-
Journal of the Endocrine Society Feb 2024Adrenocortical carcinoma (ACC) is a rare and lethal disease with a poor prognosis. This study aims to share our 41-year experience as a referral center, focusing on...
Adrenocortical carcinoma (ACC) is a rare and lethal disease with a poor prognosis. This study aims to share our 41-year experience as a referral center, focusing on identifying risk factors associated with ACC mortality. Our retrospective analysis included a cohort of 150 adult patients with ACC in all stage categories, treated between 1981 and 2022. Tumor hormonal hypersecretion was observed in 78.6% of the patients, and the median age of diagnosis was 40 years. The majority presented as European Network for the Study of Adrenal Tumors (ENSAT) III or IV (22.9% and 31.2%, respectively), and the overall mortality rate was 54.6%. Independent predictors of death were elevated secretion of cortisol (HR = 2.0), androstenedione (HR = 2.2), estradiol (HR = 2.8), 17-OH progesterone (HR = 2.0), and 11-deoxycortisol (HR = 5.1), higher Weiss (HR = 4.3), modified Weiss (HR = 4.4), and Helsinki scores (HR = 12.0), advanced ENSAT stage (HR = 27.1), larger tumor size (HR = 2.7), higher Ki-67 percentage (HR = 2.3), and incomplete surgical resection (HR = 2.5). Mitosis greater than 5/50 high-power field (HR = 5.6), atypical mitosis (HR = 2.3), confluent necrosis (HR = 15.4), venous invasion (HR = 2.8), and capsular invasion (HR = 2.4) were also identified as independent predictors of death. Knowing the risk factors for ACC's mortality may help determine the best treatment option.
PubMed: 38384443
DOI: 10.1210/jendso/bvad170 -
The Oncologist Feb 2024Adrenocortical carcinoma (ACC) is a rare and aggressive malignancy in the advanced setting with poor prognosis. This narrative review provides an overview of the...
Adrenocortical carcinoma (ACC) is a rare and aggressive malignancy in the advanced setting with poor prognosis. This narrative review provides an overview of the epidemiology of ACC and its molecular pathogenesis with a summary of the main involved signaling pathways. We then provide an update on the clinical presentation, diagnosis, and current management strategies of both localized and metastatic disease from a multidisciplinary perspective. We highlight the debate around the use of mitotane in the adjuvant setting and review the use of combination chemotherapy with etoposide, doxorubicin, and cisplatin. The review also focuses on emerging data providing hope for the use of immune checkpoint inhibitors and targeted therapies in ACC with a summary of ongoing trials.
PubMed: 38381694
DOI: 10.1093/oncolo/oyae029 -
Endocrine Jun 2024We aimed to evaluate the prognostic potential of baseline [F]FDG PET/CT for overall survival (OS) in patients with adrenocortical carcinoma (ACC).
PURPOSE
We aimed to evaluate the prognostic potential of baseline [F]FDG PET/CT for overall survival (OS) in patients with adrenocortical carcinoma (ACC).
METHODS
We performed a retrospective analysis of 67 treatment-naïve ACC patients with available [F]FDG PET/CT at time of initial diagnosis. Pretherapeutic PETs of primary tumors were manually segmented and quantitative parameters (maximum/mean/peak standardized uptake value (SUV), metabolic tumor volume (MTV) and tumor lesion glycolysis (TLG, defined as TV*SUV) were derived. Based on a visual read, absence (M0) or presence of metastatic disease (M1) were evaluated. Kaplan-Meier and Cox regression analyses were used to determine the prognostic value of the above mentioned markers on overall survival adjusted for established prognostic markers.
RESULTS
24/67 patients (36%) presented with M0 based on PET/CT, while the remaining 43/67 (64%) had M1-status. 32/67 patients died during follow-up and median OS was 48 months. In 12% of patients FDG-PET detected additional metastatic lesion not clearly visible by CT only. In univariable analysis, all quantitatively derived PET parameters failed to reach significance (P ≥ 0.1), and only PET/CT-based M1-status and Ki-67 were associated with increased mortality (M1: HR 13.89, 95% CI 4.15-86.32, P < 0.001; Ki-67 HR 1.29, 95% CI 1.16-1.42; P < 0.0001). Using multivariable Cox regression analyses, M1-status (HR 9.69, 95% CI 2.82-60.99) and Ki-67 index (HR 1.29, 95% CI 1.13-1.04; P < 0.05) remained significant associated with OS.
CONCLUSION
In treatment-naïve ACC patients, the quantitative PET parameter failed to predict OS, but presence of metastases detected by [F]FDG PET/CT and Ki-67 index were independently associated with shorter OS. Therefore, a simple visual PET-based read-out is of prognostic value at initial diagnosis, while time-consuming PET-based quantification can be omitted.
Topics: Humans; Fluorodeoxyglucose F18; Male; Female; Positron Emission Tomography Computed Tomography; Adrenocortical Carcinoma; Middle Aged; Prognosis; Adrenal Cortex Neoplasms; Retrospective Studies; Adult; Aged; Radiopharmaceuticals; Young Adult
PubMed: 38381353
DOI: 10.1007/s12020-024-03695-6 -
Journal of the Endocrine Society Jan 2024Adrenocortical carcinoma (ACC) is a malignant tumor originating from the adrenal cortex. The aim of the study was to report the incidence of ACC and survival of ACC in...
OBJECTIVES
Adrenocortical carcinoma (ACC) is a malignant tumor originating from the adrenal cortex. The aim of the study was to report the incidence of ACC and survival of ACC in Denmark. The secondary objective was to describe the impact of treatment with mitotane on survival.
DESIGN
Retrospective population study of patients diagnosed with ACC between 2003 and 2019 in Denmark.
METHODS
Individuals at risk for ACC were identified in the national Danish Health registries, and diagnosis of ACC was confirmed by review of the health records. Data on demographics, presentation, treatment, recurrence, and death was evaluated.
RESULTS
138 patients were included in the study with more females (59.4%) than males (40.6%). Incidence rate was 1.4 per million per year. The incidence rate ratio significantly increased only in females by 1.06 [95% confidence interval (CI): 1.02-1.12] per year. Overall median survival was 1.93 (95% CI: 1.24-3.00) years with no differences between males and females. The proportion of patients treated with mitotane (either as adjuvant treatment or as part of a chemotherapeutic regime) was 72.3%. Survival was significantly decreased in women not treated with mitotane compared to women treated with mitotane (either as adjuvant or as part of a chemotherapeutic regime) hazards ratio .30 (95% CI: .10-.89), adjusted for European Network for the Study of Adrenal Tumours score, age at diagnosis, and year of diagnosis, but survival was unaffected by mitotane treatment in men.
CONCLUSION
Incidence of ACC in Denmark was 1.4 per million per year and increased in women but not in males during the study period 2003-2019.
PubMed: 38370442
DOI: 10.1210/jendso/bvae012 -
Cureus Jan 2024Adrenocortical carcinoma (ACC) is a rare disease with a poor prognosis, which essentially needs an early diagnosis because surgery is the only hope of a cure. On the...
Adrenocortical carcinoma (ACC) is a rare disease with a poor prognosis, which essentially needs an early diagnosis because surgery is the only hope of a cure. On the other hand, primary aldosteronism (PA) is an overproduction of aldosterone from the adrenal glands and is known as one of the most common causes of secondary hypertension and hypokalemia. It is mostly a benign disease. ACC accompanied by PA is extremely rare, which can result in delayed diagnosis and clinical pitfalls. A 56-year-old woman was diagnosed with PA. Mild, symptomatic PA was clinically diagnosed as a right-sided aldosterone-producing adenoma (APA) with adrenal tumor using adrenal vein sampling (AVS). The tumor imaging findings showed abnormalities on computed tomography (CT) in terms of size and attenuation value compared with typical benign adenomas. Twelve months later, the tumor was confirmed to be an ACC with cortisol hypersecretion. The resected ACC specimen did not clearly show positive findings for CYP11B1 or CYP11B2, and disorganized steroid production was suspected. However, the prevalence and clinical characteristics of adrenocortical carcinomas with disorganized steroid production remain unclear. Steroidogenic enzyme immunostaining analysis is important not only for the diagnosis of adrenal adenoma but also for a better understanding of the clinical course of hormone-producing ACC.
PubMed: 38344480
DOI: 10.7759/cureus.52137 -
BJS Open Jan 2024
Topics: Humans; Adrenocortical Carcinoma; Adrenal Cortex Neoplasms
PubMed: 38323882
DOI: 10.1093/bjsopen/zrae001 -
American Journal of Translational... 2024Cancer has emerged as a global issue in terms of public health care and treatment. The significance of calcyclin binding protein () in various neoplasms suggests that it...
OBJECTIVES
Cancer has emerged as a global issue in terms of public health care and treatment. The significance of calcyclin binding protein () in various neoplasms suggests that it may serve as a novel biomarker for numerous types of human tumors.
METHODS
Our research investigated the differences in expression between cancer tissues and normal tissues using a total of 18,787 samples from multiple centers. To explore the prognostic factor of in cancers, we utilized Cox regression analysis and Kaplan-Meier curves. We also conducted Spearman's rank correlation analyses to determine the associations of expression with the immune microenvironment, etc. Additionally, we applied gene set enrichment analysis to explore the underlying mechanisms of in cancers. A partial validation of CacyBP expression in cancer tissues was performed through lung adenocarcinoma samples using Western blotting and paired -test.
RESULTS
Compared to normal tissues, exhibited high expression levels in 14 cancer types, including breast invasive carcinoma, and low expression levels in six cancers, including glioblastoma multiforme ( < 0.05). expression was found to be significantly associated with the prognosis of 13 cancers, including adrenocortical carcinoma ( < 0.05). demonstrated a robust ability to distinguish 15 cancers, including cholangiocarcinoma, from their control samples (area under the curve > 0.8). Furthermore, expression was correlated with tumor mutational burden, microsatellite instability, and immune infiltration levels, indicating its potential as an exciting target for cancer treatment. may exert its effects on several signaling pathways, including cytokine-cytokine receptor interaction, in various cancers. Compared with paired adjacent specimens, the expression level of CacyBP protein was up-regulated in lung adenocarcinoma specimens ( < 0.05), partially validating the increased expression of in cancers.
CONCLUSIONS
has the potential to serve as a novel prognostic and predictive marker for multiple human cancers.
PubMed: 38322570
DOI: 10.62347/OWVW7440 -
Asian Journal of Surgery May 2024
Topics: Female; Humans; Male; Adrenal Cortex Neoplasms; Adrenalectomy; Adrenocortical Adenoma; Adrenocortical Carcinoma; Diagnostic Errors; Tomography, X-Ray Computed; Adult
PubMed: 38302362
DOI: 10.1016/j.asjsur.2024.01.098 -
The American Journal of Case Reports Jan 2024BACKGROUND Primary extranodal diffuse large B-cell lymphoma (DLBCL) is a rare, yet highly aggressive and invasive malignancy that can masquerade as a solid organ tumor....
BACKGROUND Primary extranodal diffuse large B-cell lymphoma (DLBCL) is a rare, yet highly aggressive and invasive malignancy that can masquerade as a solid organ tumor. Timely diagnosis is critical for improving prognosis; however, it is challenging to achieve. CASE REPORT We report 2 cases treated at Yale New Haven Hospital (New Haven, CT, USA) and the West Haven Veteran's Affairs Medical Center (West Haven, CT, USA) in 2023. Case 1 describes a 69-year-old woman who presented with a large left adrenal mass that was suspicious for adrenocortical carcinoma and was found to have primary adrenal DLBCL following surgical resection. Case 2 describes a 59-year-old woman with Hashimoto's thyroiditis and goiter who was found to have primary thyroid DLBCL following partial thyroidectomy. CONCLUSIONS Primary extranodal DLBCL should be included in the differential diagnosis of solid adrenal and thyroid tumors. The risks of biopsy, given currently available techniques, should be weighed against the benefits of achieving a definite diagnosis, allowing for timely initiation of systemic immunochemotherapy. When biopsy can be safely performed, techniques designed to evaluate for DLBCL should be incorporated.
Topics: Female; Humans; Aged; Middle Aged; Lymphoma, Large B-Cell, Diffuse; Thyroid Neoplasms; Hashimoto Disease; Biopsy
PubMed: 38287660
DOI: 10.12659/AJCR.942659 -
Medicine Jan 2024Adrenocortical carcinoma (ACC) is a rare and highly aggressive endocrine malignancy. Although surgery can cure localized disease, but the majority of patients experience... (Review)
Review
Adrenocortical carcinoma (ACC) is a rare and highly aggressive endocrine malignancy. Although surgery can cure localized disease, but the majority of patients experience recurrence of ACC. The 5-year survival rate of patients with metastatic ACC is <15%, and the prognosis is poor. Therefore, it is urgent to explore the potential diagnostic markers and therapeutic targets for ACC. Recently, it has been proved that non-coding RNA (ncRNAs) is widely involved in pathological and physiological processes, including tumorigenesis and development. Aberrantly expressed ncRNAs have been found to be involved in the pathogenesis of ACC. Here, we summarized the expression patterns and the molecular mechanism of the involvement of microRNAs (miRNAs), long non-coding RNAs (lncRNAs) and circular RNAs (circRNAs) in ACC development. To explore the clinical value of ncRNAs as noninvasive biomarkers of ACC, we also displayed the relationship between the expression level of ncRNAs and the diagnosis and prognosis of patients with ACC.
Topics: Humans; Adrenocortical Carcinoma; RNA, Untranslated; MicroRNAs; RNA, Long Noncoding; Adrenal Cortex Neoplasms
PubMed: 38277554
DOI: 10.1097/MD.0000000000036908