Materiały źródłowe

• #1 Risks for adrenal gland cancer | Canadian Cancer Society

  https://cancer.ca/en/cancer-information/cancer-types/adrenal-gland/risks

  Some things can affect your risk, or chance, of developing cancer. Certain behaviours, substances or conditions can increase or decrease the risk. Most cancers are the result of many risks. But sometimes cancer develops in people who don’t have any risks. […] Adrenal gland cancer is rare. Women are slightly more likely than men to develop adrenal gland cancer. We need more research and can’t say at this time what the risk is for transgender, non-binary and gender-diverse people. […] People who have certain genetic conditions have a higher than average risk for adrenal gland cancer. A genetic condition is a disease caused by a change (mutation) in one or more genes. About 15% of adrenocortical carcinomas (ACC), the most common type of adrenal gland cancer, are linked with a genetic condition. And about 30% of pheochromocytomas, the second most common type of adrenal gland tumour, are linked with a genetic condition.

• #2 What are Adrenal Tumors? Symptoms, Risk Factors & Treatments | MD Anderson Cancer Center

  https://www.mdanderson.org/cancer-types/adrenal-tumors.html

  Adrenal tumors can be malignant (cancer) or benign (not cancerous). Even benign adrenal tumors can be dangerous or cause uncomfortable symptoms. […] Adrenocortical cancer is rare, affecting only about 300 to 500 people each year in the United States. […] Overproduction of cortisol may be caused by: A benign tumor on the adrenal gland called an adenoma, An abnormality of the pituitary gland, usually a tumor (also called Cushings disease), Long-term use of corticosteroid medications (such as prednisone), Benign or malignant tumors in other areas of the body that produce adrenocorticotropic hormone (ACTH) (ectopic ACTH syndrome). […] Certain inherited disorders can increase your risk of having an adrenal disease. These include: Li-Fraumeni syndrome, Multiple Endocrine Neoplasia type 2 (MEN2), Von Hippel-Lindau disease (VHL), Neurofibromatosis type 1, also known as von Recklinghausens disease, Paraganglioma syndrome, a condition where tumors form in structures called paraganglia, Beckwith-Wiedemann syndrome, a type of overgrowth syndrome.

• #3 Adrenocortical Carcinoma: Symptoms, Stages & Prognosis

  https://my.clevelandclinic.org/health/diseases/6152-adrenocortical-carcinoma

  Adrenocortical carcinoma is a rare cancer in your adrenal cortex, the outer layer of your adrenal glands. […] Researchers dont know the exact cause. Some people develop adrenocortical carcinoma because they have inherited conditions that increase the risk that theyll develop the condition. […] In other cases, certain genetic mutations (changes) appear to increase risk. For example, research shows changes in the tumor suppressor genes TP53 and IGF2 appear to drive adrenocortical carcinoma. […] Having any of the following conditions increases your risk of developing adrenocortical carcinoma: Beckwith-Wiedemann syndrome, Carney complex, Familial adenomatous polyposis (FAP), Li-Fraumeni syndrome, Lynch syndrome, Multiple endocrine neoplasia (MEN1), Neurofibromatosis Type 1 (NF1), Von Hippel-Lindau (VHL) syndrome. […] Researchers know about half of all cases of adrenocortical carcinoma happen when certain genes mutate and create cancerous cells that multiply and become tumors. But they dont know what triggers those mutations, so they cant recommend ways you could prevent them from happening.

• #4 Adrenal Cancer Risk Factors | Genetics & Adrenal Cancer | American Cancer Society

  https://www.cancer.org/cancer/types/adrenal-cancer/causes-risks-prevention/risk-factors.html

  Most adrenal cortex cancers are not inherited (they are sporadic), but some (up to 15%) are caused by a genetic defect. This is more common in adrenal cancers in children. […] The Li-Fraumeni syndrome is a rare condition that is most often caused by a defect in the TP53 gene. People with this syndrome have a high risk of developing several types of cancer, including breast cancer, bone cancer, brain cancer, and adrenocortical cancer. […] People with Beckwith-Wiedemann syndrome have large tongues, are tall, and have an increased risk for developing cancers of the kidney, liver and adrenal cortex. […] People with MEN1 have a very high risk of developing tumors of 3 glands: the pituitary, parathyroid, and pancreas. About one-third to one-half of people with this condition also develop adrenal tumors.

• #5

  https://winshipcancer.emory.edu/cancer-types-and-treatments/adrenal-cancer/prevention.php

  While researchers are still determining the exact cause of adrenal cancer, the following cancer-prevention measures may help lower your risk for the disease. […] When considering adrenal cancer prevention, its important to understand what adrenal cancer is and what causes it. […] Researchers are still trying to determine what causes an adrenal tumor to become cancerous and to establish adrenal cancer prevention guidelines. So far, theyve determined that inherited genetic mutations account for up to 15% of all adrenal cancers. This is more likely to be the case when adrenal cancer is diagnosed in children. […] Its important to note, however, that the majority (85%) of all adrenal cancers are sporadic, or not caused by an underlying genetic condition.

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

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

  Cancers occur when DNA in cells undergoes some abnormal changes (mutations). These changes can be inherited or acquired during life. Exposure to harmful radiations or substances can increase the risk of mutations. […] Although very little data is available about the causes of adrenal cancers, research has highlighted the presence of genetic predisposition. Such familial cases mostly appear in childhood and are associated with the presence of certain familial syndromes. […] Other studies have tried finding a relationship between smoking tobacco and adrenal cancers, but further studies are needed to validate this idea.

• #7 What Causes Adrenal Cancer? | Adrenal Cancer Causes | American Cancer Society

  https://www.cancer.org/cancer/types/adrenal-cancer/causes-risks-prevention/what-causes.html

  We do not know exactly what causes most adrenal cancers. Over the past several years, there has been a lot of progress in understanding how certain changes in a person’s DNA can cause cells in the adrenal gland to become cancerous. […] Cancers can be caused by DNA mutations (changes) that turn on oncogenes or turn off tumor suppressor genes. Some people with cancer have inherited DNA mutations from a parent, which increase their risk for developing the disease. But most DNA mutations that are seen in cancers happen after birth rather than having been inherited. Some of these mutations may result from exposure to things like radiation or cancer-causing chemicals. But most of these mutations seem to happen for no apparent reason, without having an outside cause. […] The Li-Fraumeni syndrome is caused by inherited mutations that inactivate the TP53 tumor suppressor gene. This syndrome causes a small number of adrenal cancers in adults (about 1 of every 20), but it’s often the cause of adrenal cancer in children. In fact, about 8 of every 10 cases of adrenal cancer in children are caused by Li-Fraumeni syndrome. Many other adrenal cancers have also been found to have TP53 gene changes that were acquired after birth (not inherited).

• #8 Adrenal Cancer – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK546580/

  The chromosomal rearrangements present in benign and malignant adrenocortical tumors are similar; such as the alteration of the beta-catenin pathway. […] However, these chromosomal rearrangements are more frequent in the malignant tumors compared with the benign lesion. […] A multistep tumor progression of tumorigenesis has been proposed from genes that are known to be associated with hereditary tumor syndromes described below. […] Li-Fraumeni syndrome is a familial cancer syndrome arising from a germline mutation of the TP53 gene located at 17p13. […] Patients are susceptible to breast cancer, sarcomas, brain tumors, leukemia, and adrenocortical carcinoma. […] Variants of this disease may appear in children with sporadic ACC and germline p53 mutations without a typical history of Li-Fraumeni syndrome.

• #9 Adrenal Cancer – Urology | UCLA Health

  https://www.uclahealth.org/medical-services/urology/conditions-treated/cancer-conditions/adrenal-cancer

  Adrenal cancer is an extremely rare but potentially deadly malignancy that starts in the adrenal glands, most often causing hormone overproduction. […] It is unclear what causes adrenal cancer or what puts people at higher risk, although in a minority of instances genetic factors can come into play. Mutations in tumor suppressor genes, including Li-Fraumeni syndrome and type 1 multiple endocrine neoplasia (MEN1), increase the risk for adrenal and other cancers. […] Other familial syndromes associated with a higher risk for the disease include Gardner syndrome, Carney triad, Cowden syndrome, familial polyposis and Turcot syndrome.

• #10 Adrenal Cancer – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK546580/

  The transcriptome of tumors with alterations of ZNFR3 shows activation of b-catenin targets but milder than the level observed in CTNNB1-mutated ACC. […] ZNFR3 also constitutes a new tumor suppressor gene. […] By the sum of the CTNNB1- and ZNRF3-altered ACC, activation of the Wnt/b-catenin pathway could be present in 39% of ACC. […] Beckwith-Wiedemann syndrome (BWS) characteristically demonstrates genetic and epigenetic events at the 11p15.5 region involving the cyclin-dependent kinase inhibitor 1C (CDKN1C), insulin-growth-factor II (IGF-II), and H19 genes, which results in overgrowth disorders, visceromegaly (macroglossia, hemihyperplasia), malformations (wall defect, umbilical hernia), and predisposition to embryonal malignancies. […] ACC belongs to the BWS tumor spectrum, which also includes Wilms tumor, hepatoblastoma, rhabdomyosarcoma and neuroblastoma with an overall risk for tumor development in children estimated at 7.5%; most of the tumors occur in the first 8 to 10 years of life.

• #11 What Causes Adrenal Gland Tumors? | Roswell Park Comprehensive Cancer Center – Buffalo, NY

  https://www.roswellpark.org/cancer/adrenal/about/risk-factors

  Lynch syndrome (also called hereditary nonpolyposis colorectal cancer) increases risk for colorectal cancer and other gastrointestinal and urinary tract cancers. […] Multiple Endocrine Neoplasia Syndrome (MEN1 or MEN2). People with this syndrome are at increased risk for developing tumors or abnormal growth of the adrenal glands (pheochromocytomas), thyroid gland, parathyroid gland, oral cavity and intestinal lining. […] Neurofibromatosis type 1 leads to nerve tissue tumors in several body areas, including the adrenal glands. […] Von Hippel-Lindau (VHL) syndrome. People with VHL face increased risk for developing tumors in the adrenal glands (pheochromocytomas), kidneys, brain, eyes, pancreas and the epididimys gland found next to the testicles. […] Beckwith-Wiedemann Syndrome. A rare, overgrowth disorder involving increased risk for adrenal tumors, including in childhood. Babies with this syndrome are large at birth and may develop low blood sugar. Other common symptoms are a large tongue, large internal organs and defects of the abdominal wall near the navel.

• #12 Adrenal Cancer Risk Factors | Genetics & Adrenal Cancer | American Cancer Society

  https://www.cancer.org/cancer/types/adrenal-cancer/causes-risks-prevention/risk-factors.html

  Most adrenal cortex cancers are not inherited (they are sporadic), but some (up to 15%) are caused by a genetic defect. This is more common in adrenal cancers in children. […] The Li-Fraumeni syndrome is a rare condition that is most often caused by a defect in the TP53 gene. People with this syndrome have a high risk of developing several types of cancer, including breast cancer, bone cancer, brain cancer, and adrenocortical cancer. […] People with Beckwith-Wiedemann syndrome have large tongues, are tall, and have an increased risk for developing cancers of the kidney, liver and adrenal cortex. […] People with MEN1 have a very high risk of developing tumors of 3 glands: the pituitary, parathyroid, and pancreas. About one-third to one-half of people with this condition also develop adrenal tumors.

• #13 What Causes Adrenal Gland Tumors? | Roswell Park Comprehensive Cancer Center – Buffalo, NY

  https://www.roswellpark.org/cancer/adrenal/about/risk-factors

  Lynch syndrome (also called hereditary nonpolyposis colorectal cancer) increases risk for colorectal cancer and other gastrointestinal and urinary tract cancers. […] Multiple Endocrine Neoplasia Syndrome (MEN1 or MEN2). People with this syndrome are at increased risk for developing tumors or abnormal growth of the adrenal glands (pheochromocytomas), thyroid gland, parathyroid gland, oral cavity and intestinal lining. […] Neurofibromatosis type 1 leads to nerve tissue tumors in several body areas, including the adrenal glands. […] Von Hippel-Lindau (VHL) syndrome. People with VHL face increased risk for developing tumors in the adrenal glands (pheochromocytomas), kidneys, brain, eyes, pancreas and the epididimys gland found next to the testicles. […] Beckwith-Wiedemann Syndrome. A rare, overgrowth disorder involving increased risk for adrenal tumors, including in childhood. Babies with this syndrome are large at birth and may develop low blood sugar. Other common symptoms are a large tongue, large internal organs and defects of the abdominal wall near the navel.

• #14 Adrenal Cancer Risk Factors | Genetics & Adrenal Cancer | American Cancer Society

  https://www.cancer.org/cancer/types/adrenal-cancer/causes-risks-prevention/risk-factors.html

  Lynch syndrome (formerly known as HNPCC) is an inherited genetic disorder that increases the risk of colorectal cancer, stomach cancer, and some other cancers, including adrenocortical cancer. […] Risk factors such as excess body weight, living a sedentary lifestyle, and being exposed to cancer-causing substances in the environment can affect a person’s risk of many types of cancer. Smoking, in particular, has been suggested as a risk factor for adrenal cancer.

• #15 What Is Adrenal Carcinoma?

  https://www.webmd.com/cancer/adrenal-carcinoma

  When you have adrenal carcinoma (also called adrenal cancer or adrenocortical cancer) you have a cancerous tumor in your adrenal cortex. This is the outer part of your adrenal gland. Adrenal carcinoma is rare. Your doctors may be able to cure it if you catch it early. But its aggressive. That means it spreads quickly, and if it moves beyond the adrenal gland its much harder to treat. […] Doctors arent sure what causes adrenal cancer. It starts with a change in the genes of some adrenal cells, but that change seems to happen at random. […] It can run in families, so if anyone in your family has cancer in their adrenal glands or other parts of the endocrine system, tell your doctor. If you do get it, regular checkups can help you catch it early. […] When you have a genetic condition, you have a defect in one or more genes. Some genetic conditions may give you higher odds of getting adrenal cancer: Beckwith-Wiedemann syndrome, Familial adenomatous polyposis (FAP), Hereditary nonpolyposis colorectal cancer (HNPC), Li-Fraumeni syndrome, Multiple endocrine neoplasia (MEN1).

• #16 Adrenal Cancer – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK546580/

  Loss of retinoblastoma (Rb) protein has been found in 27% of aggressive adrenocortical carcinoma. […] This defect is related in most cases to mutations of the RB1 gene or its allelic loss. […] Another study revealed the presence of RB1 mutations in 7% of tumors. […] Another 11% of the tumors of this cohort harbored mutations of cyclin-dependent kinase inhibitor 2A (CDKN2A), and 2% exhibited high-level amplification of cyclin-dependent kinase inhibitor 4 (CDK4). […] Thus, overall, 33% of the tumors had alterations of the Tp53 pathway. […] Patients with familial adenomatous polyposis (FAP) or Gardner syndrome, which is a mutation of the adenomatous polyposis coli gene (APC) located in 5q21 chromosomal, present with multiple colonic polyps and an increased risk of early colon carcinomas.

• #17 What Causes Adrenal Gland Tumors? | Roswell Park Comprehensive Cancer Center – Buffalo, NY

  https://www.roswellpark.org/cancer/adrenal/about/risk-factors

  Like many cancers, an exact cause of the disease remains unclear. However, research shows that some people are more likely to develop adrenal cancer. In addition to age – older than 40 – people with certain genetic (hereditary) conditions are at higher risk for adrenal cancer. These conditions include: […] Familial adenomatous polyposis (FAP) causes the formation of many (more than 100) colon polyps at an early age. People with FAP have a high risk for developing colorectal cancer as well as cancer of the stomach, small intestine, pancreas and bile ducts, and tumors in the adrenal glands. […] Hereditary paraganglioma-pheochromocytoma (PGL/PCC) syndrome causes tumors to form in bunches of nerve cells outside of the adrenal gland called ganglia, while pheochromocytoma is a tumor type that forms in the adrenals. Both tumor types can overproduce adrenaline-type hormones.

• #18 Adrenal Cancer Causes, Risk Factors and Is It Hereditary

  https://www.cancercenter.com/cancer-types/adrenal-cancer/risk-factors

  About 600 people in the United States are diagnosed with adrenal cancer each year, making it a very rare disease. […] Although genetic syndromes and hereditary traits have been linked to adrenal cancer, most cases occur sporadically, and the causes of adrenal cancer remain largely unknown. […] Risk factors for adrenal cancer are varied. Like many cancer types, the risk for developing adrenal cancer may increase as a result of certain lifestyles and environmental factors, including smoking and exposure to carcinogens. Genetic syndromes have been linked to adrenal cancer. […] Genetic syndromes considered risk factors for adrenal cancer include those listed below. […] A mutation in the gene TP53 causes the inherited genetic condition called Li-Fraumeni syndrome. […] A rare genetic disorder, von Hippel-Lindau syndrome increases the risk of developing adrenal gland tumors and tumors in the liver, lungs, brain, spinal cord, eyes and inner ears.

• #19 Risks for adrenal gland cancer | Canadian Cancer Society

  https://cancer.ca/en/cancer-information/cancer-types/adrenal-gland/risks

  The following genetic conditions can increase your risk for adrenal gland cancer. If you have a genetic condition that increases your risk for adrenal gland cancer, you may need to visit your doctor more often. Talk to your doctor about your risk and if you need to have certain tests to check for adrenal gland cancer. […] Li-Fraumeni syndrome is an inherited condition that increases the risk of developing certain types of cancers, including ACC, breast cancer, brain tumours, soft tissue and bone sarcomas, and acute leukemia. […] Multiple endocrine neoplasia (MEN) is an inherited condition that increases the risk of developing endocrine system cancers. It is associated with tumours in more than one endocrine gland. […] Von Hippel-Lindau (VHL) syndrome is an inherited condition that affects blood vessels in different parts of the body, including the eyes, brain, spinal cord and adrenal glands. It makes the blood vessels grow abnormally.

• #20 Risks for adrenal gland cancer | Canadian Cancer Society

  https://cancer.ca/en/cancer-information/cancer-types/adrenal-gland/risks

  Neurofibromatosis type 1 is an inherited condition that affects the nervous system. It affects the development and growth of neurons (nerve cells), causes tumours (neurofibromas) to grow on nerves and may cause other abnormalities in muscles, bones and skin. […] Beckwith-Wiedemann syndrome is an inherited condition that affects how different parts of the body grow. […] Carney complex is an inherited condition that affects the skin and causes tumours in the heart, skin, nerves and endocrine glands (such as the thyroid, pituitary gland, testicles and ovaries). It also increases the risk of ACC. […] Familial adenomatous polyposis (FAP) is an inherited condition that causes hundreds to thousands of polyps to develop, mainly in the lining of the colon and rectum. […] Lynch syndrome is an inherited condition that causes a large number of polyps to develop in the lining of the colon and rectum, but not as many polyps as are found in FAP.

• #21 What Causes Adrenal Gland Tumors? | Roswell Park Comprehensive Cancer Center – Buffalo, NY

  https://www.roswellpark.org/cancer/adrenal/about/risk-factors

  Lynch syndrome (also called hereditary nonpolyposis colorectal cancer) increases risk for colorectal cancer and other gastrointestinal and urinary tract cancers. […] Multiple Endocrine Neoplasia Syndrome (MEN1 or MEN2). People with this syndrome are at increased risk for developing tumors or abnormal growth of the adrenal glands (pheochromocytomas), thyroid gland, parathyroid gland, oral cavity and intestinal lining. […] Neurofibromatosis type 1 leads to nerve tissue tumors in several body areas, including the adrenal glands. […] Von Hippel-Lindau (VHL) syndrome. People with VHL face increased risk for developing tumors in the adrenal glands (pheochromocytomas), kidneys, brain, eyes, pancreas and the epididimys gland found next to the testicles. […] Beckwith-Wiedemann Syndrome. A rare, overgrowth disorder involving increased risk for adrenal tumors, including in childhood. Babies with this syndrome are large at birth and may develop low blood sugar. Other common symptoms are a large tongue, large internal organs and defects of the abdominal wall near the navel.

• #22 What Causes Adrenal Gland Tumors? | Roswell Park Comprehensive Cancer Center – Buffalo, NY

  https://www.roswellpark.org/cancer/adrenal/about/risk-factors

  Carney complex. This rare disorder causes dark spots on the skin and tumors in the heart, skin, nerves and endocrine glands, including the adrenals. […] Li-Fraumeni Syndrome. This rare condition is caused by an alteration in the p53 tumor suppressor gene and is associated with a predisposition to multiple cancer types.

• #23 Risks for adrenal gland cancer | Canadian Cancer Society

  https://cancer.ca/en/cancer-information/cancer-types/adrenal-gland/risks

  Neurofibromatosis type 1 is an inherited condition that affects the nervous system. It affects the development and growth of neurons (nerve cells), causes tumours (neurofibromas) to grow on nerves and may cause other abnormalities in muscles, bones and skin. […] Beckwith-Wiedemann syndrome is an inherited condition that affects how different parts of the body grow. […] Carney complex is an inherited condition that affects the skin and causes tumours in the heart, skin, nerves and endocrine glands (such as the thyroid, pituitary gland, testicles and ovaries). It also increases the risk of ACC. […] Familial adenomatous polyposis (FAP) is an inherited condition that causes hundreds to thousands of polyps to develop, mainly in the lining of the colon and rectum. […] Lynch syndrome is an inherited condition that causes a large number of polyps to develop in the lining of the colon and rectum, but not as many polyps as are found in FAP.

• #24 Risks for adrenal gland cancer | Canadian Cancer Society

  https://cancer.ca/en/cancer-information/cancer-types/adrenal-gland/risks

  Hereditary paraganglioma-pheochromocytoma syndrome is an inherited condition that causes one or more tumours to grow in paraganglia. Paraganglia are groups of cells that are found near nerve cell bunches called ganglia. A tumour in paraganglia is called a paraganglioma. Hereditary paraganglioma-pheochromocytoma syndrome increases the risk of pheochromocytoma, which is a type of paraganglioma that develops in the adrenal gland.

• #25 What Causes Adrenal Gland Tumors? | Roswell Park Comprehensive Cancer Center – Buffalo, NY

  https://www.roswellpark.org/cancer/adrenal/about/risk-factors

  Like many cancers, an exact cause of the disease remains unclear. However, research shows that some people are more likely to develop adrenal cancer. In addition to age – older than 40 – people with certain genetic (hereditary) conditions are at higher risk for adrenal cancer. These conditions include: […] Familial adenomatous polyposis (FAP) causes the formation of many (more than 100) colon polyps at an early age. People with FAP have a high risk for developing colorectal cancer as well as cancer of the stomach, small intestine, pancreas and bile ducts, and tumors in the adrenal glands. […] Hereditary paraganglioma-pheochromocytoma (PGL/PCC) syndrome causes tumors to form in bunches of nerve cells outside of the adrenal gland called ganglia, while pheochromocytoma is a tumor type that forms in the adrenals. Both tumor types can overproduce adrenaline-type hormones.

• #26 Adrenal Cancer – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK546580/

  Adrenocortical carcinomas can arise sporadically or be part of a familial cancer syndrome. […] Although the molecular mechanisms underlying tumorigenesis in many of the hereditary syndromes are well characterized, the molecular pathogenesis of sporadic ACC is less well understood. […] Furthermore, most cases of ACC appear to be sporadic. […] Several studies on the clonality of adrenocortical tumors have demonstrated that most benign adrenocortical lesions are polyclonal; however, some ACC that have been studied indicate monoclonal proliferation. […] Adrenocortical hyperplasia seems to be a polyclonal process; however, most adrenocortical tumors, whether benign or malignant, are monoclonal lesions, indicating that genetic changes at specific genomic loci may be playing a role in adrenal tumorigenesis.

• #27 Adrenal Carcinoma – Diagnosis & Disease Information

  https://www.cancertherapyadvisor.com/ddi/adrenal-carcinoma/

  The causes of adrenal carcinoma are not well understood, partially because the disease is extremely rare. Approximately 5% to 10% arise from certain hereditary diseases that increase the risk of developing adrenal carcinoma and other cancers. These syndromes include the following: Li-Fraumeni syndrome; Beckwith-Wiedemann syndrome; Birt-Hogg-Dub syndrome; Multiple endocrine neoplasia type 1; Adenomatous polyposis coli; and Lynch syndrome. […] Many cases of adrenal carcinoma arise from spontaneous somatic mutations. Two seminal studies, The European Network for the Study of Adrenal Tumors (ENS@T) and The Cancer Genome Atlas (TCGA) study, identified mutations commonly found in adrenal carcinoma. The processes and genes most commonly affected in adrenal carcinoma include the following: Wnt/-catenin signaling (ZNRF3 and CTNNB1); Cell cycle regulation (TP53, CDKN2A, RB1, and CDK); and Chromatin remodeling (MEN1 and DAXX).

• #28 Adrenal Cancer – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK546580/

  Furthermore, FAP is associated with pigmented retinal lesions, desmoids tumors, osteomas, thyroid adenoma/carcinomas, and other different malignant tumors. […] Adrenocortical tumors, especially nonfunctional nodular hyperplasia, cortisol-producing adenomas (CPA), and ACC occur in 7 to 13% of patients with FAP. […] FAP results from a germline inactivating mutation of APC, a tumor suppressor gene that inhibits Wnt/beta-catenin signaling. […] According to Knudsons model, ACC in patients with FAP exhibits somatic APC mutations as a second hit. […] The activation of the Wnt/b-catenin signaling pathway occurs in a third of ACC cases, and less commonly observed in adrenocortical adenoma. […] Activation of b-catenin is mainly related to the mutation of the Catenin beta-1 gene (CTNNB1). […] Consistently, transcriptome studies have shown an overexpression of Wnt/b-catenin target genes in ACC.

• #29 Adrenal Cancer – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK546580/

  Mutations of CTNNB1 or a histologic pattern of its activation are associated with poor outcomes. […] Somatic mutations in APC has a prevalence of 2 to 3%, suggesting the importance of somatic alterations of other mechanisms in the Wnt/beta-catenin pathway in causing ACC. […] Indeed, activating somatic mutations of beta-catenin itself have been reported with a prevalence of about 16% in large cohorts of ACC. […] Interestingly, p53 and beta-catenin mutations are frequent in ACC with poor prognosis but are almost mutually exclusive. […] Constitutive activation of beta-catenin in the adrenal cortex of transgenic mice resulted in progressive steroidogenic and undifferentiated spindle-shaped cell hyperplasia, which lead to macroscopic adenomas development. […] Malignant characteristics such as uncontrolled neovascularization and loco-regional metastatic invasion appeared only later in this context, suggesting the necessity of other associated genetic alterations.

• #30 Adrenal Cancer – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK546580/

  More recently, researchers identified a new actor with the Wnt/beta-catenin pathway involved in adrenal tumorigenesis. […] Zinc and ring finger 3 (ZNRF3), a cell-surface transmembrane E3 ubiquitin ligase that is a negative regulator of the Wnt/b-catenin pathway. […] ZNRF3 leads the Wingless-related integration site-low density lipoprotein receptor-related protein 6 (Wnt-LRP6) receptor complex to degradation. […] ZNRF3 is regulated by the R-Spondin protein that regulates the association of ZNRF3 with the related leucine-rich repeat-containing G protein-coupled receptors LGR4. […] This association results in membrane clearance of ZNFR3 and activation of the Wnt/b-catenin pathway. […] Recently, ZNRF3 was found to be the most frequently altered gene in 2 large cohorts of ACC, with a prevalence of respectively 21% and 19%.

• #31 Adrenal Cancer – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK546580/

  The transcriptome of tumors with alterations of ZNFR3 shows activation of b-catenin targets but milder than the level observed in CTNNB1-mutated ACC. […] ZNFR3 also constitutes a new tumor suppressor gene. […] By the sum of the CTNNB1- and ZNRF3-altered ACC, activation of the Wnt/b-catenin pathway could be present in 39% of ACC. […] Beckwith-Wiedemann syndrome (BWS) characteristically demonstrates genetic and epigenetic events at the 11p15.5 region involving the cyclin-dependent kinase inhibitor 1C (CDKN1C), insulin-growth-factor II (IGF-II), and H19 genes, which results in overgrowth disorders, visceromegaly (macroglossia, hemihyperplasia), malformations (wall defect, umbilical hernia), and predisposition to embryonal malignancies. […] ACC belongs to the BWS tumor spectrum, which also includes Wilms tumor, hepatoblastoma, rhabdomyosarcoma and neuroblastoma with an overall risk for tumor development in children estimated at 7.5%; most of the tumors occur in the first 8 to 10 years of life.

• #32 Adrenal Cancer – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK546580/

  Overexpression of IGF-II has been reported in approximately 60% to 90% of adrenocortical carcinoma cases, and only rarely in ACA. […] H19 is a gene for a long noncoding RNA. […] H19 is a negative regulator (or limiting) factor for body weight and cell proliferation. […] The long noncoding RNA of H19, which has been associated with several cancers and is physiologically only expressed by the maternal allele, while IGF-II only gets expressed by the paternal allele. […] The Center IC1 regulates the expression of both genes by imprinting and is methylated on the paternal allele and unmethylated on the maternal one. […] CDKN1C, a growth suppression gene that is under the control of another imprinting center IC2. […] BWS is etiologically heterogeneous, developing from dysregulation of either one or both imprinting centers and/or imprinted growth regulatory genes found on chromosome 11p15.5.

• #33 Adrenal Cancer – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK546580/

  Most BWS cases are sporadic and are the result of the loss of maternal methylation at IC2, resulting in a gain of maternal methylation at IC1 or paternal uniparental disomy. […] Mutations in CDKN1C cause the hereditary forms of BW. […] Regardless of the causative molecular defect, these alterations result in IGF-II overexpression and a decrease in H19 and/or CDKN1C expression. […] The gain of maternal methylation at IC1 or paternal uniparental disomy is associated with a higher risk of tumor development than the other forms suggesting a role of IGF-II overexpression or H19 downregulation in the tumorigenic process. […] At the somatic level, IGF-II overexpression is one of the first molecular abnormalities as described in sporadic adult ACC, with a very prevalence of about 90%. […] This increase in expression is associated with DNA demethylation at IGF-II locus and paternal isodisomy in most cases. […] Further transcriptome studies confirm that IGF-II is the most upregulated gene in ACC.

• #34 Adrenal Cancer – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK546580/

  At the somatic DNA level, mutation of TP53 is a frequent event occurring in between 16 to 70% of ACC if the whole gene is sequenced. […] TP53 is considered a tumor suppressor gene, and both alleles become inactivated in tumor tissue. […] Loss of heterozygosity (LOH) at 17p13 presents in 85% of ACC. […] However, other mechanisms that may lead to TP53 inactivation because LOH and mutation do not appear in all cases. […] The presence of abnormal nuclear staining of TP53 correlates well with TP53 mutations and could serve as a diagnostic tool. […] Somatic mutations of TP53 are associated with aggressive tumors and poor outcomes. […] Other factors that may play a role in tumorigenesis include overexpression of pituitary tumor transforming gene 1 (PTTG1), which encodes securin, a negative regulator of p53; which researchers have identified as a marker of poor survival.

• #35 Adrenal Carcinoma: Symptoms, Causes, Diagnosis, Treatment, and Prevention

  https://www.everydayhealth.com/cancer/adrenal-carcinoma/

  Adrenal carcinoma is a rare form of cancer that occurs when healthy cells in the adrenal glands mutate and grow out of control. […] While the exact cause of adrenal cancer remains unclear, several factors may contribute to its development. Adrenal carcinoma runs in families 50 percent of the time, and certain inherited conditions can increase your risk of the disease, including: Li-Fraumeni syndrome, Beckwith-Wiedemann syndrome, Carney complex, Multiple endocrine neoplasia type 1 (MEN1), Multiple endocrine neoplasia type 2 (MEN2), Familial adenomatous polyposis, Lynch syndrome, Von Hippel-Lindau disease, Von Recklinghausens disease (neurofibromatosis type 1), Paraganglioma syndrome. […] Other risk factors include: Overweight or obesity, A sedentary lifestyle, Smoking, Exposure to chemicals like asbestos and radon, Radiation therapy to your abdomen.

• #36 Adrenal Cancer: Symptoms, Prognosis & Treatment

  https://my.clevelandclinic.org/health/diseases/25236-adrenal-cancer

  Adrenal cancer occurs when cells in your adrenal glands mutate (change) and grow, forming a tumor. […] Healthcare providers dont know what causes most adrenal gland cancers. They know that mutations in the DNA can cause cells in your adrenal gland to change, grow and become cancerous. But they dont know exactly why these DNA mutations happen. […] Some people inherit a gene mutation from a biological parent, which increases their risk of adrenal cancer. Others may develop adrenal gland cancer due to exposure to certain cancer-causing substances. But in the majority of cases, these DNA mutations seem to happen for no apparent reason. […] Possible environmental risk factors for adrenal cancer include: Smoking. Exposure to certain chemicals like asbestos and radon. Prior radiation therapy to your abdomen, where your adrenal glands are located. […] While most adrenal cancers occur for no apparent reason, about 15% of people who develop adrenal tumors have a genetic disorder. This is most common in children.

• #37 Risk factors for adrenal cancer: an exploratory study – PubMed

  https://pubmed.ncbi.nlm.nih.gov/8621222/

  Adrenal cancer is a heterogeneous group of neoplasms with unknown etiology. […] Our findings suggest that cigarette smoking and use of OCs may increase the risk of adrenal cancer, but additional studies are needed with more detailed information on risk factors and histologic type of adrenal cancer.

• #38 Adrenal Carcinoma – Diagnosis & Disease Information

  https://www.cancertherapyadvisor.com/ddi/adrenal-carcinoma/

  In addition to these mutations, multiple studies have found that up to 90% of adrenal carcinoma cases have an overexpression of insulin-like growth factor-2 (IGF2). […] Certain environmental factors may modify the risk of developing adrenal carcinoma. Several studies have found a correlation between cigarette smoking and the incidence of adrenal carcinoma, particularly in males. Researchers have also identified an increased incidence of adrenal carcinoma in females who use oral contraception.

• #39 Adrenal Cancer: Symptoms, Prognosis & Treatment

  https://my.clevelandclinic.org/health/diseases/25236-adrenal-cancer

  Adrenal cancer occurs when cells in your adrenal glands mutate (change) and grow, forming a tumor. […] Healthcare providers dont know what causes most adrenal gland cancers. They know that mutations in the DNA can cause cells in your adrenal gland to change, grow and become cancerous. But they dont know exactly why these DNA mutations happen. […] Some people inherit a gene mutation from a biological parent, which increases their risk of adrenal cancer. Others may develop adrenal gland cancer due to exposure to certain cancer-causing substances. But in the majority of cases, these DNA mutations seem to happen for no apparent reason. […] Possible environmental risk factors for adrenal cancer include: Smoking. Exposure to certain chemicals like asbestos and radon. Prior radiation therapy to your abdomen, where your adrenal glands are located. […] While most adrenal cancers occur for no apparent reason, about 15% of people who develop adrenal tumors have a genetic disorder. This is most common in children.

• #40 Adrenocortical carcinoma – Symptoms, Causes, Diagnosis, Treatment PACE Hospitals – Best Hospitals in Hitech City, Hyderabad, India | Near Madhapur, Kukatpally, KPHB, Kondapur, Gachibowli, Jubilee Hills, Banjara HillsPACE Hospitals Contact

  https://www.pacehospital.com/adrenocortical-carcinoma-symptoms-causes-diagnosis-treatment

  Poor lifestyle: Certain environmental factors and lifestyles, including smoking and exposure to carcinogens can also increase the risk of cancer. […] Inherited cancer predisposition syndromes: Specific genetic conditions may increase the risk, including: […] Multiple endocrine neoplasia, type 1 (MEN 1): This is a genetic disorder that causes tumours in multiple endocrine glands, increasing the risk of adrenal cancer due to genetic mutations. […] Lynch syndrome: This condition is associated with a higher risk of various cancers due to inherited defects in DNA mismatch repair genes. […] Li-Fraumeni syndrome: It is an uncommon and inherited disorder caused by certain mutations in the TP53 gene, which predisposes individuals to various cancers, including adrenal cancer, due to defective tumor suppressor function.

• #41 Adrenal Cancer: Symptoms, Prognosis & Treatment

  https://my.clevelandclinic.org/health/diseases/25236-adrenal-cancer

  Adrenal cancer occurs when cells in your adrenal glands mutate (change) and grow, forming a tumor. […] Healthcare providers dont know what causes most adrenal gland cancers. They know that mutations in the DNA can cause cells in your adrenal gland to change, grow and become cancerous. But they dont know exactly why these DNA mutations happen. […] Some people inherit a gene mutation from a biological parent, which increases their risk of adrenal cancer. Others may develop adrenal gland cancer due to exposure to certain cancer-causing substances. But in the majority of cases, these DNA mutations seem to happen for no apparent reason. […] Possible environmental risk factors for adrenal cancer include: Smoking. Exposure to certain chemicals like asbestos and radon. Prior radiation therapy to your abdomen, where your adrenal glands are located. […] While most adrenal cancers occur for no apparent reason, about 15% of people who develop adrenal tumors have a genetic disorder. This is most common in children.

• #42 Adrenocortical Carcinoma: Causes, Symptoms And Treatment

  https://www.netmeds.com/health-library/post/adrenocortical-carcinoma-causes-symptoms-and-treatment?srsltid=AfmBOordpNQhT86BAAfheeLUxhg_6LkmNfooK0PFSRxoJSi_F0Usu2CS

  Previous exposure to high levels of radiation, particularly during childhood or as part of radiation therapy for other conditions, has been linked to an increased risk of ACC. […] In a significant number of cases, the specific cause of ACC remains unidentified. The interplay of genetic predisposition, environmental factors, and potential triggers might contribute to the development of this rare cancer.

• #43 Adrenal Carcinoma – Diagnosis & Disease Information

  https://www.cancertherapyadvisor.com/ddi/adrenal-carcinoma/

  In addition to these mutations, multiple studies have found that up to 90% of adrenal carcinoma cases have an overexpression of insulin-like growth factor-2 (IGF2). […] Certain environmental factors may modify the risk of developing adrenal carcinoma. Several studies have found a correlation between cigarette smoking and the incidence of adrenal carcinoma, particularly in males. Researchers have also identified an increased incidence of adrenal carcinoma in females who use oral contraception.

• #44 Adrenal Cancer Risk Factors | Genetics & Adrenal Cancer | American Cancer Society

  https://www.cancer.org/cancer/types/adrenal-cancer/causes-risks-prevention/risk-factors.html

  Lynch syndrome (formerly known as HNPCC) is an inherited genetic disorder that increases the risk of colorectal cancer, stomach cancer, and some other cancers, including adrenocortical cancer. […] Risk factors such as excess body weight, living a sedentary lifestyle, and being exposed to cancer-causing substances in the environment can affect a person’s risk of many types of cancer. Smoking, in particular, has been suggested as a risk factor for adrenal cancer.

• #45 Adrenal Carcinoma: Symptoms, Causes, Diagnosis, Treatment, and Prevention

  https://www.everydayhealth.com/cancer/adrenal-carcinoma/

  Adrenal carcinoma is a rare form of cancer that occurs when healthy cells in the adrenal glands mutate and grow out of control. […] While the exact cause of adrenal cancer remains unclear, several factors may contribute to its development. Adrenal carcinoma runs in families 50 percent of the time, and certain inherited conditions can increase your risk of the disease, including: Li-Fraumeni syndrome, Beckwith-Wiedemann syndrome, Carney complex, Multiple endocrine neoplasia type 1 (MEN1), Multiple endocrine neoplasia type 2 (MEN2), Familial adenomatous polyposis, Lynch syndrome, Von Hippel-Lindau disease, Von Recklinghausens disease (neurofibromatosis type 1), Paraganglioma syndrome. […] Other risk factors include: Overweight or obesity, A sedentary lifestyle, Smoking, Exposure to chemicals like asbestos and radon, Radiation therapy to your abdomen.

• #46 Adrenal Cancer – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK546580/

  Adrenocortical carcinomas can arise sporadically or be part of a familial cancer syndrome. […] Although the molecular mechanisms underlying tumorigenesis in many of the hereditary syndromes are well characterized, the molecular pathogenesis of sporadic ACC is less well understood. […] Furthermore, most cases of ACC appear to be sporadic. […] Several studies on the clonality of adrenocortical tumors have demonstrated that most benign adrenocortical lesions are polyclonal; however, some ACC that have been studied indicate monoclonal proliferation. […] Adrenocortical hyperplasia seems to be a polyclonal process; however, most adrenocortical tumors, whether benign or malignant, are monoclonal lesions, indicating that genetic changes at specific genomic loci may be playing a role in adrenal tumorigenesis.

• #47 Adrenal Cancer – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK546580/

  The chromosomal rearrangements present in benign and malignant adrenocortical tumors are similar; such as the alteration of the beta-catenin pathway. […] However, these chromosomal rearrangements are more frequent in the malignant tumors compared with the benign lesion. […] A multistep tumor progression of tumorigenesis has been proposed from genes that are known to be associated with hereditary tumor syndromes described below. […] Li-Fraumeni syndrome is a familial cancer syndrome arising from a germline mutation of the TP53 gene located at 17p13. […] Patients are susceptible to breast cancer, sarcomas, brain tumors, leukemia, and adrenocortical carcinoma. […] Variants of this disease may appear in children with sporadic ACC and germline p53 mutations without a typical history of Li-Fraumeni syndrome.

• #48 Adrenal Cancer – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK546580/

  In particular, pR337H, which involves a substitution of the amino acid arginine (R) to histidine (H), is a common mutation detected in Southern Brazil. […] The incidence of pediatric ACC is very high in Southern Brazil (1015 times the world-wide occurrence) because of the segregation of the TP53 hotspot mutation p.R337H. […] The Arginine residue at codon 337 is a critical part of an alpha-helix motif involved in the protein oligomerization. […] Functional data have shown that the replacement of arginine by histidine disrupts the tetramer formed in a pH-dependent manner, making it functionally impaired at physiologic pH. […] Beyond these mutations, the TP53 polymorphisms in adult patients seem to influence overall survival. […] The protein p53 has a fundamental role in the cellular response to stress, oncogene activation, and DNA damage by regulating the cell cycle and apoptosis.

• #49 Adrenal Tumors | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/adrenal-tumors

  Only 5-10 percent of adrenal tumors are cancerous. However, the incidence of adrenal cancer is 10-15 times higher in southern Brazil due to a genetic mutation. […] A small number of cancerous adrenal tumors are caused by genetic mutations.

• #50 Adrenal Cancer – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK546580/

  Loss of retinoblastoma (Rb) protein has been found in 27% of aggressive adrenocortical carcinoma. […] This defect is related in most cases to mutations of the RB1 gene or its allelic loss. […] Another study revealed the presence of RB1 mutations in 7% of tumors. […] Another 11% of the tumors of this cohort harbored mutations of cyclin-dependent kinase inhibitor 2A (CDKN2A), and 2% exhibited high-level amplification of cyclin-dependent kinase inhibitor 4 (CDK4). […] Thus, overall, 33% of the tumors had alterations of the Tp53 pathway. […] Patients with familial adenomatous polyposis (FAP) or Gardner syndrome, which is a mutation of the adenomatous polyposis coli gene (APC) located in 5q21 chromosomal, present with multiple colonic polyps and an increased risk of early colon carcinomas.

• #51

  https://winshipcancer.emory.edu/cancer-types-and-treatments/adrenal-cancer/prevention.php

  While researchers are still determining the exact cause of adrenal cancer, the following cancer-prevention measures may help lower your risk for the disease. […] When considering adrenal cancer prevention, its important to understand what adrenal cancer is and what causes it. […] Researchers are still trying to determine what causes an adrenal tumor to become cancerous and to establish adrenal cancer prevention guidelines. So far, theyve determined that inherited genetic mutations account for up to 15% of all adrenal cancers. This is more likely to be the case when adrenal cancer is diagnosed in children. […] Its important to note, however, that the majority (85%) of all adrenal cancers are sporadic, or not caused by an underlying genetic condition.

• #52 Adrenocortical Carcinoma: Symptoms, Stages & Prognosis

  https://my.clevelandclinic.org/health/diseases/6152-adrenocortical-carcinoma

  Adrenocortical carcinoma is a rare cancer in your adrenal cortex, the outer layer of your adrenal glands. […] Researchers dont know the exact cause. Some people develop adrenocortical carcinoma because they have inherited conditions that increase the risk that theyll develop the condition. […] In other cases, certain genetic mutations (changes) appear to increase risk. For example, research shows changes in the tumor suppressor genes TP53 and IGF2 appear to drive adrenocortical carcinoma. […] Having any of the following conditions increases your risk of developing adrenocortical carcinoma: Beckwith-Wiedemann syndrome, Carney complex, Familial adenomatous polyposis (FAP), Li-Fraumeni syndrome, Lynch syndrome, Multiple endocrine neoplasia (MEN1), Neurofibromatosis Type 1 (NF1), Von Hippel-Lindau (VHL) syndrome. […] Researchers know about half of all cases of adrenocortical carcinoma happen when certain genes mutate and create cancerous cells that multiply and become tumors. But they dont know what triggers those mutations, so they cant recommend ways you could prevent them from happening.

• #53 Adrenal Cancer Symptoms, Causes & Treatment | Baptist Health

  https://www.baptisthealth.com/care-services/conditions-treatments/adrenal-cancer

  Like other cancers, adrenal cancers result from DNA mutations that drive changes in cell behavior. Cancer cells live longer and reproduce more rapidly than healthy cells, leading to the buildup of cancerous masses or tumors that interrupt normal bodily functions. Cancer cells also metastasize or spread, extending the range and degree of disease. Medical researchers have yet to determine the exact cause or causes of the mutations. […] There is evidence that genes plays a role in some adrenal tumors. There are several heritable disorders that increase the likelihood of developing this type of cancer: Beckwith-Wiedemann syndrome, Carney syndrome, Cowden syndrome, Gardner syndrome, Li-Fraumeni syndrome, Lynch syndrome, Multiple endocrine neoplasia 1 (MEN1), Turcot syndrome.

• #54 Adrenal Cancer: Causes, Symptoms, and Treatment

  https://www.medicoverhospitals.in/diseases/adrenal-cancer/

  The exact cause of adrenal cancer remains unclear, but several factors are believed to increase the risk: […] Certain genetic syndromes, such as Li-Fraumeni syndrome and Beckwith-Wiedemann syndrome, are associated with a higher risk of developing adrenal cancer. Genetic mutations in the TP53 gene, among others, have also been linked to the disease. […] Exposure to certain environmental factors, including prolonged exposure to industrial chemicals and radiation, may increase the risk of adrenal cancer. However, more research is needed to establish a definitive link. […] The exact cause is unknown, but it may be related to genetic mutations or inherited conditions.

Zobacz więcej