this study was to provide RET genotype-specific data on recurrent ipsilateral pheochromocytoma in multiple endocrine neoplasia type 2A (MEN2A), which are sparse. METHODS: Kaplan-Meier analyses were performed to determine the risk of recurrent ipsilateral adrenalectomy after subtotal and total adrenalectomy in 221 carriers of RET p.Cys634 missense mutations. RESULTS: Altogether, pheochromocytoma emerged in 112 of 442 adrenals at risk, for which 63 adrenals underwent total adrenalectomy and 49 adrenals subtotal adrenalectomy. After a mean (median) of 99 (132.9) months, 10 recurrent ipsilateral pheochromocytomas arose in 10 (20.4%) of 49 adrenal remnants. Seven of these 10 adrenal remnants were subjected to total adrenalectomy and 3 to another subtotal adrenalectomy. After 23 and 250 (mean/median 136.5) more months, 2 of the 3 remaining adrenal remnants gave rise to 2 further recurrent ipsilateral pheochromocytomas, which were removed by total adrenalectomy. When the rare publications in which carriers of RET p.Cys634 mutations made up 81-84% of MEN2A patients were combined with the present RET p.Cys634-specific series, the risk of recurrent ipsilateral pheochromocytoma was 6.7% (25 recurrent ipsilateral pheochromocytomas in 375 adrenal remnants), with a mean time interval of 146 months after initial subtotal adrenalectomy. CONCLUSION: Subtotal adrenalectomy is a viable treatment option for many carriers of RET p.Cys634 mutations who develop an initial pheochromocytoma. Although the adrenal remnant may give rise to recurrent ipsilateral pheochromocytoma after 8-11 years in up to 20% of patients, it is manageable very well in experienced hands, buying the patient valuable time off steroids.


Assuntos
Neoplasias das Glândulas Suprarrenais , Neoplasia Endócrina Múltipla Tipo 2a , Feocromocitoma , Neoplasias das Glândulas Suprarrenais/genética , Neoplasias das Glândulas Suprarrenais/cirurgia , Adrenalectomia , Humanos , Neoplasia Endócrina Múltipla Tipo 2a/genética , Neoplasia Endócrina Múltipla Tipo 2a/cirurgia , Mutação de Sentido Incorreto , Recidiva Local de Neoplasia/genética , Feocromocitoma/genética , Feocromocitoma/cirurgia , Proteínas Proto-Oncogênicas c-ret/genética
14.
Horm Metab Res ; 54(4): 224-231, 2022 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-35413743

RESUMO

The aim of the study was to clarify the relationship and the time of aldosterone and renin recoveries at immediate and long-term follow-up in aldosterone-producing adenoma (APA) patients who underwent adrenalectomy. Prospective and longitudinal protocol in a cohort of APA patients was followed in a single center. Among 43 patients with primary aldosteronism (PA), thirteen APA patients were enrolled in this study. Blood was collected for aldosterone, renin, potassium, creatinine, cortisol, and ACTH before and 1, 3, 5, 7, 15, 30, 60, 90, 120, 180, 270, 360 days after adrenalectomy. At diagnosis, most patients (84%) had hypokalemia and high median aldosterone levels (54.8; 24.0-103 ng/dl) that decreased to undetectable (<2.2) or very low (<3.0) levels between fifth to seventh days after surgery; then, between 3-12 months, its levels gradually increased to the lower normal range. The suppressed renin (2.3; 2.3-2.3 mU/l) became detectable between the fifteen and thirty days after surgery, remaining normal throughout the study. The aldosterone took longer than renin to recover (60 vs.15 days; p<0.002) and patients with higher aldosterone had later recovery (p=0.03). The cortisol/ACTH levels remained normal despite the presence of a post-operative hypoaldosteronism. Blood pressure and antihypertensive requirement decreased after adrenalectomy. In conclusion, our prospective study shows the borderline persistent post-operative hypoaldosteronism in the presence of early renin recovery indicating incapability of the zona glomerulosa of the remaining adrenal gland to produce aldosterone. These findings contribute to the comprehension of differences in renin and aldosterone regulation in APA patients, although both are part of the same interconnected system.


Assuntos
Adenoma , Neoplasias das Glândulas Suprarrenais , Adenoma Adrenocortical , Hiperaldosteronismo , Hipertensão , Hipoaldosteronismo , Adenoma/cirurgia , Neoplasias das Glândulas Suprarrenais/cirurgia , Adrenalectomia , Hormônio Adrenocorticotrópico , Aldosterona , Humanos , Hidrocortisona , Hiperaldosteronismo/cirurgia , Estudos Prospectivos , Renina
15.
BMJ Case Rep ; 15(4)2022 Apr 20.
Artigo em Inglês | MEDLINE | ID: mdl-35444021

RESUMO

Increasing incidence of adrenal incidentalomas with hypercortisolemia not associated with overt features of Cushing's syndrome has led to the evolution of the term autonomous cortisol secretion. Oncocytic adrenocortical neoplasms (OANs) are rare adrenal cortex neoplasms with only 250 reported cases worldwide. We present a woman in her 30s with menometrorrhagia, weight gain and increasing abdominal girth who was found to have a large right adrenal mass on abdominal CT scan. Serum cortisol was not suppressed after 1 mg dexamethasone (31.5 µg/dL) and adrenocorticotrophic hormone was not detectable (<1.0 pg/mL). She underwent right adrenalectomy under glucocorticoid coverage. Hypertension, weight, visceral adiposity and menometrorrhagia improved postoperatively. Histopathology with immunohistochemistry showed OAN with undetermined malignant potential. These kinds of tumours can only be definitively diagnosed postsurgically using the Lin-Weiss-Bisceglia system. Although they have better prognosis compared with adrenocortical carcinomas and do not require chemotherapy, patients should be closely monitored to identify recurrence promptly.


Assuntos
Neoplasias do Córtex Suprarrenal , Neoplasias das Glândulas Suprarrenais , Síndrome de Cushing , Neoplasias do Córtex Suprarrenal/complicações , Neoplasias do Córtex Suprarrenal/diagnóstico , Neoplasias do Córtex Suprarrenal/cirurgia , Neoplasias das Glândulas Suprarrenais/diagnóstico , Adrenalectomia/efeitos adversos , Síndrome de Cushing/diagnóstico , Feminino , Humanos , Hidrocortisona
16.
Endocr J ; 69(4): 327-359, 2022 Apr 28.
Artigo em Inglês | MEDLINE | ID: mdl-35418526

RESUMO

Primary aldosteronism (PA) is associated with higher cardiovascular morbidity and mortality rates than essential hypertension. The Japan Endocrine Society (JES) has developed an updated guideline for PA, based on the evidence, especially from Japan. We should preferentially screen hypertensive patients with a high prevalence of PA with aldosterone to renin ratio ≥200 and plasma aldosterone concentrations (PAC) ≥60 pg/mL as a cut-off of positive results. While we should confirm excess aldosterone secretion by one positive confirmatory test, we could bypass patients with typical PA findings. Since PAC became lower due to a change in assay methods from radioimmunoassay to chemiluminescent enzyme immunoassay, borderline ranges were set for screening and confirmatory tests and provisionally designated as positive. We recommend individualized medicine for those in the borderline range for the next step. We recommend evaluating cortisol co-secretion in patients with adrenal macroadenomas. Although we recommend adrenal venous sampling for lateralization before adrenalectomy, we should carefully select patients rather than all patients, and we suggest bypassing in young patients with typical PA findings. A selectivity index ≥5 and a lateralization index >4 after adrenocorticotropic hormone stimulation defines successful catheterization and unilateral subtype diagnosis. We recommend adrenalectomy for unilateral PA and mineralocorticoid receptor antagonists for bilateral PA. Systematic as well as individualized clinical practice is always warranted. This JES guideline 2021 provides updated rational evidence and recommendations for the clinical practice of PA, leading to improved quality of the clinical practice of hypertension.


Assuntos
Hiperaldosteronismo , Hipertensão , Adrenalectomia , Aldosterona , Humanos , Hiperaldosteronismo/diagnóstico , Hiperaldosteronismo/terapia , Hipertensão/complicações , Japão , Antagonistas de Receptores de Mineralocorticoides , Renina
17.
Front Endocrinol (Lausanne) ; 13: 855326, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35418944

RESUMO

The interest on partial adrenalectomy has steadily increased over the past twenty years. Adrenal pathologies are mostly benign, making an organ-preserving procedure attractive for many patients. The introduction of minimally invasive techniques played probably an important role in this process because they transformed a complex surgical procedure, related to the difficult access to the retroperitoneal space, into a simple operation improving the accessibility to this organ. In this review we summarize the role of partial retroperitoneoscopic adrenalectomy over the years and the current indications and technique.


Assuntos
Neoplasias das Glândulas Suprarrenais , Adrenalectomia , Neoplasias das Glândulas Suprarrenais/patologia , Neoplasias das Glândulas Suprarrenais/cirurgia , Adrenalectomia/métodos , Humanos , Espaço Retroperitoneal/patologia , Espaço Retroperitoneal/cirurgia
19.
Sci Rep ; 12(1): 5781, 2022 04 06.
Artigo em Inglês | MEDLINE | ID: mdl-35388079

RESUMO

Unilateral subtype of primary aldosteronism (PA) is a common surgically curable form of endocrine hypertension. However, more than half of the patients with PA who undergo unilateral adrenalectomy suffer from persistent hypertension, which may discourage those with PA from undergoing adrenalectomy even when appropriate. The aim of this retrospective cross-sectional study was to develop machine learning-based models for predicting postoperative hypertensive remission using preoperative predictors that are readily available in routine clinical practice. A total of 107 patients with PA who achieved complete biochemical success after adrenalectomy were included and randomly assigned to the training and test datasets. Predictive models of complete clinical success were developed using supervised machine learning algorithms. Of 107 patients, 40 achieved complete clinical success after adrenalectomy in both datasets. Six clinical features associated with complete clinical success (duration of hypertension, defined daily dose (DDD) of antihypertensive medication, plasma aldosterone concentration (PAC), sex, body mass index (BMI), and age) were selected based on predictive performance in the machine learning-based model. The predictive accuracy and area under the curve (AUC) for the developed model in the test dataset were 77.3% and 0.884 (95% confidence interval: 0.737-1.000), respectively. In an independent external cohort, the performance of the predictive model was found to be comparable with an accuracy of 80.4% and AUC of 0.867 (95% confidence interval: 0.763-0.971). The duration of hypertension, DDD of antihypertensive medication, PAC, and BMI were non-linearly related to the prediction of complete clinical success. The developed predictive model may be useful in assessing the benefit of unilateral adrenalectomy and in selecting surgical treatment and antihypertensive medication for patients with PA in clinical practice.


Assuntos
Hiperaldosteronismo , Hipertensão , Adrenalectomia , Aldosterona , Anti-Hipertensivos/uso terapêutico , Estudos Transversais , Humanos , Hiperaldosteronismo/diagnóstico , Hiperaldosteronismo/cirurgia , Hipertensão/complicações , Hipertensão/etiologia , Aprendizado de Máquina , Estudos Retrospectivos
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