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2.
Article in English | MEDLINE | ID: mdl-39026485

ABSTRACT

INTRODUCTION: Adrenocortical carcinoma (ACC) is a rare malignancy in children. Because of this, each patient with suspected ACC requires individualised management, which should be determined at a meeting of a team of multidisciplinary experts in the field. AIM OF THE STUDY: To summarise data on symptoms, genetic predisposition, and diagnostic procedures for ACC in children. MATERIAL AND METHODS: Papers were searched in the PubMed database to identify published randomised clinical trials, reviews, systematic reviews, meta-analyses, and case reports. RESULTS: Most cases of ACC in children occur under the age of 5 years. The most common presenting symptom in 60-80% of paediatric patients is rapidly progressive virilisation. Diagnostics are based on laboratory and imaging evaluation. The mainstay of treatment is surgery, with laparotomy being the preferred method of surgery. Diagnosis is based on histological examination of surgically removed tissue. The Wieneke index is most commonly used in paediatric practice. However, some cases are still classified as "indeterminate histology". Predisposing genetic factors are found in most children with ACC, most commonly a mutation of the TP53 gene. CONCLUSIONS: Patients should be diagnosed in large clinical centres with experience in this field. The treatment strategy should be individualised. Genetic testing for TP53 gene mutations is indicated in patients with ACC.


Subject(s)
Adrenal Cortex Neoplasms , Adrenocortical Carcinoma , Humans , Adrenocortical Carcinoma/diagnosis , Adrenocortical Carcinoma/genetics , Adrenocortical Carcinoma/therapy , Adrenocortical Carcinoma/surgery , Adrenal Cortex Neoplasms/diagnosis , Adrenal Cortex Neoplasms/therapy , Adrenal Cortex Neoplasms/genetics , Adrenal Cortex Neoplasms/surgery , Child , Child, Preschool , Male , Female , Infant , Adolescent , Genetic Predisposition to Disease
3.
Beijing Da Xue Xue Bao Yi Xue Ban ; 56(4): 624-630, 2024 Dec 18.
Article in Chinese | MEDLINE | ID: mdl-39041556

ABSTRACT

OBJECTIVE: To analyze the clinicopathological features, prognostic value and surgical treatment experience in patients with adrenocortical carcinoma with venous tumor thrombus. METHODS: We collected relevant data of the patients with adrenocortical carcinoma who had undergone surgery in Peking University Third Hospital from 2018 to 2023. The patients were divided into venous tumor thrombus group and non-tumor thrombus group. The Wilcoxon rank sum test was used to compare the quantitative variables. The chi-squared test and Fisher's exact test were used to compare the categorical variables. The Kaplan-Meier method was used to estimate the survival rate. RESULTS: A total of 27 patients with adrenocortical carcinoma were included, of whom 11 cases (40.7%) had venous tumor thrombus. In the patients with venous tumor thrombus, 8 patients were female and 3 were male. The median age was 49 (36, 58) years. The median body mass index was 26.0 (24.1, 30.4) kg/m2. Seven patients presented with symptoms at their initial visit. Six patients had a history of hypertension. Elevated levels of cortisol were observed in 2 cases. Three tumors were found on the left side, while 8 were found on the right side. Median tumor diameter was 9.4 (6.5, 12.5) cm. On the left, there was a case of tumor thrombus limited to the central vein of the left adrenal gland without invasion into the left renal vein, and two cases of tumor thrombus growth extending into the inferior vena cava below the liver. One case of tumor thrombus on the right adrenal central vein did not invade the inferior vena cava. Four cases of tumor thrombus invaded the inferior vena cava below the liver and three cases extended to the posterior of the liver. Ten patients were in European Network for the Study of Adrenal Tumors (ENSAT) stage Ⅲ and one was in ENSAT stage Ⅳ. Open surgery was performed in 6 cases, laparoscopic surgery alone in 4 cases and robot-assisted laparoscopic surgery in 1 case. Two patients underwent ipsilateral kidney resection. Median operative time was 332 (261, 440) min. Median intraoperative bleeding was 900 (700, 2 200) mL. Median hospital stay was 9 (5, 10) days. Median survival time for the patients with tumor thrombus was 24.0 months and median time to recurrence was 7.0 months. The median survival and recurrence time of 16 patients without tumor thrombus were not reached. The patients with tumor thrombus had worse 3-year overall survival (OS) rate (40.9% vs. 71.4%; Log-rank, P=0.038) and 2-year recurrence-free survival (RFS) (9.1% vs.53.7%; Log-rank, P=0.015) rates compared with the patients with non-tumor thrombus. CONCLUSION: Patients with adrenocortical carcinoma with venous tumor thrombus have poor prognosis. Different adrenal tumor resections and venous tumor thrombus removal procedures based on different tumor thrombus locations are safe and effective in treating this disease.


Subject(s)
Adrenal Cortex Neoplasms , Adrenalectomy , Adrenocortical Carcinoma , Humans , Male , Female , Middle Aged , Adrenocortical Carcinoma/surgery , Adrenocortical Carcinoma/complications , Adrenocortical Carcinoma/pathology , Adrenal Cortex Neoplasms/surgery , Adrenal Cortex Neoplasms/complications , Adrenal Cortex Neoplasms/pathology , Adult , Prognosis , Adrenalectomy/methods , Survival Rate , Venous Thrombosis/etiology , Venous Thrombosis/surgery , Vena Cava, Inferior/pathology
4.
Eur J Endocrinol ; 191(1): 47-54, 2024 Jul 02.
Article in English | MEDLINE | ID: mdl-38941271

ABSTRACT

OBJECTIVE: The aim of this study is to assess whether clinical and imaging characteristics are associated with the hormonal subtype, growth, and adrenalectomy for incidental adrenal cortical adenomas (ACAs). DESIGN: This is a single-center cohort study. METHODS: Consecutive adult patients with incidental ACA were diagnosed between 2000 and 2016. RESULTS: Of the 1516 patients with incidental ACA (median age 59 years, 62% women), 699 (46%) had nonfunctioning adenomas (NFAs), 482 (31%) had mild autonomous cortisol secretion (MACS), 62 (4%) had primary aldosteronism (PA), 39 (3%) had Cushing syndrome, 18 (1%) had PA and MACS, and 226 (15%) had incomplete work-up. Age, sex, tumor size, and tumor laterality, but not unenhanced computed tomography Hounsfield units (HU), were associated with hormonal subtypes. In a multivariable analysis, ≥1 cm growth was associated with younger age (odds ratio [OR] = 0.8 per 5-year increase, P = .0047) and longer imaging follow-up (OR = 1.2 per year, P < .0001). Adrenalectomy was performed in 355 (23%) patients, including 38% of MACS and 15% of NFA. Adrenalectomy for NFA and MACS was more common in younger patients (OR = 0.79 per 5-year increase, P = .002), larger initial tumor size (OR = 2.3 per 1 cm increase, P < .0001), ≥1 cm growth (OR = 15.3, P < .0001), and higher postdexamethasone cortisol (OR = 6.6 for >5 vs <1.8 µg/dL, P = .002). CONCLUSIONS: Age, sex, tumor size, and laterality were associated with ACA hormonal subtype and can guide diagnosis and management. Tumor growth was more common with younger age and longer follow-up. Unenhanced HU did not predict hormonal subtype or growth. Adrenalectomy for MACS and NFA was mainly performed in younger patients with larger tumor size, growth, and elevated postdexamethasone cortisol.


Subject(s)
Adrenal Cortex Neoplasms , Adrenalectomy , Adrenocortical Adenoma , Incidental Findings , Humans , Female , Male , Middle Aged , Adrenocortical Adenoma/surgery , Adrenocortical Adenoma/diagnostic imaging , Adrenocortical Adenoma/pathology , Retrospective Studies , Adrenal Cortex Neoplasms/surgery , Adrenal Cortex Neoplasms/diagnostic imaging , Adrenal Cortex Neoplasms/pathology , Aged , Adult , Cohort Studies , Hydrocortisone/blood , Cushing Syndrome/surgery , Cushing Syndrome/diagnostic imaging , Tomography, X-Ray Computed , Hyperaldosteronism/surgery , Hyperaldosteronism/diagnostic imaging
5.
J Med Case Rep ; 18(1): 276, 2024 Jun 12.
Article in English | MEDLINE | ID: mdl-38863038

ABSTRACT

BACKGROUND: Currently, there is a scarcity of cases and diagnostic data regarding ectopic adrenocortical adenomas, particularly in relation to their impact on gonadal function and localization diagnostic techniques. We report a typical case of ectopic adrenocortical adenomas and the data of treatment follow-up, and review the literature of 31 available cases of ectopic adrenocortical adenomas. CASE PRESENTATION: A 27-year-old Chinese female patient was admitted to our hospital for hypertension, hyperglycaemia and primary amenorrhea. The patient was functionally diagnosed with ACTH-independent CS and hypogonadotropic hypogonadism. Radiological evaluations, including Computed Tomography (CT) and functional imaging, identified a mass at the left renal hilum. Histological assessments post-surgical excision confirmed the mass to be an ectopic adrenocortical adenoma. A subsequent 3-month follow-up showed no signs of disease recurrence, a swift recovery of the cortisol axis was observed, with a partial recuperation of the gonadal axis. REVIEW: Our literature review shows that the most common ectopic areas of cortisol adenomas are renal hilum and hepatic region. The most positive biomarker is Melan A, and only a few cases have been diagnosed with functional localization. CONCLUSION: Ectopic adrenocortical adenomas may be asymptomatic in the early stage and can impact gonadal function. Physicians who treat hypogonadism must be aware of the need to test cortisol levels and perform functional localization in patients with lumps present.


Subject(s)
Adrenal Cortex Neoplasms , Adrenocortical Adenoma , Hypogonadism , Humans , Female , Adult , Adrenocortical Adenoma/surgery , Adrenocortical Adenoma/pathology , Adrenocortical Adenoma/complications , Adrenal Cortex Neoplasms/surgery , Adrenal Cortex Neoplasms/pathology , Adrenal Cortex Neoplasms/diagnostic imaging , Tomography, X-Ray Computed , Hydrocortisone
6.
BMJ Case Rep ; 17(6)2024 Jun 08.
Article in English | MEDLINE | ID: mdl-38851224

ABSTRACT

Adrenocortical tumours are rare in children and account for only 0.3%-0.4% of all neoplasms in childhood. They present with variable signs and symptoms, depending on the type of hormonal hypersecretion. The majority of the adrenocortical tumours in children are functional (90%) and malignant (88%). Here, we describe a functional plurihormonal oncocytic adrenal cortical adenoma in a young girl, that mimicked a malignant adrenal lesion, clinically as well as on imaging and biochemical features. This report bears the objective of being aware of the atypical biochemical as well as imaging characteristics of oncocytic adrenal tumours.


Subject(s)
Adrenal Cortex Neoplasms , Adrenocortical Adenoma , Female , Humans , Adenoma, Oxyphilic/pathology , Adenoma, Oxyphilic/diagnosis , Adenoma, Oxyphilic/diagnostic imaging , Adenoma, Oxyphilic/surgery , Adrenal Cortex Neoplasms/diagnosis , Adrenal Cortex Neoplasms/surgery , Adrenal Cortex Neoplasms/diagnostic imaging , Adrenal Cortex Neoplasms/pathology , Adrenocortical Adenoma/diagnosis , Adrenocortical Adenoma/surgery , Adrenocortical Adenoma/diagnostic imaging , Adrenocortical Adenoma/pathology , Diagnosis, Differential , Tomography, X-Ray Computed , Adolescent
7.
Ann Surg Oncol ; 31(8): 5122-5127, 2024 Aug.
Article in English | MEDLINE | ID: mdl-38802716

ABSTRACT

BACKGROUND: Many adrenal tumors are deemed radiologically indeterminate and surgically removed. Adrenal tissue, like parathyroid glands, exhibits near-infrared autofluorescence (NIRAF) properties. This study was designed to investigate the potential of NIRAF to differentiate benign versus malignant adrenal tumors. METHODS: Patients undergoing adrenalectomy between October 2021 and May 2023 were prospectively studied. Adrenalectomy specimens were inspected with NIRAF imaging. Specimen autofluorescence (AF) characteristics were recorded. Comparisons were made between different tumor types and a logistic regression model was constructed to differentiate benign versus malignant tumors. A receiver operating characteristic curve was used to identify an optimal AF threshold differentiating benign versus malignant tumors. RESULTS: A total of 108 adrenal specimens were examined: adrenocortical adenomas/other benign lesions (n = 72), pheochromocytomas (n = 18), adrenocortical neoplasms of uncertain behavior (n = 4), and malignant tumors (n = 14). A significant difference in normalized AF intensity was identified when comparing adrenocortical adenomas (3.08 times background) with pheochromocytomas (1.95, p = 0.001) and malignant tumors (1.11, p < 0.0001). The Area Under the Curve differentiating benign vs malignant tumors was 0.87, with an optimal normalized AF threshold at 1.93. CONCLUSIONS: Different adrenal pathologies exhibit diverse AF properties. These findings suggest a potential intraoperative utility of NIRAF in predicting benign versus malignant nature for radiologically indeterminate adrenal tumors.


Subject(s)
Adrenal Gland Neoplasms , Optical Imaging , Pheochromocytoma , Humans , Adrenal Gland Neoplasms/pathology , Adrenal Gland Neoplasms/surgery , Adrenal Gland Neoplasms/diagnostic imaging , Female , Male , Middle Aged , Pheochromocytoma/pathology , Pheochromocytoma/surgery , Pheochromocytoma/diagnostic imaging , Prospective Studies , Optical Imaging/methods , Adrenalectomy , Adult , ROC Curve , Adrenocortical Adenoma/pathology , Adrenocortical Adenoma/surgery , Adrenocortical Adenoma/diagnostic imaging , Aged , Diagnosis, Differential , Follow-Up Studies , Prognosis , Adrenal Cortex Neoplasms/pathology , Adrenal Cortex Neoplasms/surgery , Adrenal Cortex Neoplasms/diagnostic imaging , Spectroscopy, Near-Infrared/methods
8.
Urol Oncol ; 42(8): 248.e1-248.e9, 2024 Aug.
Article in English | MEDLINE | ID: mdl-38653591

ABSTRACT

PURPOSE: The role of lymphadenectomy and the optimal lymph node count (LNC) cut-off in nonmetastatic adrenocortical carcinoma (nmACC) are unclear. METHODS: Within the Surveillance, Epidemiology, and End Results (SEER) database, surgically treated nmACC patients with T2-4 stages were identified between 2004 and 2020. We tested for cancer-specific mortality (CSM) differences according to pathological N-stage (pN0 vs. pN1) and two previously recommended LNC cut-offs (≥4 vs. ≥5) were tested in pN0 and subsequently in pN1 subgroups in Kaplan-Meier plots and multivariable Cox regression models. RESULTS: Of 710 surgically treated nmACC patients, 185 (26%) underwent lymphadenectomy and were assessable for further analyses based on available LNC data. Of 185 assessable patients, 152 (82%) were pN0 and 33 (18%) were pN1. In Kaplan-Meier analyses, CSM-free survival was 74 vs. 14 months (Δ 60 months, P ≤ 0.001) in pN0 vs. pN1 patients, respectively. In multivariable analyses, pN1 was an independent predictor of higher CSM (HR:3.13, P < 0.001). In sensitivity analyses addressing pN0, LNC cut-off of ≥4 was associated with lower CSM (multivariable hazard ratio [HR]: 0.52; P = 0.002). In sensitivity analyses addressing pN0, no difference was recorded when a LNC cut-off of ≥5 was used (HR:0.60, P = 0.09). In pN1 patients, neither of the cut-offs (≥4 and ≥5) resulted in a statistically significant stratification of CSM rate, and neither reached independent predictor status (all P > 0.05). CONCLUSIONS: Lymphadenectomy provides a prognostic benefit in nmACC patients and identifies pN1 patients with dismal prognosis. Conversely, in pN0 patients, a LNC cut-off ≥4 identifies those with particularly favorable prognosis.


Subject(s)
Adrenal Cortex Neoplasms , Adrenocortical Carcinoma , Neoplasm Staging , Humans , Male , Adrenocortical Carcinoma/surgery , Adrenocortical Carcinoma/pathology , Adrenocortical Carcinoma/mortality , Female , Middle Aged , Prognosis , Adrenal Cortex Neoplasms/surgery , Adrenal Cortex Neoplasms/pathology , Adrenal Cortex Neoplasms/mortality , Lymph Node Excision , Adult , Lymph Nodes/pathology , Lymph Nodes/surgery , Aged , Retrospective Studies
9.
Clin Genitourin Cancer ; 22(3): 102077, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38626660

ABSTRACT

INTRODUCTION: Adrenocortical carcinoma (ACC) is a rare yet highly malignant tumor associated with significant morbidity and mortality. This study aims to delineate the clinical features, survival patterns, and treatment modalities of ACC, providing insights into the disease's prognosis. MATERIALS AND METHODS: A retrospective analysis of 157 ACC patients was performed to assess treatment methodologies, demographic patterns, pathological and clinical attributes, and laboratory results. The data were extracted from the hospital's database. Survival analyses were conducted using the Kaplan-Meier method, with univariate and multivariate analyses being performed through the log-rank test and Cox regression analyses. RESULTS: The median age was 45, and 89.4% had symptoms at the time of diagnosis. The median tumor size was 12 cm. A total of 117 (79.6%) patients underwent surgery. A positive surgical border was detected in 26 (24.1%) patients. Adjuvant therapy was administered to 44.4% of patients. The median overall survival for the entire cohort was 44.3 months. Median OS was found to be 87.3 months (95% confidence interval [CI] 74.4-100.2) in stage 2, 25.8 (95% CI 6.5-45.1) months in stage 3, and 13.3 (95% CI 7.0-19.6) months in stage 4 disease. Cox regression analysis identified age, Ki67 value, Eastern Cooperative Oncology Group performance status, and hormonal activity as significant factors associated with survival in patients with nonmetastatic disease. In metastatic disease, only patients who underwent surgery exhibited significantly improved overall survival in univariate analyses. CONCLUSION: ACC is an uncommon tumor with a generally poor prognosis. Understanding the defining prognostic factors in both localized and metastatic diseases is vital. This study underscores age, Ki67 value, Eastern Cooperative Oncology Group performance status, and hormonal activity as key prognostic determinants for localized disease, offering critical insights into the complexities of ACC management and potential avenues for targeted therapeutic interventions.


Subject(s)
Adrenal Cortex Neoplasms , Adrenocortical Carcinoma , Humans , Male , Female , Middle Aged , Retrospective Studies , Adrenal Cortex Neoplasms/therapy , Adrenal Cortex Neoplasms/pathology , Adrenal Cortex Neoplasms/mortality , Adrenal Cortex Neoplasms/surgery , Adrenal Cortex Neoplasms/drug therapy , Adrenocortical Carcinoma/therapy , Adrenocortical Carcinoma/pathology , Adrenocortical Carcinoma/mortality , Adrenocortical Carcinoma/drug therapy , Adrenocortical Carcinoma/surgery , Adult , Aged , Turkey/epidemiology , Prognosis , Young Adult , Survival Analysis , Adolescent , Kaplan-Meier Estimate , Treatment Outcome
10.
Ann Ital Chir ; 95(1): 13-16, 2024.
Article in English | MEDLINE | ID: mdl-38469607

ABSTRACT

AIM: We present a case of adrenocortical adenoma originating from the adrenohepatic fusion (AHF) region, accompanied by advanced hepatosteatosis in the liver tissue, and discuss its distinction from hepatocellular carcinoma.  Case Experience: A 68-year-old male patient was admitted to the hospital following a fall from a height. He was referred to our hospital after an incidental discovery of a liver mass during an abdominal ultrasound examination. Subsequently, magnetic resonance imaging (MRI) imaging was conducted, followed by segmental liver resection with right adrenalectomy, and histological analysis of a biopsy from the lesion.  Results: Upon histologic examination, the case was determined to be an adrenocortical adenoma originating from the AHF.  Discussion: Adrenohepatic fusion (AHF) denotes the histological amalgamation of cells from the right adrenal cortex and right hepatic parenchyma. Only a limited number of cases of neoplasia originating from this region have been documented. These rare instances often present a diagnostic challenge, with preoperative imaging frequently misidentifying them as primary malignancies of either hepatic or adrenal origin, potentially leading to unnecessary extensive resections. The integration of immunohistochemical staining alongside clinical and radiological data proves helpful for accurately diagnosing this condition.  Conclusion: Awareness among clinicians, radiologists, and pathologists regarding the tumors that may arise from this region can mitigate the risk of performing extensive resections unnecessarily.


Subject(s)
Adrenal Cortex Neoplasms , Adrenocortical Adenoma , Carcinoma, Hepatocellular , Liver Neoplasms , Male , Humans , Aged , Adrenocortical Adenoma/diagnostic imaging , Adrenocortical Adenoma/surgery , Adrenocortical Adenoma/pathology , Carcinoma, Hepatocellular/diagnostic imaging , Carcinoma, Hepatocellular/surgery , Carcinoma, Hepatocellular/pathology , Adrenal Cortex Neoplasms/diagnostic imaging , Adrenal Cortex Neoplasms/surgery , Liver Neoplasms/diagnostic imaging , Liver Neoplasms/surgery
13.
J Endocrinol Invest ; 47(7): 1751-1762, 2024 Jul.
Article in English | MEDLINE | ID: mdl-38386266

ABSTRACT

BACKGROUND: Limited information exists on postoperative hypocortisolism and hypothalamus-pituitary-adrenal axis recovery in patients with adrenal incidentaloma following unilateral adrenalectomy. We evaluated frequency of postoperative hypocortisolism and predictors for recovery in non-aldosterone-producing adrenocortical adenoma patients after unilateral adrenalectomy. METHODS: A retrospective analysis of 32 adrenal incidentaloma patients originally included in the ITACA trial (NCT04127552) with confirmed non-aldosterone-producing adrenocortical adenoma undergoing unilateral adrenalectomy from September 2019 to April 2023 was conducted. Preoperative assessments included adrenal MRI, anthropometrics, evaluation of comorbidities, adrenal function assessed via ACTH, urinary free cortisol, and 1 mg dexamethasone suppression test. ACTH and serum cortisol or Short Synacthen test were performed within 6 days, 6 weeks, 6 months, and a year after surgery. RESULTS: Six days postoperative, 18.8% of patients had normal adrenal function. Among those with postoperative hypocortisolism, 53.8% recovered by 6 weeks. Patients with earlier adrenal recovery (6 weeks) had lower preoperative 1 mg dexamethasone suppression test (median 1 mg dexamethasone suppression test 76.2 [61.8-111.0] nmol/L vs 260.0 [113.0-288.5] nmol/L, p < 0.001). Univariate analysis showed preoperative 1 mg dexamethasone suppression test negatively related with baseline ACTH levels (r = - 0.376; p = 0.041) and negatively associated with the 6-week baseline (r = - 0.395, p = 0.034) and 30-min cortisol levels during Short Synacthen test (r = - 0.534, p = 0.023). Logistic regression analysis demonstrated preoperative 1 mg dexamethasone suppression test as the only biochemical predictor for 6-week adrenal recovery: ROC curve identified a 1 mg dexamethasone suppression test threshold of 131 nmol/L predicting 6-week recovery with 89.5% sensitivity and 72.7% specificity (AUC 0.87; 95% CI 66.9-98.7, p < 0.001). Other preoperative assessments (tumor size, ACTH levels and anthropometrics) were not associated with postoperative hypothalamus-pituitary-adrenal axis function, but the presence of diabetes was associated with a lower probability of recovery (OR = 24.55, p = 0.036). ACTH levels increased postoperatively in all patients but did not predict hypothalamus-pituitary-adrenal axis recovery. CONCLUSIONS: The preoperative 1 mg dexamethasone suppression test cortisol value and presence of diabetes are the only relevant predictor of hypothalamus-pituitary-adrenal axis recovery in patients with non-aldosterone- producing adrenocortical adenoma undergoing surgery, regardless other clinical and biochemical variables. Notably, pre- and postoperative ACTH levels did not predict hypothalamus-pituitary-adrenal axis recovery. These findings point towards the potential for saving resources by optimizing their allocation during follow-up assessments for patients with non-aldosterone-producing adrenocortical adenoma undergoing unilateral adrenalectomy.


Subject(s)
Adrenalectomy , Adrenocortical Adenoma , Hydrocortisone , Postoperative Complications , Humans , Female , Male , Retrospective Studies , Middle Aged , Hydrocortisone/blood , Adrenocortical Adenoma/surgery , Adrenocortical Adenoma/blood , Postoperative Complications/diagnosis , Postoperative Complications/etiology , Postoperative Complications/blood , Aged , Adrenal Cortex Neoplasms/surgery , Adrenal Cortex Neoplasms/blood , Pituitary-Adrenal System/metabolism , Hypothalamo-Hypophyseal System/metabolism , Prognosis , Adult , Adrenocorticotropic Hormone/blood , Follow-Up Studies , Dexamethasone , Adrenal Gland Neoplasms
14.
Horm Metab Res ; 56(1): 91-98, 2024 Jan.
Article in English | MEDLINE | ID: mdl-38171374

ABSTRACT

International guidelines emphasise the role of local therapies (LT) for the treatment of advanced adrenocortical carcinoma (ACC). However, large studies are lacking in this field. Therefore, we performed a review of the literature to synthesise current evidence and develop clinical guidance. PubMed database was searched for systematic literature. We identified 119 potentially relevant articles, of which 21 could be included in our final analysis. All were retrospective and reported on 374 patients treated with LT for advanced ACC (12 studies on radiotherapy, 3 on transarterial chemoembolisation and radioembolisation, 4 on image-guided thermal ablation [radiofrequency, microwave ablation, and cryoablation, and two studies reporting treatment with several different LT]). Radiotherapy was frequently performed with palliative intention. However, in most patients, disease control and with higher dosage also partial responses could be achieved. Data for other LT were more limited, but also point towards local disease control in a significant percentage of patients. Very few studies tried to identify factors that are predictive on response. Patients with a disease-free interval after primary surgery of more than 9 months and lesions<5 cm might benefit most. Underreporting of toxicities may be prevalent, but LT appear to be relatively safe overall. Available evidence on LT for ACC is limited. LT appears to be safe and effective in cases with limited disease and should be considered depending on local expertise in a multidisciplinary team discussion.


Subject(s)
Adrenal Cortex Neoplasms , Adrenocortical Carcinoma , Carcinoma, Hepatocellular , Liver Neoplasms , Humans , Adrenocortical Carcinoma/radiotherapy , Adrenocortical Carcinoma/surgery , Retrospective Studies , Carcinoma, Hepatocellular/pathology , Liver Neoplasms/pathology , Adrenal Cortex Neoplasms/surgery
16.
J Clin Endocrinol Metab ; 109(8): e1582-e1592, 2024 Jul 12.
Article in English | MEDLINE | ID: mdl-38127970

ABSTRACT

CONTEXT: Clinical implications of unilateral primary aldosteronism (PA) histopathology remain to be determined in various ethnic populations. OBJECTIVE: We examined the histopathology of unilateral PA using CYP11B2 immunostaining in relation to clinical phenotypes and postsurgical outcomes. METHODS: Patients consecutively operated for unilateral PA from 2010 to 2020 at 3 tertiary hospitals in South Korea were retrospectively enrolled. Adrenals with solitary aldosterone-producing adenomas and/or dominant aldosterone-producing nodules were classified as the classical and the others as the nonclassical groups. The classical group was subdivided into mixed or solitary group according to whether other aldosterone-producing lesions coexist or not. RESULTS: Of the 240 cases, 124 were solitary, 86 mixed, and 30 nonclassical. Baseline serum potassium concentration was lower in the solitary group than the mixed or nonclassical group. Plasma aldosterone concentration after saline loading was the highest in the solitary group (median 31.65 ng/dL), followed by the mixed group (median 25.40 ng/dL), and the lowest in the nonclassical group (median 14.20 ng/dL). Solitary and mixed groups showed higher lateralization indices and lower contralateral indices than the nonclassical group. The contralateral index was lower in the solitary group than the mixed group. At 6 to 12 months after adrenalectomy, fewer antihypertensive medications were required for the solitary and mixed groups than the nonclassical group. CONCLUSION: The solitary group, followed by the mixed group, was associated with more severe hyperaldosteronism and more suppressed aldosterone production from the contralateral side than the nonclassical group. Histopathologic phenotypes were related to the clinical manifestations and may suggest postoperative prognosis.


Subject(s)
Adrenalectomy , Aldosterone , Hyperaldosteronism , Phenotype , Humans , Hyperaldosteronism/surgery , Hyperaldosteronism/pathology , Hyperaldosteronism/blood , Female , Male , Middle Aged , Retrospective Studies , Adult , Aldosterone/blood , Treatment Outcome , Republic of Korea/epidemiology , Adrenal Cortex Neoplasms/surgery , Adrenal Cortex Neoplasms/pathology , Adrenal Cortex Neoplasms/complications , Cytochrome P-450 CYP11B2 , Prognosis , Aged , Adrenocortical Adenoma/surgery , Adrenocortical Adenoma/pathology , Adrenocortical Adenoma/complications , Adrenocortical Adenoma/blood
17.
Surgery ; 175(1): 80-89, 2024 01.
Article in English | MEDLINE | ID: mdl-37945477

ABSTRACT

BACKGROUND: Current evidence suggests that cortisol secreting adrenocortical carcinoma has worse prognosis compared to non-secreting adrenocortical carcinoma. However, the effect of other secretory subtypes is unknown. METHODS: This multicenter study within the American-Australian-Asian Adrenal Alliance included adults with adrenocortical carcinoma (1997-2020). We compared overall survival and disease-free survival among cortisol secreting, mixed cortisol/androgen secreting, androgen secreting, and non-secreting adrenocortical carcinoma. RESULTS: Of the 807 patients (mean age 50), 719 included in the secretory subtype analysis: 24.5% were cortisol secreting, 13% androgen secreting, 28% mixed cortisol/androgen, 32.5% non-secreting, and 2% were mineralocorticoid secreting. Median overall survival and disease-free survival for the entire cohort were 60 and 9 months, respectively. Median overall survival was 36 months for cortisol, 30 for mixed, 60 for androgen secreting, and 115 for non-secreting adrenocortical carcinoma, P < .01. Median disease-free survival was 7 months for cortisol, 8 for mixed, 10 for androgen, and 12 for non-secreting adrenocortical carcinoma, P = .06. On multivariable analysis of age, sex, Ki67%, secretory subtype, stage, resection, and adjuvant therapy, predictors of worse overall survival were older age, higher Ki67%, stage IV, mixed secreting, R1, and no adjuvant therapy, P < .05. On subgroup analysis of R0 resection, predictors of worse overall survival included older age and higher Ki67%. Ki67% ≥40, stage III and cortisol secretion were associated with worse disease-free survival. CONCLUSION: Mixed cortisol/androgen secreting adrenocortical carcinoma was associated with worse overall survival, while cortisol or androgen secreting alone were not. Notably, among patients after R0 resection, secretory subtype did not affect overall survival. Cortisol secreting adrenocortical carcinoma demonstrated worse disease-free survival. Ki67% remained a strong predictor of worse overall survival and disease-free survival independent of stage.


Subject(s)
Adrenal Cortex Neoplasms , Adrenocortical Carcinoma , Adult , Humans , Middle Aged , Adrenal Cortex Neoplasms/surgery , Androgens , Hydrocortisone , Ki-67 Antigen , Australia , Retrospective Studies
18.
J Investig Med High Impact Case Rep ; 11: 23247096231218135, 2023.
Article in English | MEDLINE | ID: mdl-38105189

ABSTRACT

A 57-year-old woman was diagnosed with adrenocortical carcinoma. Following the adrenalectomy, she underwent adjuvant radiation and mitotane therapy; however, liver metastases were observed. Repeated radiofrequency ablation (RFA) was performed for liver metastases. In addition, a multidisciplinary approach combining systemic chemotherapy, radiotherapy, and surgery was used for lung and distant lymph node metastases that arose during the course of treatment. Notably, 49 months have passed since the adrenalectomy and 36 months since the recurrence of the liver metastases, and the patient remains on multidisciplinary therapy. Thus, RFA for liver metastasis of adrenocortical carcinoma may be an effective component of a multidisciplinary treatment.


Subject(s)
Adrenal Cortex Neoplasms , Adrenocortical Carcinoma , Catheter Ablation , Liver Neoplasms , Radiofrequency Ablation , Female , Humans , Middle Aged , Adrenocortical Carcinoma/surgery , Liver Neoplasms/surgery , Liver Neoplasms/pathology , Adrenal Cortex Neoplasms/surgery , Adrenal Cortex Neoplasms/pathology
19.
Front Endocrinol (Lausanne) ; 14: 1308231, 2023.
Article in English | MEDLINE | ID: mdl-38260140

ABSTRACT

Context: Adrenocortical carcinoma (ACC) is rare and have high rates of recurrence and mortality. The role of adjuvant radiation therapy (RT) in localized ACC was controversial. Methods: We conducted a retrospective study in our center between 2015 and 2021 to evaluate the efficacy and safety of adjuvant RT in localized ACC. Overall survival (OS) and disease-free survival (DFS) were estimated using the Kaplan-Meier method. Cox proportional hazards regression models were used to estimate the independent risk factors. Adverse events associated with RT were documented according to the toxicity criteria of the radiation therapy oncology group (RTOG) and the common terminology criteria for adverse events (CTCAE v5.0). Results: Of 105 patients with localized ACC, 46 (43.8%) received adjuvant RT after surgery. The median radiation dose was 45.0Gy (range:30.0-50.4) and median follow up time was 36.5 (IQR: 19.7-51.8) months. In comparison to the no adjuvant RT group, patients with adjuvant RT had better 3-year OS (87.9% vs 79.5%, P=0.039), especially for patients with ENSAT I/II stage (P=0.004). Adjuvant RT also improved the median DFS time from 16.5months (95%CI, 12.0-20.9) to 34.6months (95%CI, 16.1-53.0). Toxicity of RT was generally mild and moderate with six grade 3 events. Conclusions: Postoperative adjuvant RT significantly improved OS and DFS compared with the use of surgery alone in resected ACC patients. Although this retrospective study on RT in localized ACC indicates that RT is effective in ACC, its findings need to be prospectively confirmed.


Subject(s)
Adrenal Cortex Neoplasms , Adrenocortical Carcinoma , Humans , Adrenocortical Carcinoma/radiotherapy , Adrenocortical Carcinoma/surgery , Radiotherapy, Adjuvant , Retrospective Studies , Disease-Free Survival , Adrenal Cortex Neoplasms/radiotherapy , Adrenal Cortex Neoplasms/surgery
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