Materiały źródłowe

• #1 Epidemiology and treatment of adrenal cancer. a case series | ECE2024 | 26th European Congress of Endocrinology | Endocrine Abstracts

  https://www.endocrine-abstracts.org/ea/0099/ea0099ep767

  Epidemiology and treatment of adrenal cancer. a case series […] Adrenal cancer is a rare, highly invasive malignant tumour with the incidence estimated for approximately 0,5-2,0 cases per milion people annually. […] Considering the fact that adrenal cancer is very sporadic not much data on its epidemiology is available. […] The aim of the study was to present the single-center data on the epidemiology of the adrenal cancer. […] In ENSAT staging classification for adrenocortical carcinoma, four patients had ENSAT II stage; seven patients had stage III; three patients had stage IV. […] Adrenal cancer is a neoplasm with varied clinical presentation. It demands specific diagnostic tools and treatment protocols. Therefore, treatment should be conducted in the high-reference centers.

• #2 Adrenocortical carcinoma: a literature review – Thampi – Translational Cancer Research

  https://tcr.amegroups.org/article/view/34606/html

  Adrenal tumors are reported to be present in 3% to 10% of the population, and most adrenal tumors are small, benign nonfunctioning adenoma. In contrast, adrenocortical carcinomas (ACCs) are rare, lethal malignancies with poor overall survival. The National Institute of Health (NIH) Office of Rare Diseases research reports that fewer than 200,000 ACC cases exist within the United States (US) and that deaths from ACC account for 0.2% of the deaths annually. The incidence of ACC is 0.72 per million in the USA while 0.5 to 2 per million worldwide. Interestingly, South and Southeastern Brazil have a greater than 10- to 15-fold increase in cases of ACCs due to the higher prevalence of tumor protein p53 (TP53) germline mutation of the tumor suppressor gene allele R175H and R337H alleles secondary to a founder effect, with approximately 78% found in children and 13% found in adults. When divided further, ACC presents with a bimodal distribution (i.e., in children younger than 5 years and adults aged 40 to 60 years) with an incidence of 1.3% of all childhood cancers versus 0.02% to 0.2% of adult cancers and occurs 1.5 to 2.5 times more in female patients than male patients. Conditions associated with ACC in adults may include Li-Fraumeni syndrome, Familial Adenomatous Polyposis (FAP), Multiple Endocrine Neoplasia type I, and Lynch syndrome.

• #3 Key Statistics for Adrenal Cancer | American Cancer Society

  https://www.cancer.org/cancer/types/adrenal-cancer/about/key-statistics.html

  Adrenal tumors are found in about 1 in every 10 people who have an imaging test (like a CT or MRI) of the adrenal gland. Most are benign adenomas. […] Adrenocortical carcinomas are much less common than adenomas. In fact, they are very rare. The exact number diagnosed in the United States each year is not known. It is probably around 200 per year. […] Patients with adrenocortical carcinoma are usually either very young (less than 5 years old) or middle-aged (40 to 50s). Women appear to be more likely to develop this cancer than men.

• #4 Adrenocortical Carcinoma Study – NCI

  https://www.cancer.gov/ccg/research/genome-sequencing/tcga/studied-cancers/adrenocortical-carcinoma-study

  In the United States, an estimated 300 people are diagnosed with adrenocortical carcinoma each year. If detected at an early stage, this type of cancer can often be successfully treated. However, almost 70% of people are diagnosed with advanced adrenocortical carcinoma. For patients at the latest stage of this cancer, less than 20% survive five years after diagnosis. […] Further evidence that overexpression of IGF2, a gene encoding a hormone involved in cell growth, and mutation of TP53, a very common tumor suppressor gene, are hallmarks of adrenocortical carcinoma.

• #5 Azthena logo with the word Azthena

  https://www.news-medical.net/news/20230825/Study-shows-not-all-adrenal-carcinoma-patients-need-mitotane-after-surgery.aspx

  After the complete tumor resection, not all patients with an adrenocortical carcinoma require the previous standard therapy Mitotane. […] In a new clinical study published in August 2023 in the journal The Lancet Diabetes Endocrinology, he and Massimo Terzolo and other collaborators found that the adjuvant treatment with Mitotane is not necessary if the patients fulfill three factors. […] ADIUVO is the first-ever randomized trial worldwide of adjuvant treatment for adrenocortical carcinoma. […] The efficacy of Mitotane versus surveillance only was assessed by recurrence-free survival (RFS). […] Martin Fassnacht sums up: „Concomitant therapy with Mitotane is not indicated in patients with low-grade, localized adrenocortical carcinoma in which the tumor has not yet metastasized and could be completely removed, as their prognoses are relatively good and treatment with Mitotane does not show a statistically significant improvement in the relapse rate, but is associated with side effects.” […] Endocrinology at Wrzburg University Hospital is considered an international reference center for the diagnosis, treatment and research of adrenocortical carcinoma and is currently the largest center worldwide. […] In Germany, it is estimated that there are about 80 to 120 new cases every year.

• #6 Current prospects of hereditary adrenal tumors: towards better clinical management | Hereditary Cancer in Clinical Practice | Full Text

  https://hccpjournal.biomedcentral.com/articles/10.1186/s13053-024-00276-6

  Adrenocortical carcinoma (ACC) and pheochromocytoma/paraganglioma (PPGL) are two rare types of adrenal gland malignancies. […] According to the Rare Cancers in Europe (RARECARE) project data, the annual incidences of ACC, malignant pheochromocytoma, and paraganglioma are 0.22, 0.04, and 0.02 per 100,000 individuals per year, meeting the criteria for rare malignancies (6 per 100,000). […] According to a large genomic analysis of pediatric cancers, the percentage of pathogenic germline variant carriers among all types of malignancies was the highest in ACC (approximately 50%). […] Recent investigations have broadened this spectrum to include a wider range of syndromes, such as Lynch syndrome. […] There is no established treatment approach yet for hereditary adrenal tumors, unlike well-known hereditary syndromes such as breast and ovarian cancer or Lynch syndrome.

• #7 Adrenal Carcinoma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/276264-overview

  AC occurs in 2 major peaks: in the first decade of life and again in the fourth to fifth decades. While, functional tumors are more common in children, however, nonfunctional tumors are more common in adults. […] Based on data from the International Pediatric Adrenocortical Tumor Registry, the median age at which children develop adrenal carcinomas is 3.2 years; 60% are younger than four years, and 14% are older than 13 years.

• #8 Pathology Core Reference Laboratory Case Study: Virilizing Tumors of the Adrenal Gland – Penn State College of Medicine Research

  https://research.med.psu.edu/core-facilities/pathology-core-reference-laboratory/case-study-2/

  Virilizing tumors of the adrenal gland are relatively rare entities not commonly seen in clinical practice. The estimated incidence is 1 per 1.7 million. The presentation of precocious virilization however, is not uncommon in children and can present from birth up through puberty. […] DHEA-S measurements are also considered to be an important marker for adrenal tumors. As with this patient, other adrenal steroids including aldosterone and cortisol are sometimes found normal in patients with tumors of the adrenal. The discriminating steroid to measure when suspecting an adrenal tumor is DHEA-S which is usually markedly raised in patients with an adrenal carcinoma. […] Although rare, there is a bimodal peak in the age of incidence of virilizing adrenal tumors in the first and fourth decades of life.

• #9 Adrenocortical carcinoma: a literature review – Thampi – Translational Cancer Research

  https://tcr.amegroups.org/article/view/34606/html

  Adrenal tumors are reported to be present in 3% to 10% of the population, and most adrenal tumors are small, benign nonfunctioning adenoma. In contrast, adrenocortical carcinomas (ACCs) are rare, lethal malignancies with poor overall survival. The National Institute of Health (NIH) Office of Rare Diseases research reports that fewer than 200,000 ACC cases exist within the United States (US) and that deaths from ACC account for 0.2% of the deaths annually. The incidence of ACC is 0.72 per million in the USA while 0.5 to 2 per million worldwide. Interestingly, South and Southeastern Brazil have a greater than 10- to 15-fold increase in cases of ACCs due to the higher prevalence of tumor protein p53 (TP53) germline mutation of the tumor suppressor gene allele R175H and R337H alleles secondary to a founder effect, with approximately 78% found in children and 13% found in adults. When divided further, ACC presents with a bimodal distribution (i.e., in children younger than 5 years and adults aged 40 to 60 years) with an incidence of 1.3% of all childhood cancers versus 0.02% to 0.2% of adult cancers and occurs 1.5 to 2.5 times more in female patients than male patients. Conditions associated with ACC in adults may include Li-Fraumeni syndrome, Familial Adenomatous Polyposis (FAP), Multiple Endocrine Neoplasia type I, and Lynch syndrome.

• #10 Adrenal Carcinoma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/276264-overview

  AC occurs in 2 major peaks: in the first decade of life and again in the fourth to fifth decades. While, functional tumors are more common in children, however, nonfunctional tumors are more common in adults. […] Based on data from the International Pediatric Adrenocortical Tumor Registry, the median age at which children develop adrenal carcinomas is 3.2 years; 60% are younger than four years, and 14% are older than 13 years.

• #11 Adrenal Carcinoma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/276264-overview

  Adrenocortical carcinomas (ACs) are rare malignancies, with an annual incidence rate of only 0.72 cases per million population in Western countries. […] AC tumors are uncommon, having an incidence of approximately 0.6-1.67 cases per million persons per year. In southern Brazil, however, the incidence of adrenal tumors is 10-15 times that of the general population, a difference that has been associated with a mutation in the P53 gene. […] The female-to-male ratio for ACs is approximately 2.5-3:1. The accumulation of data, especially in international registries, revealed the incidence of adrenal tumors to be higher in female individuals than had previously been thought, particularly in those aged 0-3 years and those over 13 years. Nonfunctional ACs are distributed equally between the sexes.

• #12 Adrenocortical carcinoma: a literature review – Thampi – Translational Cancer Research

  https://tcr.amegroups.org/article/view/34606/html

  Adrenal tumors are reported to be present in 3% to 10% of the population, and most adrenal tumors are small, benign nonfunctioning adenoma. In contrast, adrenocortical carcinomas (ACCs) are rare, lethal malignancies with poor overall survival. The National Institute of Health (NIH) Office of Rare Diseases research reports that fewer than 200,000 ACC cases exist within the United States (US) and that deaths from ACC account for 0.2% of the deaths annually. The incidence of ACC is 0.72 per million in the USA while 0.5 to 2 per million worldwide. Interestingly, South and Southeastern Brazil have a greater than 10- to 15-fold increase in cases of ACCs due to the higher prevalence of tumor protein p53 (TP53) germline mutation of the tumor suppressor gene allele R175H and R337H alleles secondary to a founder effect, with approximately 78% found in children and 13% found in adults. When divided further, ACC presents with a bimodal distribution (i.e., in children younger than 5 years and adults aged 40 to 60 years) with an incidence of 1.3% of all childhood cancers versus 0.02% to 0.2% of adult cancers and occurs 1.5 to 2.5 times more in female patients than male patients. Conditions associated with ACC in adults may include Li-Fraumeni syndrome, Familial Adenomatous Polyposis (FAP), Multiple Endocrine Neoplasia type I, and Lynch syndrome.

• #13 Adrenal Carcinoma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/276264-overview

  Adrenocortical carcinomas (ACs) are rare malignancies, with an annual incidence rate of only 0.72 cases per million population in Western countries. […] AC tumors are uncommon, having an incidence of approximately 0.6-1.67 cases per million persons per year. In southern Brazil, however, the incidence of adrenal tumors is 10-15 times that of the general population, a difference that has been associated with a mutation in the P53 gene. […] The female-to-male ratio for ACs is approximately 2.5-3:1. The accumulation of data, especially in international registries, revealed the incidence of adrenal tumors to be higher in female individuals than had previously been thought, particularly in those aged 0-3 years and those over 13 years. Nonfunctional ACs are distributed equally between the sexes.

• #14 Adrenal cortical carcinoma | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/adrenal-cortical-carcinoma-1?lang=us

  An adrenal cortical carcinoma, also known as adrenocortical carcinoma, is a highly malignant but rare primary neoplasm of the adrenal gland. It may present as a hormonally active or inactive tumor. […] Although men and women are affected equally, functioning tumors are more common in females, who are also more likely to have an associated endocrine syndrome. The median age of presentation is around 50 years, but tumors have been found at all ages. […] Incidence is low, ranging from 0.6 to 1.67 per million, per year.

• #15 Adrenal Carcinoma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/276264-overview

  Adrenocortical carcinomas (ACs) are rare malignancies, with an annual incidence rate of only 0.72 cases per million population in Western countries. […] AC tumors are uncommon, having an incidence of approximately 0.6-1.67 cases per million persons per year. In southern Brazil, however, the incidence of adrenal tumors is 10-15 times that of the general population, a difference that has been associated with a mutation in the P53 gene. […] The female-to-male ratio for ACs is approximately 2.5-3:1. The accumulation of data, especially in international registries, revealed the incidence of adrenal tumors to be higher in female individuals than had previously been thought, particularly in those aged 0-3 years and those over 13 years. Nonfunctional ACs are distributed equally between the sexes.

• #16 Adrenocortical carcinoma: a literature review – Thampi – Translational Cancer Research

  https://tcr.amegroups.org/article/view/34606/html

  Adrenal tumors are reported to be present in 3% to 10% of the population, and most adrenal tumors are small, benign nonfunctioning adenoma. In contrast, adrenocortical carcinomas (ACCs) are rare, lethal malignancies with poor overall survival. The National Institute of Health (NIH) Office of Rare Diseases research reports that fewer than 200,000 ACC cases exist within the United States (US) and that deaths from ACC account for 0.2% of the deaths annually. The incidence of ACC is 0.72 per million in the USA while 0.5 to 2 per million worldwide. Interestingly, South and Southeastern Brazil have a greater than 10- to 15-fold increase in cases of ACCs due to the higher prevalence of tumor protein p53 (TP53) germline mutation of the tumor suppressor gene allele R175H and R337H alleles secondary to a founder effect, with approximately 78% found in children and 13% found in adults. When divided further, ACC presents with a bimodal distribution (i.e., in children younger than 5 years and adults aged 40 to 60 years) with an incidence of 1.3% of all childhood cancers versus 0.02% to 0.2% of adult cancers and occurs 1.5 to 2.5 times more in female patients than male patients. Conditions associated with ACC in adults may include Li-Fraumeni syndrome, Familial Adenomatous Polyposis (FAP), Multiple Endocrine Neoplasia type I, and Lynch syndrome.

• #17 A Rare But Challenging Disease – CSRF – Cushing’s Support & Research Foundation

  https://csrf.net/understanding-cushings/adrenal-cancer/a-rare-but-challenging-disease/

  Adrenal cancer is a rare adrenal tumor that accounts for only 0.2% of deaths due to cancer. The incidence has been estimated at 2 per million people per year. […] Approximately 50% of these tumors are functioning and produce hormonal and metabolic syndromes leading to their discovery. The other 50% are silent and discovered only when they are so large that they produce localized abdominal symptoms or when they metastasize. […] An exception to this age distribution occurs in southern Brazil, where the annual incidence of adrenal cancer in children is unusually high, ranging from 3.4-4.2 per million children, compared with a worldwide incidence of 0.3 per million children younger than 15. […] The cause of adrenal cancer is unknown but studies in the past five years suggest genetic mutations in the adrenal gland leading to the initiation of a malignant tumor.

• #18 Current prospects of hereditary adrenal tumors: towards better clinical management | Hereditary Cancer in Clinical Practice | Full Text

  https://hccpjournal.biomedcentral.com/articles/10.1186/s13053-024-00276-6

  Adrenocortical carcinoma (ACC) and pheochromocytoma/paraganglioma (PPGL) are two rare types of adrenal gland malignancies. […] According to the Rare Cancers in Europe (RARECARE) project data, the annual incidences of ACC, malignant pheochromocytoma, and paraganglioma are 0.22, 0.04, and 0.02 per 100,000 individuals per year, meeting the criteria for rare malignancies (6 per 100,000). […] According to a large genomic analysis of pediatric cancers, the percentage of pathogenic germline variant carriers among all types of malignancies was the highest in ACC (approximately 50%). […] Recent investigations have broadened this spectrum to include a wider range of syndromes, such as Lynch syndrome. […] There is no established treatment approach yet for hereditary adrenal tumors, unlike well-known hereditary syndromes such as breast and ovarian cancer or Lynch syndrome.

• #19 Current prospects of hereditary adrenal tumors: towards better clinical management | Hereditary Cancer in Clinical Practice | Full Text

  https://hccpjournal.biomedcentral.com/articles/10.1186/s13053-024-00276-6

  The clinical and genomic features of representative hereditary tumors associated with adrenal tumors are presented in Table 1, and the available surveillance strategies proposed in the guidelines or expert statements are listed in Table 2. […] The National Comprehensive Cancer Network (NCCN) guidelines for neuroendocrine and adrenal tumors include LFS, Lynch syndrome (LS), multiple endocrine neoplasia type 1 (MEN1), and familial adenomatous polyposis (APC) as hereditary cancer predisposition syndromes associated with ACC. […] The clinical evidence for routine ACC screening is insufficient for individuals with LS. […] An international consensus statement for the clinical management of BWS mentioned a screening strategy using clinical evaluation, adrenal ultrasound, and evaluating serum dehydroepiandrosterone sulfate levels every 4-6 months.

• #20 Adrenocortical carcinoma: a literature review – Thampi – Translational Cancer Research

  https://tcr.amegroups.org/article/view/34606/html

  Adrenal tumors are reported to be present in 3% to 10% of the population, and most adrenal tumors are small, benign nonfunctioning adenoma. In contrast, adrenocortical carcinomas (ACCs) are rare, lethal malignancies with poor overall survival. The National Institute of Health (NIH) Office of Rare Diseases research reports that fewer than 200,000 ACC cases exist within the United States (US) and that deaths from ACC account for 0.2% of the deaths annually. The incidence of ACC is 0.72 per million in the USA while 0.5 to 2 per million worldwide. Interestingly, South and Southeastern Brazil have a greater than 10- to 15-fold increase in cases of ACCs due to the higher prevalence of tumor protein p53 (TP53) germline mutation of the tumor suppressor gene allele R175H and R337H alleles secondary to a founder effect, with approximately 78% found in children and 13% found in adults. When divided further, ACC presents with a bimodal distribution (i.e., in children younger than 5 years and adults aged 40 to 60 years) with an incidence of 1.3% of all childhood cancers versus 0.02% to 0.2% of adult cancers and occurs 1.5 to 2.5 times more in female patients than male patients. Conditions associated with ACC in adults may include Li-Fraumeni syndrome, Familial Adenomatous Polyposis (FAP), Multiple Endocrine Neoplasia type I, and Lynch syndrome.

• #21 Overview of endocrine tumor syndromes manifesting as adrenal tumors

  https://e-emj.org/journal/view.php?number=16

  The overall tumor risk associated with BWS is approximately 5%10%, with adrenocortical carcinoma accounting for about 7% of malignancies. […] For patients with BWS, screening for adrenal tumors is recommended, including clinical evaluations for hormone excess, adrenal ultrasound, and monitoring serum dihydroepiandrosterone sulfate levels every 4 to 6 months. […] Adrenocortical tumors are rare in the pediatric population. […] For adrenal screening in MEN1, abdominal imaging with CT or MRI is recommended every 3 years, and adrenal lesions require ongoing radiologic surveillance for signs of malignancy. […] The typical age of diagnosis is usually between 40 and 60 years, and the condition is rare in children. […] These tumors are associated with hereditary cancer syndromes such as MEN2, neurofibromatosis type 1 (NF1), and von Hippel-Lindau (VHL) disease.

• #22 Childhood Adrenocortical Carcinoma Treatment (PDQ®) – NCI

  https://www.cancer.gov/types/adrenocortical/hp/child-adrenocortical-treatment-pdq

  Predisposing genetic factors have been implicated in more than 50% of adrenocortical carcinoma cases in North America and Europe and in 95% of cases in Brazil. Germline TP53 pathogenic variants are almost always the predisposing factor for adrenocortical tumors. The likelihood of a TP53 germline pathogenic variant is highest in the first years of life and diminishes with age. […] Patients with Beckwith-Wiedemann and hemihypertrophy syndromes have a predisposition to cancer, and as many as 16% of their neoplasms are adrenocortical tumors. […] The overall probability of 5-year survival for children with adrenocortical tumors depends on stage. Survival rates range from greater than 80% for patients with resectable disease to less than 20% for patients with metastases. Overall, adverse prognostic factors for adrenocortical carcinoma include the following: Presence of somatic TP53 variants; Larger tumor size; Disease Stage; Age older than 4 or 5 years; Microscopic tumor necrosis; Para-aortic lymph node involvement; Incomplete resection or spillage during surgery; Low HLA class II antigen expression; Higher Ki-67 labeling index; Grade, Resection status, Age, and Symptom (GRAS) score. […] Tumor staging is performed using the tumor-node-metastasis (TNM) classification system of the American Joint Committee on Cancer (AJCC).

• #23 Overview of endocrine tumor syndromes manifesting as adrenal tumors

  https://e-emj.org/journal/view.php?number=16

  Adrenocortical carcinomas, however, are rare, with an incidence of 0.2 to 0.3 cases per million in adults. […] Most adrenocortical tumors in adults are discovered incidentally, without excess adrenal hormone production, although 15% are functioning tumors. […] In pediatric cases, androgen excess is the commonly observed presentation, followed by Cushing syndrome or a combination of both. […] Therefore, clinical suspicion of adrenal tumors is warranted when patients present with symptoms of virilization and Cushing syndrome. […] Notably, adrenocortical carcinomas are more common in pediatric cases than in adults. […] Therefore, when diagnosing adrenocortical carcinoma in pediatric patients, the possibility of familial cancer syndromes must be considered. […] The incidence of germline TP53 mutations is particularly high in those diagnosed with adrenocortical carcinoma during childhood, with the rate decreasing from 58% in those diagnosed before the age of 12% to 25% in those diagnosed between the ages of 12 and 20.

• #24 Adrenocortical carcinoma: a literature review – Thampi – Translational Cancer Research

  https://tcr.amegroups.org/article/view/34606/html

  Adrenal tumors are reported to be present in 3% to 10% of the population, and most adrenal tumors are small, benign nonfunctioning adenoma. In contrast, adrenocortical carcinomas (ACCs) are rare, lethal malignancies with poor overall survival. The National Institute of Health (NIH) Office of Rare Diseases research reports that fewer than 200,000 ACC cases exist within the United States (US) and that deaths from ACC account for 0.2% of the deaths annually. The incidence of ACC is 0.72 per million in the USA while 0.5 to 2 per million worldwide. Interestingly, South and Southeastern Brazil have a greater than 10- to 15-fold increase in cases of ACCs due to the higher prevalence of tumor protein p53 (TP53) germline mutation of the tumor suppressor gene allele R175H and R337H alleles secondary to a founder effect, with approximately 78% found in children and 13% found in adults. When divided further, ACC presents with a bimodal distribution (i.e., in children younger than 5 years and adults aged 40 to 60 years) with an incidence of 1.3% of all childhood cancers versus 0.02% to 0.2% of adult cancers and occurs 1.5 to 2.5 times more in female patients than male patients. Conditions associated with ACC in adults may include Li-Fraumeni syndrome, Familial Adenomatous Polyposis (FAP), Multiple Endocrine Neoplasia type I, and Lynch syndrome.

• #25 Adrenal Incidentaloma: Practice Essentials, Anatomy, Pathophysiology

  https://emedicine.medscape.com/article/116587-overview

  Adrenal incidentalomas (AIs) are a common finding on cross-sectional abdominal images. In about 1-5% of all cases, abdominal computed tomography (CT) scans that are obtained for reasons other than the evaluation for possible adrenal neoplasm demonstrate an adrenal mass; most of these are AIs. The autopsy prevalence for AIs is 2-9%. […] Approximately 1-10% of CT scans and magnetic resonance images (MRIs) detect AIs that are 5 mm or larger. An Italian study of incidentally discovered AIs among subjects undergoing chest CT scan found that the prevalence of AIs was approximately 4%. […] Prevalence increases with age; the rate is less than 1% for patients younger than 30 years and is 7% for patients 70 years or older. Evidence suggests that the incidence in teenage girls is slightly higher than that of teenage boys, but no sex-related predilection is found in adults. AI prevalence is higher in white than in black people and in obese, diabetic, and hypertensive patients.

• #26 Adrenal Incidentaloma: Practice Essentials, Anatomy, Pathophysiology

  https://emedicine.medscape.com/article/116587-overview

  Adrenal incidentalomas (AIs) are a common finding on cross-sectional abdominal images. In about 1-5% of all cases, abdominal computed tomography (CT) scans that are obtained for reasons other than the evaluation for possible adrenal neoplasm demonstrate an adrenal mass; most of these are AIs. The autopsy prevalence for AIs is 2-9%. […] Approximately 1-10% of CT scans and magnetic resonance images (MRIs) detect AIs that are 5 mm or larger. An Italian study of incidentally discovered AIs among subjects undergoing chest CT scan found that the prevalence of AIs was approximately 4%. […] Prevalence increases with age; the rate is less than 1% for patients younger than 30 years and is 7% for patients 70 years or older. Evidence suggests that the incidence in teenage girls is slightly higher than that of teenage boys, but no sex-related predilection is found in adults. AI prevalence is higher in white than in black people and in obese, diabetic, and hypertensive patients.

• #27 Epidemiology of Adrenal Tumors – a Population-based Study in Olmsted County, Minnesota

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7601441/

  Adrenal tumors are commonly encountered in clinical practice, but current epidemiological data mainly originate from referral centers. We aimed to determine incidence rates (SIR), prevalence, and rates of malignancy and hormone excess in a standardized setting. […] An adrenal tumor was diagnosed in 1,287 patients (median age 62 years; 713 (554%) were women, and 13 (10%) were children). SIR increased from 44 (95%CI 0386) per 100,000 person-years in 1995 to 478 (95%CI 369587) in 2017, mainly due to incidental discovery of adenomas 40mm in 40-year-olds. Prevalence of adrenal tumors in 2017 was 532/100,000 inhabitants, ranging from 13/100,000 in children to 1,900/100,000 among 65-year-olds. Of 1,287 patients, 111 (86%) were diagnosed with malignancy (96 (75%) metastases), 14 (11%) with pheochromocytoma, and 53 (41%) with overt steroid hormone excess. Malignancy was more common in children (62% vs 8% in 18-year-olds, P0001), tumors discovered non-incidentally (32% vs 3% in incidentalomas, P0001), tumors 4 cm (34% vs 7% in 4 cm, P0001), tumors with unenhanced CT attenuation 20 Hounsfield units (15% vs 1% for 20 Hounsfield units, P0001), and bilateral masses (16% vs 7% for unilateral, P=0004). […] Adrenal tumor SIR increased 10 times from 1995 to 2017. Population-based data revealed lower rates of malignancy, pheochromocytoma, and overt steroid hormone excess than previously reported.

• #28 Science Saturday: All adrenal tumors should be investigated – Mayo Clinic News Network

  https://newsnetwork.mayoclinic.org/discussion/science-saturday-all-adrenal-tumors-should-be-investigated/

  The study examined medical records collected as part of the Rochester Epidemiology Project. Using information collected by the project, researchers can identify what causes diseases; how diseases may progress over time; and how patients with certain conditions respond to surgery, medication or other interventions. […] Medical record review yielded 1,287 people diagnosed with adrenal tumors from 1995 to 2017. Due to increased use of medical imaging, the researchers found that diagnoses of any kind of adrenal tumor became 10 times more common by the end of the 23-year period. […] Of the incidentaloma group — which accounted for about 81.5% of the tumors found — some had malignant adrenal masses (3.3%) and a smaller group had obvious signs of elevated hormones (1.9%). […] „The simple but important thing we discovered was a population-based proportion of malignant tumors, which is very helpful when discussing management with a patient newly diagnosed with an adrenal mass,” she says.

• #29 Epidemiology of Adrenal Tumors – a Population-based Study in Olmsted County, Minnesota

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7601441/

  Adrenal tumors are commonly encountered in clinical practice, but current epidemiological data mainly originate from referral centers. We aimed to determine incidence rates (SIR), prevalence, and rates of malignancy and hormone excess in a standardized setting. […] An adrenal tumor was diagnosed in 1,287 patients (median age 62 years; 713 (554%) were women, and 13 (10%) were children). SIR increased from 44 (95%CI 0386) per 100,000 person-years in 1995 to 478 (95%CI 369587) in 2017, mainly due to incidental discovery of adenomas 40mm in 40-year-olds. Prevalence of adrenal tumors in 2017 was 532/100,000 inhabitants, ranging from 13/100,000 in children to 1,900/100,000 among 65-year-olds. Of 1,287 patients, 111 (86%) were diagnosed with malignancy (96 (75%) metastases), 14 (11%) with pheochromocytoma, and 53 (41%) with overt steroid hormone excess. Malignancy was more common in children (62% vs 8% in 18-year-olds, P0001), tumors discovered non-incidentally (32% vs 3% in incidentalomas, P0001), tumors 4 cm (34% vs 7% in 4 cm, P0001), tumors with unenhanced CT attenuation 20 Hounsfield units (15% vs 1% for 20 Hounsfield units, P0001), and bilateral masses (16% vs 7% for unilateral, P=0004). […] Adrenal tumor SIR increased 10 times from 1995 to 2017. Population-based data revealed lower rates of malignancy, pheochromocytoma, and overt steroid hormone excess than previously reported.

• #30 A case of adrenal cortical carcinoma arising from a regularly monitored sub-centimetre adrenal incidentaloma in: Endocrinology, Diabetes & Metabolism Case Reports Volume 2024 Issue 1 (2024)

  https://edm.bioscientifica.com/view/journals/edm/2024/1/EDM23-0120.xml

  A recent large series of 512 patients with ACC reported that ACC first detected as an incidentaloma accounted for almost 40% of cases, with a frequency that increased with age. Patients who had an incidental finding of ACC were more likely to have a better outcome compared with patients who presented with symptomatic disease. […] We present a case of the diagnosis of ACC more than 7 years after the commencement of surveillance of a benign adrenal adenoma, well beyond the time frame for surveillance recommended in all major clinical guidelines.

• #31 A case of adrenal cortical carcinoma arising from a regularly monitored sub-centimetre adrenal incidentaloma in: Endocrinology, Diabetes & Metabolism Case Reports Volume 2024 Issue 1 (2024)

  https://edm.bioscientifica.com/view/journals/edm/2024/1/EDM23-0120.xml

  A recent large series of 512 patients with ACC reported that ACC first detected as an incidentaloma accounted for almost 40% of cases, with a frequency that increased with age. Patients who had an incidental finding of ACC were more likely to have a better outcome compared with patients who presented with symptomatic disease. […] We present a case of the diagnosis of ACC more than 7 years after the commencement of surveillance of a benign adrenal adenoma, well beyond the time frame for surveillance recommended in all major clinical guidelines.

• #32 A Rare But Challenging Disease – CSRF – Cushing’s Support & Research Foundation

  https://csrf.net/understanding-cushings/adrenal-cancer/a-rare-but-challenging-disease/

  Adrenal cancer is a rare adrenal tumor that accounts for only 0.2% of deaths due to cancer. The incidence has been estimated at 2 per million people per year. […] Approximately 50% of these tumors are functioning and produce hormonal and metabolic syndromes leading to their discovery. The other 50% are silent and discovered only when they are so large that they produce localized abdominal symptoms or when they metastasize. […] An exception to this age distribution occurs in southern Brazil, where the annual incidence of adrenal cancer in children is unusually high, ranging from 3.4-4.2 per million children, compared with a worldwide incidence of 0.3 per million children younger than 15. […] The cause of adrenal cancer is unknown but studies in the past five years suggest genetic mutations in the adrenal gland leading to the initiation of a malignant tumor.

• #33 A Rare But Challenging Disease – CSRF – Cushing’s Support & Research Foundation

  https://csrf.net/understanding-cushings/adrenal-cancer/a-rare-but-challenging-disease/

  Adrenal cancer is a rare adrenal tumor that accounts for only 0.2% of deaths due to cancer. The incidence has been estimated at 2 per million people per year. […] Approximately 50% of these tumors are functioning and produce hormonal and metabolic syndromes leading to their discovery. The other 50% are silent and discovered only when they are so large that they produce localized abdominal symptoms or when they metastasize. […] An exception to this age distribution occurs in southern Brazil, where the annual incidence of adrenal cancer in children is unusually high, ranging from 3.4-4.2 per million children, compared with a worldwide incidence of 0.3 per million children younger than 15. […] The cause of adrenal cancer is unknown but studies in the past five years suggest genetic mutations in the adrenal gland leading to the initiation of a malignant tumor.

• #34 Overview of endocrine tumor syndromes manifesting as adrenal tumors

  https://e-emj.org/journal/view.php?number=16

  Adrenocortical carcinomas, however, are rare, with an incidence of 0.2 to 0.3 cases per million in adults. […] Most adrenocortical tumors in adults are discovered incidentally, without excess adrenal hormone production, although 15% are functioning tumors. […] In pediatric cases, androgen excess is the commonly observed presentation, followed by Cushing syndrome or a combination of both. […] Therefore, clinical suspicion of adrenal tumors is warranted when patients present with symptoms of virilization and Cushing syndrome. […] Notably, adrenocortical carcinomas are more common in pediatric cases than in adults. […] Therefore, when diagnosing adrenocortical carcinoma in pediatric patients, the possibility of familial cancer syndromes must be considered. […] The incidence of germline TP53 mutations is particularly high in those diagnosed with adrenocortical carcinoma during childhood, with the rate decreasing from 58% in those diagnosed before the age of 12% to 25% in those diagnosed between the ages of 12 and 20.

• #35 Overview of endocrine tumor syndromes manifesting as adrenal tumors

  https://e-emj.org/journal/view.php?number=16

  Adrenocortical carcinomas, however, are rare, with an incidence of 0.2 to 0.3 cases per million in adults. […] Most adrenocortical tumors in adults are discovered incidentally, without excess adrenal hormone production, although 15% are functioning tumors. […] In pediatric cases, androgen excess is the commonly observed presentation, followed by Cushing syndrome or a combination of both. […] Therefore, clinical suspicion of adrenal tumors is warranted when patients present with symptoms of virilization and Cushing syndrome. […] Notably, adrenocortical carcinomas are more common in pediatric cases than in adults. […] Therefore, when diagnosing adrenocortical carcinoma in pediatric patients, the possibility of familial cancer syndromes must be considered. […] The incidence of germline TP53 mutations is particularly high in those diagnosed with adrenocortical carcinoma during childhood, with the rate decreasing from 58% in those diagnosed before the age of 12% to 25% in those diagnosed between the ages of 12 and 20.

• #36 Epidemiology of Adrenal Tumors – a Population-based Study in Olmsted County, Minnesota

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7601441/

  Adrenal tumors are commonly encountered in clinical practice, but current epidemiological data mainly originate from referral centers. We aimed to determine incidence rates (SIR), prevalence, and rates of malignancy and hormone excess in a standardized setting. […] An adrenal tumor was diagnosed in 1,287 patients (median age 62 years; 713 (554%) were women, and 13 (10%) were children). SIR increased from 44 (95%CI 0386) per 100,000 person-years in 1995 to 478 (95%CI 369587) in 2017, mainly due to incidental discovery of adenomas 40mm in 40-year-olds. Prevalence of adrenal tumors in 2017 was 532/100,000 inhabitants, ranging from 13/100,000 in children to 1,900/100,000 among 65-year-olds. Of 1,287 patients, 111 (86%) were diagnosed with malignancy (96 (75%) metastases), 14 (11%) with pheochromocytoma, and 53 (41%) with overt steroid hormone excess. Malignancy was more common in children (62% vs 8% in 18-year-olds, P0001), tumors discovered non-incidentally (32% vs 3% in incidentalomas, P0001), tumors 4 cm (34% vs 7% in 4 cm, P0001), tumors with unenhanced CT attenuation 20 Hounsfield units (15% vs 1% for 20 Hounsfield units, P0001), and bilateral masses (16% vs 7% for unilateral, P=0004). […] Adrenal tumor SIR increased 10 times from 1995 to 2017. Population-based data revealed lower rates of malignancy, pheochromocytoma, and overt steroid hormone excess than previously reported.

• #37 A Rare But Challenging Disease – CSRF – Cushing’s Support & Research Foundation

  https://csrf.net/understanding-cushings/adrenal-cancer/a-rare-but-challenging-disease/

  The result of therapy for adrenal cancer is generally poor but there are a significant number of patients on whom therapy can extend life expectancy with acceptable side effects. […] In a study of 49 patients with adrenal cancer, surgical excision offered the best opportunity for prolonged survival; 43 % of patients with a completely resectable tumor were alive with no evidence of disease an average of 7.3 years after surgery. […] A hormonal profile should be determined in every patient with an adrenal mass and especially patients who may have a primary adrenal cancer.

• #38 Childhood Adrenocortical Carcinoma Treatment (PDQ®) – NCI

  https://www.cancer.gov/types/adrenocortical/hp/child-adrenocortical-treatment-pdq

  Predisposing genetic factors have been implicated in more than 50% of adrenocortical carcinoma cases in North America and Europe and in 95% of cases in Brazil. Germline TP53 pathogenic variants are almost always the predisposing factor for adrenocortical tumors. The likelihood of a TP53 germline pathogenic variant is highest in the first years of life and diminishes with age. […] Patients with Beckwith-Wiedemann and hemihypertrophy syndromes have a predisposition to cancer, and as many as 16% of their neoplasms are adrenocortical tumors. […] The overall probability of 5-year survival for children with adrenocortical tumors depends on stage. Survival rates range from greater than 80% for patients with resectable disease to less than 20% for patients with metastases. Overall, adverse prognostic factors for adrenocortical carcinoma include the following: Presence of somatic TP53 variants; Larger tumor size; Disease Stage; Age older than 4 or 5 years; Microscopic tumor necrosis; Para-aortic lymph node involvement; Incomplete resection or spillage during surgery; Low HLA class II antigen expression; Higher Ki-67 labeling index; Grade, Resection status, Age, and Symptom (GRAS) score. […] Tumor staging is performed using the tumor-node-metastasis (TNM) classification system of the American Joint Committee on Cancer (AJCC).

• #39 Epidemiology and treatment of adrenal cancer. a case series | ECE2024 | 26th European Congress of Endocrinology | Endocrine Abstracts

  https://www.endocrine-abstracts.org/ea/0099/ea0099ep767

  Epidemiology and treatment of adrenal cancer. a case series […] Adrenal cancer is a rare, highly invasive malignant tumour with the incidence estimated for approximately 0,5-2,0 cases per milion people annually. […] Considering the fact that adrenal cancer is very sporadic not much data on its epidemiology is available. […] The aim of the study was to present the single-center data on the epidemiology of the adrenal cancer. […] In ENSAT staging classification for adrenocortical carcinoma, four patients had ENSAT II stage; seven patients had stage III; three patients had stage IV. […] Adrenal cancer is a neoplasm with varied clinical presentation. It demands specific diagnostic tools and treatment protocols. Therefore, treatment should be conducted in the high-reference centers.

• #40 Epidemiology and treatment of adrenal cancer. a case series | ECE2024 | 26th European Congress of Endocrinology | Endocrine Abstracts

  https://www.endocrine-abstracts.org/ea/0099/ea0099ep767

  Epidemiology and treatment of adrenal cancer. a case series […] Adrenal cancer is a rare, highly invasive malignant tumour with the incidence estimated for approximately 0,5-2,0 cases per milion people annually. […] Considering the fact that adrenal cancer is very sporadic not much data on its epidemiology is available. […] The aim of the study was to present the single-center data on the epidemiology of the adrenal cancer. […] In ENSAT staging classification for adrenocortical carcinoma, four patients had ENSAT II stage; seven patients had stage III; three patients had stage IV. […] Adrenal cancer is a neoplasm with varied clinical presentation. It demands specific diagnostic tools and treatment protocols. Therefore, treatment should be conducted in the high-reference centers.

• #41 Adrenal Cancer Survival Rates

  https://www.healthline.com/health/cancer/adrenal-cancer-survival-rate

  Keep in mind that these numbers apply only to the stage of the cancer when it was first diagnosed. They do not account for individual factors such as age, overall health, and response to treatment. […] Several factors can affect the treatment outcomes for someone with adrenal cancer, including: Cancer stage: Treatment is often more successful when adrenal cancer has not spread and is diagnosed at an early stage. […] If adrenal cancer is found early, theres often a good chance it can be cured. However, early detection is difficult because adrenal cancer is rare and does not have specific screening guidelines. […] Adrenal cancer is often aggressive and spreads quickly. […] Adrenal cancer is an aggressive cancer thats usually not diagnosed until its later stages. If diagnosed early, adrenal cancer has a good chance of being curable. However, the overall survival rate for adrenal cancer is around 50%. […] Survival rates for adrenal cancer depend on many factors, including the cancers stage at diagnosis, a persons overall health, and the cancers response to treatment.

• #42 Adrenocortical Carcinoma > Fact Sheets > Yale Medicine

  https://www.yalemedicine.org/conditions/adrenocortical-carcinoma

  Adrenocortical carcinoma is very rare. It accounts for about 1% of all cancer diagnoses among children, and between 0.02 and 0.2% of all cancer diagnoses among adults. It occurs more frequently in females than in males. […] Treatments are not always as effective for adrenocortical carcinoma as they are for other types of cancer, so early diagnosis and treatment are especially important in helping to improve outcomes. […] Because adrenocortical carcinoma is such a rare cancer, reported survival rates may be underestimated. […] Adrenocortical carcinoma is best treated by endocrinologists, endocrine oncology specialists, endocrine surgeons, and others who are familiar with this rare disease. […] Patients with adrenocortical carcinoma, a rare malignant lesion, have a poor prognosis. Two determinants of long-term survival are the tumor stage at presentation and curative resection by an experienced surgeon. […] However, overall 5-year survival rates can be as high as 45%.

• #43 Adrenal tumor: Outlook and more

  https://www.medicalnewstoday.com/articles/how-serious-is-a-tumor-on-the-adrenal-gland

  Adrenal gland tumors may be cancerous or benign. Most adrenal tumors are benign, but can produce excess levels of certain hormones. Cancerous adrenal tumors will require treatment or removal. […] For adrenal cancer, the outlook may depend on whether cancer has spread beyond the adrenal gland to the lymph nodes or to distant areas of the body. […] The American Cancer Society (ACS) uses a 5-year relative survival rate to assess the outlook for cancer. This compares survival data from those with the same type and stage of cancer to data from people in the overall population. […] According to the ACS, the 5-year relative survival rates for people with a diagnosis of adrenal gland cancer between 2012-2018 were as follows: Localized: 73%, Regional: 53%, Distant: 38%, All stages combined: 50%.

• #44 A Rare But Challenging Disease – CSRF – Cushing’s Support & Research Foundation

  https://csrf.net/understanding-cushings/adrenal-cancer/a-rare-but-challenging-disease/

  Adrenal cancer occasionally develops in families with susceptibility for other types of cancer. Environmental factors have been implicated in southern Brazil because the distribution of the tumors follows a regional rather than familial pattern. […] Patients with clinical and imaging diagnosis of an adrenal cancer should undergo staging of their disease by appropriate imaging procedures. Staging not only determines prognosis but also the selection of treatment. […] The stage at which an adrenal cortical carcinoma is defined determines prognosis for life expectancy. […] While 50% of patients in stages I, II or III are alive 40 months after diagnosis, only 10% of patients in stage IV are alive at that time. […] Large-scale clinical trials are needed to establish consensus treatment guidelines for patients with state III and IV adrenal cancer.

• #45 Childhood Adrenocortical Carcinoma Treatment (PDQ®) – NCI

  https://www.cancer.gov/types/adrenocortical/hp/child-adrenocortical-treatment-pdq

  Predisposing genetic factors have been implicated in more than 50% of adrenocortical carcinoma cases in North America and Europe and in 95% of cases in Brazil. Germline TP53 pathogenic variants are almost always the predisposing factor for adrenocortical tumors. The likelihood of a TP53 germline pathogenic variant is highest in the first years of life and diminishes with age. […] Patients with Beckwith-Wiedemann and hemihypertrophy syndromes have a predisposition to cancer, and as many as 16% of their neoplasms are adrenocortical tumors. […] The overall probability of 5-year survival for children with adrenocortical tumors depends on stage. Survival rates range from greater than 80% for patients with resectable disease to less than 20% for patients with metastases. Overall, adverse prognostic factors for adrenocortical carcinoma include the following: Presence of somatic TP53 variants; Larger tumor size; Disease Stage; Age older than 4 or 5 years; Microscopic tumor necrosis; Para-aortic lymph node involvement; Incomplete resection or spillage during surgery; Low HLA class II antigen expression; Higher Ki-67 labeling index; Grade, Resection status, Age, and Symptom (GRAS) score. […] Tumor staging is performed using the tumor-node-metastasis (TNM) classification system of the American Joint Committee on Cancer (AJCC).

• #46 Childhood Adrenocortical Carcinoma Treatment (PDQ®) – NCI

  https://www.cancer.gov/types/adrenocortical/hp/child-adrenocortical-treatment-pdq

  Predisposing genetic factors have been implicated in more than 50% of adrenocortical carcinoma cases in North America and Europe and in 95% of cases in Brazil. Germline TP53 pathogenic variants are almost always the predisposing factor for adrenocortical tumors. The likelihood of a TP53 germline pathogenic variant is highest in the first years of life and diminishes with age. […] Patients with Beckwith-Wiedemann and hemihypertrophy syndromes have a predisposition to cancer, and as many as 16% of their neoplasms are adrenocortical tumors. […] The overall probability of 5-year survival for children with adrenocortical tumors depends on stage. Survival rates range from greater than 80% for patients with resectable disease to less than 20% for patients with metastases. Overall, adverse prognostic factors for adrenocortical carcinoma include the following: Presence of somatic TP53 variants; Larger tumor size; Disease Stage; Age older than 4 or 5 years; Microscopic tumor necrosis; Para-aortic lymph node involvement; Incomplete resection or spillage during surgery; Low HLA class II antigen expression; Higher Ki-67 labeling index; Grade, Resection status, Age, and Symptom (GRAS) score. […] Tumor staging is performed using the tumor-node-metastasis (TNM) classification system of the American Joint Committee on Cancer (AJCC).

• #47 Conditional survival among patients with adrenal cortical carcinoma determined using a national population-based surveillance, epidemiology, and end results… | Oncotarget

  https://www.oncotarget.com/article/5831/text/

  The median overall survival (OS) among the entire cohort was 51 (4370) months. One-year and 5-year OS rates were 81.4% and 47.2%, respectively. […] CS estimates are more encouraging than static survival probabilities. Patients who had survived 24 months after their initial diagnosis had a better 1-year CS than those who had survived 24 months since diagnosis. Age, tumor stage, marital status and tumor grade also significantly affected CS. […] Although disease stage remains an important prognostic factor, earlier studies showed that between-stage differences in CS at diagnosis decrease with time from diagnosis, or even disappear. We found that for ACC between-stage differences in CS disappeared as follow-up became longer, and we observed a similar pattern for tumor grades. […] SEER data provide strong clinical insight into survival among patients in the U.S. with rare malignancies, including ACC.

• #48 Conditional survival among patients with adrenal cortical carcinoma determined using a national population-based surveillance, epidemiology, and end results… | Oncotarget

  https://www.oncotarget.com/article/5831/text/

  The median overall survival (OS) among the entire cohort was 51 (4370) months. One-year and 5-year OS rates were 81.4% and 47.2%, respectively. […] CS estimates are more encouraging than static survival probabilities. Patients who had survived 24 months after their initial diagnosis had a better 1-year CS than those who had survived 24 months since diagnosis. Age, tumor stage, marital status and tumor grade also significantly affected CS. […] Although disease stage remains an important prognostic factor, earlier studies showed that between-stage differences in CS at diagnosis decrease with time from diagnosis, or even disappear. We found that for ACC between-stage differences in CS disappeared as follow-up became longer, and we observed a similar pattern for tumor grades. […] SEER data provide strong clinical insight into survival among patients in the U.S. with rare malignancies, including ACC.

• #49 Follow-up after treatment for adrenal gland cancer | Canadian Cancer Society

  https://cancer.ca/en/cancer-information/cancer-types/adrenal-gland/treatment/follow-up

  Follow-up care lets your healthcare team keep track of your health for a period of time after treatment ends. This important part of cancer care is often shared among your surgeon, endocrinologist (a doctor who specializes in diseases of the endocrine system) and your family doctor. They will help you recover from treatment side effects and monitor you for any signs that the cancer has come back (recurred). […] Adrenal gland cancer can come back (recur) at any time, so close follow-up is needed. […] Follow-up visits for adrenocortical carcinoma are usually scheduled: 2 to 6 weeks after surgery to check hormone levels in the blood, every 3 months for the first 2 or 3 years, every 6 months for the next 5 years. […] Follow-up visits for pheochromocytoma are usually scheduled: 2 weeks after surgery to check hormone levels in the blood or urine, 3 to 12 months after surgery to check for a recurrence on imaging tests, 6 to 12 months for the first 3 years, every year for the rest of your life.

• #50 Adrenocortical carcinoma: a literature review – Thampi – Translational Cancer Research

  https://tcr.amegroups.org/article/view/34606/html

  At the time of presentation, a detailed history taking with attention to the history of symptoms of hormonal excess and family history of possible hereditary influence is the first step of evaluation. It is followed by a thorough physical examination for evaluation of ACC. Management of ACC poses a unique challenge as it involves oncologic and endocrine issues. […] Patients should be followed up every 3 months during and after initial treatment. After the first 2 years, the imaging interval is increased. Follow-up is recommended up to 10 years after the surgical resection of the primary tumor. Genetic counseling is recommended, as ACC is associated with a germline mutation in TP53 and Li-Fraumeni syndrome, which includes MLH1, MSH2, MSH6, PMS2 mutations.

• #51 Current prospects of hereditary adrenal tumors: towards better clinical management | Hereditary Cancer in Clinical Practice | Full Text

  https://hccpjournal.biomedcentral.com/articles/10.1186/s13053-024-00276-6

  The clinical and genomic features of representative hereditary tumors associated with adrenal tumors are presented in Table 1, and the available surveillance strategies proposed in the guidelines or expert statements are listed in Table 2. […] The National Comprehensive Cancer Network (NCCN) guidelines for neuroendocrine and adrenal tumors include LFS, Lynch syndrome (LS), multiple endocrine neoplasia type 1 (MEN1), and familial adenomatous polyposis (APC) as hereditary cancer predisposition syndromes associated with ACC. […] The clinical evidence for routine ACC screening is insufficient for individuals with LS. […] An international consensus statement for the clinical management of BWS mentioned a screening strategy using clinical evaluation, adrenal ultrasound, and evaluating serum dehydroepiandrosterone sulfate levels every 4-6 months.

• #52 Overview of endocrine tumor syndromes manifesting as adrenal tumors

  https://e-emj.org/journal/view.php?number=16

  The overall tumor risk associated with BWS is approximately 5%10%, with adrenocortical carcinoma accounting for about 7% of malignancies. […] For patients with BWS, screening for adrenal tumors is recommended, including clinical evaluations for hormone excess, adrenal ultrasound, and monitoring serum dihydroepiandrosterone sulfate levels every 4 to 6 months. […] Adrenocortical tumors are rare in the pediatric population. […] For adrenal screening in MEN1, abdominal imaging with CT or MRI is recommended every 3 years, and adrenal lesions require ongoing radiologic surveillance for signs of malignancy. […] The typical age of diagnosis is usually between 40 and 60 years, and the condition is rare in children. […] These tumors are associated with hereditary cancer syndromes such as MEN2, neurofibromatosis type 1 (NF1), and von Hippel-Lindau (VHL) disease.

• #53 Adrenocortical carcinoma: a literature review – Thampi – Translational Cancer Research

  https://tcr.amegroups.org/article/view/34606/html

  At the time of presentation, a detailed history taking with attention to the history of symptoms of hormonal excess and family history of possible hereditary influence is the first step of evaluation. It is followed by a thorough physical examination for evaluation of ACC. Management of ACC poses a unique challenge as it involves oncologic and endocrine issues. […] Patients should be followed up every 3 months during and after initial treatment. After the first 2 years, the imaging interval is increased. Follow-up is recommended up to 10 years after the surgical resection of the primary tumor. Genetic counseling is recommended, as ACC is associated with a germline mutation in TP53 and Li-Fraumeni syndrome, which includes MLH1, MSH2, MSH6, PMS2 mutations.

• #54 Incidence of adrenal gland tumor as a second primary malignancy: SEER-based study in: Endocrine Connections Volume 7 Issue 10 (2018)

  https://ec.bioscientifica.com/view/journals/ec/7/10/EC-18-0304.xml

  Advances in cancer treatment achieved during the past decades have resulted in increased survival of most pediatric and adult patients that suffered from different adrenal tumor types. This article reviews the incidence and survival of adrenal gland tumors as second primary tumors, according to data from the Surveillance, Epidemiology, and End Results (SEER) database. […] Data for a total of 2,887,468 persons with cancer were reviewed; 117 of whom had suffered second primary adrenal tumors. The overall SIR of adrenal gland tumor as a second primary was 1.5. A high incidence ratio of the event was detected in specific primary tumor sites: hypopharynx (observed/expected (O/E)=44.6); other endocrine tissue (including the thymus) (O/E=38.3); small intestine (O/E=8.9); liver (O/E=8.7); stomach (O/E=5); nodal NHL (O/E=3.8); kidney and renal pelvis (O/E=3.2) and breast (O/E=1.8).

• #55 Incidence of adrenal gland tumor as a second primary malignancy: SEER-based study in: Endocrine Connections Volume 7 Issue 10 (2018)

  https://ec.bioscientifica.com/view/journals/ec/7/10/EC-18-0304.xml

  The overall SIR of adrenal gland tumors as second primaries was higher than expected (O/E=1.5, 95% CI=1.21.8). When divided according to age groups, the highest increase in risk was in these three groups: 3539, 5054 and 6569. Examining the incidence of events in relation to first primary tumor site showed significantly higher incidences in specific sites including the hypopharynx (O/E=44.6, 95% CI=9.2130.4); other endocrine tissue (including the thymus and excluding thyroid) (O/E=38.3, 95% CI=10.498.1); the small intestine (O/E=8.9, 95% CI=1.132); the liver (O/E=8.7, 95% CI=1.131.6); the stomach (O/E=5, 95% CI=114.5); nodal NHL (O/E=3.8, 95% CI=1.57.8) and the kidneys and renal pelvis (O/E=3.2, 95% CI=1.26.9). […] The risk of developing an adrenal malignancy as a second primary was higher than a first primary, taking into consideration the rarity of adrenal malignancies, and the sustained decline of cancer mortality in the United States that started from early and mid-1990s, which may play a role in increasing the risk of developing a subsequent cancer among primary cancers survivors.

• #56 Incidence of adrenal gland tumor as a second primary malignancy: SEER-based study in: Endocrine Connections Volume 7 Issue 10 (2018)

  https://ec.bioscientifica.com/view/journals/ec/7/10/EC-18-0304.xml

  Advances in cancer treatment achieved during the past decades have resulted in increased survival of most pediatric and adult patients that suffered from different adrenal tumor types. This article reviews the incidence and survival of adrenal gland tumors as second primary tumors, according to data from the Surveillance, Epidemiology, and End Results (SEER) database. […] Data for a total of 2,887,468 persons with cancer were reviewed; 117 of whom had suffered second primary adrenal tumors. The overall SIR of adrenal gland tumor as a second primary was 1.5. A high incidence ratio of the event was detected in specific primary tumor sites: hypopharynx (observed/expected (O/E)=44.6); other endocrine tissue (including the thymus) (O/E=38.3); small intestine (O/E=8.9); liver (O/E=8.7); stomach (O/E=5); nodal NHL (O/E=3.8); kidney and renal pelvis (O/E=3.2) and breast (O/E=1.8).

• #57 Incidence of adrenal gland tumor as a second primary malignancy: SEER-based study in: Endocrine Connections Volume 7 Issue 10 (2018)

  https://ec.bioscientifica.com/view/journals/ec/7/10/EC-18-0304.xml

  The overall SIR of adrenal gland tumors as second primaries was higher than expected (O/E=1.5, 95% CI=1.21.8). When divided according to age groups, the highest increase in risk was in these three groups: 3539, 5054 and 6569. Examining the incidence of events in relation to first primary tumor site showed significantly higher incidences in specific sites including the hypopharynx (O/E=44.6, 95% CI=9.2130.4); other endocrine tissue (including the thymus and excluding thyroid) (O/E=38.3, 95% CI=10.498.1); the small intestine (O/E=8.9, 95% CI=1.132); the liver (O/E=8.7, 95% CI=1.131.6); the stomach (O/E=5, 95% CI=114.5); nodal NHL (O/E=3.8, 95% CI=1.57.8) and the kidneys and renal pelvis (O/E=3.2, 95% CI=1.26.9). […] The risk of developing an adrenal malignancy as a second primary was higher than a first primary, taking into consideration the rarity of adrenal malignancies, and the sustained decline of cancer mortality in the United States that started from early and mid-1990s, which may play a role in increasing the risk of developing a subsequent cancer among primary cancers survivors.

• #58 Adrenal metastasis | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/adrenal-metastasis-1?lang=us

  Adrenal metastases are the most common malignant lesions involving the adrenal gland. Metastases are usually bilateral but may also be unilateral. Unilateral involvement is more prevalent on the left side (ratio of 1.5:1). […] They are present at autopsy in up to 27% of patients with known malignant epithelial tumors.

• #59 Adrenal metastasis | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/adrenal-metastasis-1?lang=us

  Adrenal metastases are the most common malignant lesions involving the adrenal gland. Metastases are usually bilateral but may also be unilateral. Unilateral involvement is more prevalent on the left side (ratio of 1.5:1). […] They are present at autopsy in up to 27% of patients with known malignant epithelial tumors.

• #60

  https://link.springer.com/article/10.1007/s00268-020-05479-1

  To evaluate the incidence of adrenal metastases in patient with colorectal cancer (CRC) and determine the clinical and radiographic features associated metastatic CRC to the adrenal glands. […] The review of consecutive adults with newly diagnosed CRC found to have adrenal tumors 1 cm in size on staging or surveillance CT scans with at least two scans to evaluate progression or stability of disease. […] Fifty-eight of 856 (6.8%) CRC patients had an adrenal tumor. […] On univariate analysis, patients with metastatic CRC had larger adrenal tumors (26.7 mm vs 12.4 mm, p 0.01), a higher mean CEA (239 ng/mL vs 14.2 ng/mL, p=0.03), and were more likely to have other sites of metastatic disease seen on imaging 8/43 (19%) vs 14/15 (93%), p 0.01. […] On multivariable analysis, adrenal tumor size 1.8 cm (OR 49.6 CI 8306), CEA 2.5 ng/mL (OR 15.8 CI 1.7144) and other metastatic disease seen on imaging (OR 68.1 CI 7661) were independently associated with adrenal metastases. […] CRC patients with small adrenal tumors, normal CEA levels and no evidence of other metastatic disease are unlikely to have spread to the adrenal glands. Adrenal tumors found during staging and surveillance of CRC patients should be evaluated with appropriate imaging and biochemical analysis.

• #61

  https://link.springer.com/article/10.1007/s00268-020-05479-1

  To evaluate the incidence of adrenal metastases in patient with colorectal cancer (CRC) and determine the clinical and radiographic features associated metastatic CRC to the adrenal glands. […] The review of consecutive adults with newly diagnosed CRC found to have adrenal tumors 1 cm in size on staging or surveillance CT scans with at least two scans to evaluate progression or stability of disease. […] Fifty-eight of 856 (6.8%) CRC patients had an adrenal tumor. […] On univariate analysis, patients with metastatic CRC had larger adrenal tumors (26.7 mm vs 12.4 mm, p 0.01), a higher mean CEA (239 ng/mL vs 14.2 ng/mL, p=0.03), and were more likely to have other sites of metastatic disease seen on imaging 8/43 (19%) vs 14/15 (93%), p 0.01. […] On multivariable analysis, adrenal tumor size 1.8 cm (OR 49.6 CI 8306), CEA 2.5 ng/mL (OR 15.8 CI 1.7144) and other metastatic disease seen on imaging (OR 68.1 CI 7661) were independently associated with adrenal metastases. […] CRC patients with small adrenal tumors, normal CEA levels and no evidence of other metastatic disease are unlikely to have spread to the adrenal glands. Adrenal tumors found during staging and surveillance of CRC patients should be evaluated with appropriate imaging and biochemical analysis.

• #62 Adrenal Cancer | Center for Urologic Care of Berks County

  https://www.centerforurologiccare.com/patient-education/adrenal-cancer/

  Adrenal cancer is quite rare, usually affecting people in their first and fifth decades of life.1 […] Women are affected more often than men.1 […] Most cases of adrenal gland tumors are adrenocortical carcinomas.2 […] Although very little data is available about the causes of adrenal cancers, research has highlighted the presence of genetic predisposition.5 […] Other studies have tried finding a relationship between smoking tobacco and adrenal cancers,6 but further studies are needed to validate this idea. […] Spread of cancers from other areas of the body to the adrenal gland is much more common than cancer primarily originating there, it is important to keep this information in mind when making a diagnosis.9

• #63 Azthena logo with the word Azthena

  https://www.news-medical.net/news/20230825/Study-shows-not-all-adrenal-carcinoma-patients-need-mitotane-after-surgery.aspx

  After the complete tumor resection, not all patients with an adrenocortical carcinoma require the previous standard therapy Mitotane. […] In a new clinical study published in August 2023 in the journal The Lancet Diabetes Endocrinology, he and Massimo Terzolo and other collaborators found that the adjuvant treatment with Mitotane is not necessary if the patients fulfill three factors. […] ADIUVO is the first-ever randomized trial worldwide of adjuvant treatment for adrenocortical carcinoma. […] The efficacy of Mitotane versus surveillance only was assessed by recurrence-free survival (RFS). […] Martin Fassnacht sums up: „Concomitant therapy with Mitotane is not indicated in patients with low-grade, localized adrenocortical carcinoma in which the tumor has not yet metastasized and could be completely removed, as their prognoses are relatively good and treatment with Mitotane does not show a statistically significant improvement in the relapse rate, but is associated with side effects.” […] Endocrinology at Wrzburg University Hospital is considered an international reference center for the diagnosis, treatment and research of adrenocortical carcinoma and is currently the largest center worldwide. […] In Germany, it is estimated that there are about 80 to 120 new cases every year.

• #64 Azthena logo with the word Azthena

  https://www.news-medical.net/news/20230825/Study-shows-not-all-adrenal-carcinoma-patients-need-mitotane-after-surgery.aspx

  After the complete tumor resection, not all patients with an adrenocortical carcinoma require the previous standard therapy Mitotane. […] In a new clinical study published in August 2023 in the journal The Lancet Diabetes Endocrinology, he and Massimo Terzolo and other collaborators found that the adjuvant treatment with Mitotane is not necessary if the patients fulfill three factors. […] ADIUVO is the first-ever randomized trial worldwide of adjuvant treatment for adrenocortical carcinoma. […] The efficacy of Mitotane versus surveillance only was assessed by recurrence-free survival (RFS). […] Martin Fassnacht sums up: „Concomitant therapy with Mitotane is not indicated in patients with low-grade, localized adrenocortical carcinoma in which the tumor has not yet metastasized and could be completely removed, as their prognoses are relatively good and treatment with Mitotane does not show a statistically significant improvement in the relapse rate, but is associated with side effects.” […] Endocrinology at Wrzburg University Hospital is considered an international reference center for the diagnosis, treatment and research of adrenocortical carcinoma and is currently the largest center worldwide. […] In Germany, it is estimated that there are about 80 to 120 new cases every year.

• #65 Recent Updates on the Management of Adrenal Incidentalomas

  https://www.e-enm.org/journal/view.php?number=2413

  Adrenal incidentalomas represent an increasingly common clinical conundrum with significant implications for patients. The revised 2023 European Society of Endocrinology (ESE) guideline incorporates cutting-edge evidence for managing adrenal incidentalomas. […] The 2023 ESE guideline has updated the previous 2016 version, specifically in terms of the assessment of malignancy risk, the definition and management of mild autonomous cortisol secretion (MACS), and the follow-up strategies for adrenal tumors. […] The 2023 ESE guideline defines MACS as cases where serum cortisol levels after the 1-mg DST exceed 1.8 g/dL without any further stratification by degree of post-dexamethasone cortisol level. […] The 2023 ESE guideline recommends a multidisciplinary team approach to determine the necessity of surgical intervention in MACS patients.

• #66 Recent Updates on the Management of Adrenal Incidentalomas

  https://www.e-enm.org/journal/view.php?number=2413

  Adrenal incidentalomas represent an increasingly common clinical conundrum with significant implications for patients. The revised 2023 European Society of Endocrinology (ESE) guideline incorporates cutting-edge evidence for managing adrenal incidentalomas. […] The 2023 ESE guideline has updated the previous 2016 version, specifically in terms of the assessment of malignancy risk, the definition and management of mild autonomous cortisol secretion (MACS), and the follow-up strategies for adrenal tumors. […] The 2023 ESE guideline defines MACS as cases where serum cortisol levels after the 1-mg DST exceed 1.8 g/dL without any further stratification by degree of post-dexamethasone cortisol level. […] The 2023 ESE guideline recommends a multidisciplinary team approach to determine the necessity of surgical intervention in MACS patients.

• #67 Overview of endocrine tumor syndromes manifesting as adrenal tumors

  https://www.e-emj.org/journal/view.php?number=16

  Therefore, when diagnosing adrenocortical carcinoma in pediatric patients, the possibility of familial cancer syndromes must be considered. […] For adrenal screening in MEN1, abdominal imaging with CT or MRI is recommended every 3 years, and adrenal lesions require ongoing radiologic surveillance for signs of malignancy. […] The growth of adrenal nodules is related to the activation of the cAMP/protein kinase A (PKA) pathway. […] These findings underscore the significant incidence of PHEO and PGL in patients with VHL and highlight the necessity for early and lifelong screening. […] In addition to abdominal imaging, annual testing of plasma metanephrines and plasma normetanephrines is recommended starting at the age of 5. […] This approach is essential as it informs not only the management of the current tumor but also the monitoring for other neoplasms associated with these syndromes. […] Over the past 20 years, significant progress has been made in understanding the syndromes that lead to endocrine tumors, greatly improving the diagnostic and therapeutic approaches available to clinicians.

• #68 Overview of endocrine tumor syndromes manifesting as adrenal tumors

  https://www.e-emj.org/journal/view.php?number=16

  Therefore, when diagnosing adrenocortical carcinoma in pediatric patients, the possibility of familial cancer syndromes must be considered. […] For adrenal screening in MEN1, abdominal imaging with CT or MRI is recommended every 3 years, and adrenal lesions require ongoing radiologic surveillance for signs of malignancy. […] The growth of adrenal nodules is related to the activation of the cAMP/protein kinase A (PKA) pathway. […] These findings underscore the significant incidence of PHEO and PGL in patients with VHL and highlight the necessity for early and lifelong screening. […] In addition to abdominal imaging, annual testing of plasma metanephrines and plasma normetanephrines is recommended starting at the age of 5. […] This approach is essential as it informs not only the management of the current tumor but also the monitoring for other neoplasms associated with these syndromes. […] Over the past 20 years, significant progress has been made in understanding the syndromes that lead to endocrine tumors, greatly improving the diagnostic and therapeutic approaches available to clinicians.

• #69 Epidemiology and treatment of adrenal cancer. a case series | ECE2024 | 26th European Congress of Endocrinology | Endocrine Abstracts

  https://www.endocrine-abstracts.org/ea/0099/ea0099ep767

  Epidemiology and treatment of adrenal cancer. a case series […] Adrenal cancer is a rare, highly invasive malignant tumour with the incidence estimated for approximately 0,5-2,0 cases per milion people annually. […] Considering the fact that adrenal cancer is very sporadic not much data on its epidemiology is available. […] The aim of the study was to present the single-center data on the epidemiology of the adrenal cancer. […] In ENSAT staging classification for adrenocortical carcinoma, four patients had ENSAT II stage; seven patients had stage III; three patients had stage IV. […] Adrenal cancer is a neoplasm with varied clinical presentation. It demands specific diagnostic tools and treatment protocols. Therefore, treatment should be conducted in the high-reference centers.

• #70 Adrenal Carcinoma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/276264-overview

  AC occurs in 2 major peaks: in the first decade of life and again in the fourth to fifth decades. While, functional tumors are more common in children, however, nonfunctional tumors are more common in adults. […] Based on data from the International Pediatric Adrenocortical Tumor Registry, the median age at which children develop adrenal carcinomas is 3.2 years; 60% are younger than four years, and 14% are older than 13 years.

• #71 Adrenal Carcinoma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/276264-overview

  Adrenocortical carcinomas (ACs) are rare malignancies, with an annual incidence rate of only 0.72 cases per million population in Western countries. […] AC tumors are uncommon, having an incidence of approximately 0.6-1.67 cases per million persons per year. In southern Brazil, however, the incidence of adrenal tumors is 10-15 times that of the general population, a difference that has been associated with a mutation in the P53 gene. […] The female-to-male ratio for ACs is approximately 2.5-3:1. The accumulation of data, especially in international registries, revealed the incidence of adrenal tumors to be higher in female individuals than had previously been thought, particularly in those aged 0-3 years and those over 13 years. Nonfunctional ACs are distributed equally between the sexes.

• #72 Epidemiology of Adrenal Tumors – a Population-based Study in Olmsted County, Minnesota

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7601441/

  Adrenal tumors are commonly encountered in clinical practice, but current epidemiological data mainly originate from referral centers. We aimed to determine incidence rates (SIR), prevalence, and rates of malignancy and hormone excess in a standardized setting. […] An adrenal tumor was diagnosed in 1,287 patients (median age 62 years; 713 (554%) were women, and 13 (10%) were children). SIR increased from 44 (95%CI 0386) per 100,000 person-years in 1995 to 478 (95%CI 369587) in 2017, mainly due to incidental discovery of adenomas 40mm in 40-year-olds. Prevalence of adrenal tumors in 2017 was 532/100,000 inhabitants, ranging from 13/100,000 in children to 1,900/100,000 among 65-year-olds. Of 1,287 patients, 111 (86%) were diagnosed with malignancy (96 (75%) metastases), 14 (11%) with pheochromocytoma, and 53 (41%) with overt steroid hormone excess. Malignancy was more common in children (62% vs 8% in 18-year-olds, P0001), tumors discovered non-incidentally (32% vs 3% in incidentalomas, P0001), tumors 4 cm (34% vs 7% in 4 cm, P0001), tumors with unenhanced CT attenuation 20 Hounsfield units (15% vs 1% for 20 Hounsfield units, P0001), and bilateral masses (16% vs 7% for unilateral, P=0004). […] Adrenal tumor SIR increased 10 times from 1995 to 2017. Population-based data revealed lower rates of malignancy, pheochromocytoma, and overt steroid hormone excess than previously reported.

• #73 Science Saturday: All adrenal tumors should be investigated – Mayo Clinic News Network

  https://newsnetwork.mayoclinic.org/discussion/science-saturday-all-adrenal-tumors-should-be-investigated/

  „In this particular case, the question of how common the dangerous adrenal subtypes are out of all the adrenal incidentalomas is key,” says Dr. Ebbehøj. „For example, you see a patient with an adrenal incidentaloma. It does not appear to produce hormones, and it does not seem to be malignant. But should you recommend a follow-up CT scan and repeated biochemical testing to be sure, when you know this means extra radiation, patient anxiety and costs? Or should you recommend surgery to be on the safe side? We need good epidemiological data to design rational guidelines that can balance between benefits and harms.” […] „Our message is, again, to recognize the adrenal mass as a problem that needs full investigation at the time of discovery,” reiterates Dr. Bancos. […] This study is an example of descriptive epidemiology, examining incidence, prevalence, morbidity and mortality. It describes the scope of adrenal tumors, which helps shared decision-making with patients.

• #74 Current prospects of hereditary adrenal tumors: towards better clinical management | Hereditary Cancer in Clinical Practice | Full Text

  https://hccpjournal.biomedcentral.com/articles/10.1186/s13053-024-00276-6

  The clinical and genomic features of representative hereditary tumors associated with adrenal tumors are presented in Table 1, and the available surveillance strategies proposed in the guidelines or expert statements are listed in Table 2. […] The National Comprehensive Cancer Network (NCCN) guidelines for neuroendocrine and adrenal tumors include LFS, Lynch syndrome (LS), multiple endocrine neoplasia type 1 (MEN1), and familial adenomatous polyposis (APC) as hereditary cancer predisposition syndromes associated with ACC. […] The clinical evidence for routine ACC screening is insufficient for individuals with LS. […] An international consensus statement for the clinical management of BWS mentioned a screening strategy using clinical evaluation, adrenal ultrasound, and evaluating serum dehydroepiandrosterone sulfate levels every 4-6 months.

• #75 Adrenocortical Carcinoma > Fact Sheets > Yale Medicine

  https://www.yalemedicine.org/conditions/adrenocortical-carcinoma

  Adrenocortical carcinoma is very rare. It accounts for about 1% of all cancer diagnoses among children, and between 0.02 and 0.2% of all cancer diagnoses among adults. It occurs more frequently in females than in males. […] Treatments are not always as effective for adrenocortical carcinoma as they are for other types of cancer, so early diagnosis and treatment are especially important in helping to improve outcomes. […] Because adrenocortical carcinoma is such a rare cancer, reported survival rates may be underestimated. […] Adrenocortical carcinoma is best treated by endocrinologists, endocrine oncology specialists, endocrine surgeons, and others who are familiar with this rare disease. […] Patients with adrenocortical carcinoma, a rare malignant lesion, have a poor prognosis. Two determinants of long-term survival are the tumor stage at presentation and curative resection by an experienced surgeon. […] However, overall 5-year survival rates can be as high as 45%.

• #76 Epidemiology and treatment of adrenal cancer. a case series | ECE2024 | 26th European Congress of Endocrinology | Endocrine Abstracts

  https://www.endocrine-abstracts.org/ea/0099/ea0099ep767

  Epidemiology and treatment of adrenal cancer. a case series […] Adrenal cancer is a rare, highly invasive malignant tumour with the incidence estimated for approximately 0,5-2,0 cases per milion people annually. […] Considering the fact that adrenal cancer is very sporadic not much data on its epidemiology is available. […] The aim of the study was to present the single-center data on the epidemiology of the adrenal cancer. […] In ENSAT staging classification for adrenocortical carcinoma, four patients had ENSAT II stage; seven patients had stage III; three patients had stage IV. […] Adrenal cancer is a neoplasm with varied clinical presentation. It demands specific diagnostic tools and treatment protocols. Therefore, treatment should be conducted in the high-reference centers.

• #77 Recent Updates on the Management of Adrenal Incidentalomas

  https://www.e-enm.org/journal/view.php?number=2413

  The 2023 ESE guideline recommends that patients with MACS should receive perioperative stress-dose glucocorticoid treatment and should be followed up by an endocrinologist until the hypothalamic-pituitary-adrenal axis recovers. […] The management of adrenal incidentalomas has significantly advanced over the years, with updated guidelines aiming to optimize diagnostic processes and therapeutic interventions. The 2023 ESE guideline, in particular, highlights the need for individualized approaches over standardized ones, introducing key modifications in hormonal evaluations, the assessment of malignancy risk, and surgical treatment.

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