Adrenal Tumor
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An adrenal tumor or adrenal mass is any
benign Malignancy () is the tendency of a medical condition to become progressively worse; the term is most familiar as a characterization of cancer. A ''malignant'' tumor contrasts with a non-cancerous benign tumor, ''benign'' tumor in that a malig ...
or
malignant Malignancy () is the tendency of a medical condition to become progressively worse; the term is most familiar as a characterization of cancer. A ''malignant'' tumor contrasts with a non-cancerous benign tumor, ''benign'' tumor in that a malig ...
neoplasms A neoplasm () is a type of abnormal and excessive growth of tissue (biology), tissue. The process that occurs to form or produce a neoplasm is called neoplasia. The growth of a neoplasm is uncoordinated with that of the normal surrounding tiss ...
of the
adrenal gland The adrenal glands (also known as suprarenal glands) are endocrine glands that produce a variety of hormones including adrenaline and the steroids aldosterone and cortisol. They are found above the kidneys. Each gland has an outer adrenal corte ...
, several of which are notable for their ability to overproduce
endocrine The endocrine system is a messenger system in an organism comprising feedback loops of hormones that are released by internal glands directly into the circulatory system and that target and regulate distant organs. In vertebrates, the hypotha ...
hormone A hormone (from the Ancient Greek, Greek participle , "setting in motion") is a class of cell signaling, signaling molecules in multicellular organisms that are sent to distant organs or tissues by complex biological processes to regulate physio ...
s. Adrenal cancer is the presence of malignant adrenal tumors, which include neuroblastoma, adrenocortical carcinoma and some adrenal pheochromocytomas. Most adrenal pheochromocytomas and all adrenocortical adenomas are benign tumors, which do not
metastasize Metastasis is a pathogenic agent's spreading from an initial or primary site to a different or secondary site within the host's body; the term is typically used when referring to metastasis by a cancerous tumor. The newly pathological sites, ...
or invade nearby tissues, but may cause significant symptoms by dysregulating hormones.


Adrenal Glands

Adrenal glands, also known as the suprarenal glands, are located above each kidney and in the retroperitoneal space. They produce and regulate different hormones, including stress hormones like
cortisol Cortisol is a steroid hormone in the glucocorticoid class of hormones and a stress hormone. When used as medication, it is known as hydrocortisone. Cortisol is produced in many animals, mainly by the ''zona fasciculata'' of the adrenal corte ...
and hormones that regulate salt and water balance like
aldosterone Aldosterone is the main mineralocorticoid steroid hormone produced by the zona glomerulosa of the adrenal cortex in the adrenal gland. It is essential for sodium conservation in the kidney, salivary glands, sweat glands, and colon. It plays ...
. The glands are triangular-shaped and are composed of two types of tissues: the cortex and the medulla. The glands are surrounded by a capsule made up of connective tissue and consist of outer portion or the cortex, and the inner portion or the medulla. The outer cortex is further divided into three layers, named the
zona glomerulosa The ''zona glomerulosa'' (sometimes, glomerular zone) of the adrenal gland is the most superficial layer of the adrenal cortex, lying directly beneath the renal capsule. Its cells are ovoid and arranged in clusters or arches (''glomus'' is Latin ...
, zona fasciculata and zona reticularis, all which produce critical
steroid hormone A steroid hormone is a steroid that acts as a hormone. Steroid hormones can be grouped into two classes: corticosteroids (typically made in the adrenal cortex, hence ''cortico-'') and sex steroids (typically made in the gonads or placenta). Wit ...
s. The inner portion or the medulla produces catecholamines such as adrenaline (epinephrine) and
norepinephrine Norepinephrine (NE), also called noradrenaline (NA) or noradrenalin, is an organic compound, organic chemical in the catecholamine family that functions in the brain and human body, body as a hormone, neurotransmitter and neuromodulator. The ...
. Mineralocorticoid hormones like
aldosterone Aldosterone is the main mineralocorticoid steroid hormone produced by the zona glomerulosa of the adrenal cortex in the adrenal gland. It is essential for sodium conservation in the kidney, salivary glands, sweat glands, and colon. It plays ...
, which regulates
blood pressure Blood pressure (BP) is the pressure of Circulatory system, circulating blood against the walls of blood vessels. Most of this pressure results from the heart pumping blood through the circulatory system. When used without qualification, the term ...
,
kidney In humans, the kidneys are two reddish-brown bean-shaped blood-filtering organ (anatomy), organs that are a multilobar, multipapillary form of mammalian kidneys, usually without signs of external lobulation. They are located on the left and rig ...
function, and certain
sex hormone Sex hormones, also known as sex steroids, gonadocorticoids and gonadal steroids, are steroid hormones that interact with vertebrate steroid hormone receptors. The sex hormones include the androgens, estrogens, and progestogens. Their effects a ...
s, are produced by the outer layer of the adrenal cortex or the zona glomerulosa.
Glucocorticoid Glucocorticoids (or, less commonly, glucocorticosteroids) are a class of corticosteroids, which are a class of steroid hormones. Glucocorticoids are corticosteroids that bind to the glucocorticoid receptor that is present in almost every vertebra ...
s, which are produced by the middle layer of the adrenal cortex, or the zona fasciculata. Glucocorticoids are critical for regulation of
blood sugar The blood sugar level, blood sugar concentration, blood glucose level, or glycemia is the measure of glucose concentrated in the blood. The body tightly regulates blood glucose levels as a part of metabolic homeostasis. For a 70 kg (1 ...
and the
immune system The immune system is a network of biological systems that protects an organism from diseases. It detects and responds to a wide variety of pathogens, from viruses to bacteria, as well as Tumor immunology, cancer cells, Parasitic worm, parasitic ...
, as well as response to physiological stress.
Androgens An androgen (from Greek ''andr-'', the stem of the word meaning ) is any natural or synthetic steroid hormone that regulates the development and maintenance of male characteristics in vertebrates by binding to androgen receptors. This includes ...
, which aid in the development of secondary sex characteristics, are produced by the inner zona reticularis. DHEA (dehydroepiandrosterone) is the most commonly found hormone in the body and is a precursor in the production of progesterone, estrogen, cortisol and testosterone.


Tumors of the adrenal cortex

Adrenal tumors can be classified using the TMN Staging System to determine the stage and grade of masses. Along with stage and grade, pathologic testing of the tumor margins can help determine
prognosis Prognosis ( Greek: πρόγνωσις "fore-knowing, foreseeing"; : prognoses) is a medical term for predicting the likelihood or expected development of a disease, including whether the signs and symptoms will improve or worsen (and how quickly) ...
. Benign tumors in the cortex are known as adenomas while malignant tumors are known as carcinomas. In 2022, the
World Health Organization (WHO) The World Health Organization (WHO) is a specialized agency of the United Nations which coordinates responses to international public health issues and emergencies. It is headquartered in Geneva, Switzerland, and has 6 regional offices and 15 ...
updated a report on the classification of adrenal masses and determined genetic differences in mutations leading to each type of tumor. Mutations in the genes that produce ion channels have been implicated in the development of adenomas. Adenomas may also be a result of increased hypermethylation of DNA. Carcinomas were determined to be caused by mutations in Wnt and p53 pathways. Both adenoma and carcinoma tumors of the adrenal cortex may produce steroid hormones, with important clinical consequences.


Adrenocortical adenoma

''Adrenocortical adenomas'' are benign tumors of the adrenal cortex that are extremely common (present in 1-10% of persons at autopsy). They can be confused with adrenocortical "nodules", however nodules are not true neoplasms. Adrenocortical adenomas are uncommon in patients younger than 30 years old, and have equal incidence in both sexes. The clinical significance of these neoplasms is two-fold. First, they have been detected as incidental findings with increasing frequency in recent years, due to the increasing use of
CT scan A computed tomography scan (CT scan), formerly called computed axial tomography scan (CAT scan), is a medical imaging technique used to obtain detailed internal images of the body. The personnel that perform CT scans are called radiographers or ...
s and
magnetic resonance imaging Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to generate pictures of the anatomy and the physiological processes inside the body. MRI scanners use strong magnetic fields, magnetic field gradients, and ...
in a variety of medical settings. This can result in additional testing and invasive procedures to rule out the slight possibility of an early adrenocortical carcinoma. Second, a minority (about 15%) of adrenocortical adenomas are "functional", meaning that they produce
glucocorticoid Glucocorticoids (or, less commonly, glucocorticosteroids) are a class of corticosteroids, which are a class of steroid hormones. Glucocorticoids are corticosteroids that bind to the glucocorticoid receptor that is present in almost every vertebra ...
s,
mineralocorticoid Mineralocorticoids are a class of corticosteroids, which in turn are a class of steroid hormones. Mineralocorticoids are produced in the adrenal cortex and influence salt and water balances (electrolyte balance and fluid balance). The primary ...
s, and/or
sex steroids Sex hormones, also known as sex steroids, gonadocorticoids and gonadal steroids, are steroid hormones that interact with vertebrate steroid hormone receptors. The sex hormones include the androgens, estrogens, and progestogens. Their effects a ...
, resulting in endocrine disorders such as Cushing's syndrome, Conn's syndrome (hyperaldosteronism),
virilization Virilization or masculinization is the biological development of adult male characteristics in young males or females. Most of the changes of virilization are produced by androgens. Virilization is a medical term commonly used in three medical a ...
of females, or feminization of males. Functional adrenocortical adenomas are surgically curable. Most of the adrenocortical adenomas are less than 2 cm in greatest dimension and less than 50 grams in weight. However, size and weight of the adrenal cortical tumors are no longer considered to be a reliable sign of benignity or malignancy. Grossly, adrenocortical adenomas are encapsulated, well-circumscribed, solitary tumors with solid, homogeneous yellow-cut surface. Necrosis and hemorrhage are rare findings.


Adrenocortical carcinoma

Adrenocortical carcinoma (ACC) is a rare, highly aggressive
cancer Cancer is a group of diseases involving Cell growth#Disorders, abnormal cell growth with the potential to Invasion (cancer), invade or Metastasis, spread to other parts of the body. These contrast with benign tumors, which do not spread. Po ...
of adrenal cortical cells, which may occur in children or adults. ACCs may be "functional", producing
steroid hormone A steroid hormone is a steroid that acts as a hormone. Steroid hormones can be grouped into two classes: corticosteroids (typically made in the adrenal cortex, hence ''cortico-'') and sex steroids (typically made in the gonads or placenta). Wit ...
s and consequent
endocrine The endocrine system is a messenger system in an organism comprising feedback loops of hormones that are released by internal glands directly into the circulatory system and that target and regulate distant organs. In vertebrates, the hypotha ...
dysfunction similar to that seen in many adrenocortical adenomas. Due to their location deep in the retroperitoneum, most adrenocortical carcinomas are not diagnosed until they have grown quite large. Adrenocortical carcinomas frequently invade large vessels, such as the renal vein and
inferior vena cava The inferior vena cava is a large vein that carries the deoxygenated blood from the lower and middle body into the right atrium of the heart. It is formed by the joining of the right and the left common iliac veins, usually at the level of the ...
, as well as
metastasize Metastasis is a pathogenic agent's spreading from an initial or primary site to a different or secondary site within the host's body; the term is typically used when referring to metastasis by a cancerous tumor. The newly pathological sites, ...
via the lymphatics and through the
blood Blood is a body fluid in the circulatory system of humans and other vertebrates that delivers necessary substances such as nutrients and oxygen to the cells, and transports metabolic waste products away from those same cells. Blood is com ...
to the
lung The lungs are the primary Organ (biology), organs of the respiratory system in many animals, including humans. In mammals and most other tetrapods, two lungs are located near the Vertebral column, backbone on either side of the heart. Their ...
s and other organs. The most effective treatment is
surgery Surgery is a medical specialty that uses manual and instrumental techniques to diagnose or treat pathological conditions (e.g., trauma, disease, injury, malignancy), to alter bodily functions (e.g., malabsorption created by bariatric surgery s ...
, although this is not feasible for many patients, and the overall
prognosis Prognosis ( Greek: πρόγνωσις "fore-knowing, foreseeing"; : prognoses) is a medical term for predicting the likelihood or expected development of a disease, including whether the signs and symptoms will improve or worsen (and how quickly) ...
of the disease is poor.
Chemotherapy Chemotherapy (often abbreviated chemo, sometimes CTX and CTx) is the type of cancer treatment that uses one or more anti-cancer drugs (list of chemotherapeutic agents, chemotherapeutic agents or alkylating agents) in a standard chemotherapy re ...
,
radiation therapy Radiation therapy or radiotherapy (RT, RTx, or XRT) is a therapy, treatment using ionizing radiation, generally provided as part of treatment of cancer, cancer therapy to either kill or control the growth of malignancy, malignant cell (biology), ...
, and hormonal therapy may also be employed in the treatment of this disease.


Tumors of the adrenal medulla

The
adrenal medulla The adrenal medulla () is the inner part of the adrenal gland. It is located at the center of the gland, being surrounded by the adrenal cortex. It is the innermost part of the adrenal gland, consisting of chromaffin cells that secrete catecho ...
is located anatomically at the center of each adrenal gland, and is composed of neuroendocrine (chromaffin) cells which produce and release
epinephrine Adrenaline, also known as epinephrine, is a hormone and medication which is involved in regulating visceral functions (e.g., respiration). It appears as a white microcrystalline granule. Adrenaline is normally produced by the adrenal glands a ...
(adrenaline) into the bloodstream in response to activation of the
sympathetic nervous system The sympathetic nervous system (SNS or SANS, sympathetic autonomic nervous system, to differentiate it from the somatic nervous system) is one of the three divisions of the autonomic nervous system, the others being the parasympathetic nervous sy ...
. Neuroblastoma and pheochromocytoma are the two most important tumors which arise from the adrenal medulla. Both tumors may also arise from extra-adrenal sites, specifically, in the paraganglia of the
sympathetic chain The sympathetic trunk (sympathetic chain, gangliated cord) is a paired bundle of nerve fibers that run from the base of the skull to the coccyx. It is a major component of the sympathetic nervous system. Structure The sympathetic trunk lies just ...
.


Neuroblastoma

Neuroblastoma is an aggressive
cancer Cancer is a group of diseases involving Cell growth#Disorders, abnormal cell growth with the potential to Invasion (cancer), invade or Metastasis, spread to other parts of the body. These contrast with benign tumors, which do not spread. Po ...
of immature neuroblastic cells (precursors of
neuron A neuron (American English), neurone (British English), or nerve cell, is an membrane potential#Cell excitability, excitable cell (biology), cell that fires electric signals called action potentials across a neural network (biology), neural net ...
s), and is one of the most common
pediatric Pediatrics (American English) also spelled paediatrics (British English), is the branch of medicine that involves the medical care of infants, children, adolescents, and young adults. In the United Kingdom, pediatrics covers many of their youth ...
cancers, with a median age at diagnosis of two years. Adrenal neuroblastoma typically presents with a rapidly enlarging abdominal mass. Although the tumor has often spread to distant parts of the body at the time of diagnosis, this cancer is unusual in that many cases are highly curable when the spread is limited to the
liver The liver is a major metabolic organ (anatomy), organ exclusively found in vertebrates, which performs many essential biological Function (biology), functions such as detoxification of the organism, and the Protein biosynthesis, synthesis of var ...
,
skin Skin is the layer of usually soft, flexible outer tissue covering the body of a vertebrate animal, with three main functions: protection, regulation, and sensation. Other animal coverings, such as the arthropod exoskeleton, have different ...
, and/or
bone marrow Bone marrow is a semi-solid biological tissue, tissue found within the Spongy bone, spongy (also known as cancellous) portions of bones. In birds and mammals, bone marrow is the primary site of new blood cell production (or haematopoiesis). It i ...
(stage IVS). Related, but less aggressive tumors composed of more mature neural cells include ''ganglioneuroblastoma'' and ''ganglioneuroma''. Neuroblastic tumors often produce elevated levels of
catecholamine A catecholamine (; abbreviated CA), most typically a 3,4-dihydroxyphenethylamine, is a monoamine neurotransmitter, an organic compound that has a catechol (benzene with two hydroxyl side groups next to each other) and a side-chain amine. Cate ...
hormone metabolites, such as vanillylmandelic acid (VMA) and homovanillic acid, and may produce severe watery
diarrhea Diarrhea (American English), also spelled diarrhoea or diarrhœa (British English), is the condition of having at least three loose, liquid, or watery bowel movements in a day. It often lasts for a few days and can result in dehydration d ...
through production of
vasoactive intestinal peptide Vasoactive intestinal peptide, also known as vasoactive intestinal polypeptide or VIP, is a peptide hormone that is vasoactive in the intestine. VIP is a peptide of 28 amino acid residue (chemistry), residues that belongs to a Secretin family, glu ...
. Treatment of neuroblastoma includes
surgery Surgery is a medical specialty that uses manual and instrumental techniques to diagnose or treat pathological conditions (e.g., trauma, disease, injury, malignancy), to alter bodily functions (e.g., malabsorption created by bariatric surgery s ...
and
radiation therapy Radiation therapy or radiotherapy (RT, RTx, or XRT) is a therapy, treatment using ionizing radiation, generally provided as part of treatment of cancer, cancer therapy to either kill or control the growth of malignancy, malignant cell (biology), ...
for localized disease, and
chemotherapy Chemotherapy (often abbreviated chemo, sometimes CTX and CTx) is the type of cancer treatment that uses one or more anti-cancer drugs (list of chemotherapeutic agents, chemotherapeutic agents or alkylating agents) in a standard chemotherapy re ...
for
metastatic Metastasis is a pathogenic agent's spreading from an initial or primary site to a different or secondary site within the host's body; the term is typically used when referring to metastasis by a cancerous tumor. The newly pathological sites, ...
disease.


Pheochromocytoma

Pheochromocytoma is a
neoplasm A neoplasm () is a type of abnormal and excessive growth of tissue. The process that occurs to form or produce a neoplasm is called neoplasia. The growth of a neoplasm is uncoordinated with that of the normal surrounding tissue, and persists ...
composed of cells similar to the
chromaffin cell Chromaffin cells, also called pheochromocytes (or phaeochromocytes), are neuroendocrine cells found mostly in the adrenal medulla, medulla of the adrenal glands in mammals. These cells serve a variety of functions such as serving as a response to ...
s of the mature adrenal medulla. Pheochromocytomas occur in patients of all ages, and may be sporadic, or associated with a hereditary cancer syndrome, such as multiple endocrine neoplasia (MEN) types IIA and IIB,
neurofibromatosis Neurofibromatosis (NF) refers to a group of three distinct genetic conditions in which tumors grow in the nervous system. The tumors are non-cancerous (benign) and often involve the skin or surrounding bone. Although symptoms are often mild, e ...
type I, or von Hippel–Lindau syndrome. Only 10% of adrenal pheochromocytomas are
malignant Malignancy () is the tendency of a medical condition to become progressively worse; the term is most familiar as a characterization of cancer. A ''malignant'' tumor contrasts with a non-cancerous benign tumor, ''benign'' tumor in that a malig ...
, while the rest are
benign tumor A benign tumor is a mass of Cell (biology), cells (tumor) that does not Cancer invasion, invade neighboring tissue or Metastasis, metastasize (spread throughout the body). Compared to Cancer, malignant (cancerous) tumors, benign tumors generally ...
s. The most clinically important feature of pheochromocytomas is their tendency to produce large amounts of the
catecholamine A catecholamine (; abbreviated CA), most typically a 3,4-dihydroxyphenethylamine, is a monoamine neurotransmitter, an organic compound that has a catechol (benzene with two hydroxyl side groups next to each other) and a side-chain amine. Cate ...
hormones
epinephrine Adrenaline, also known as epinephrine, is a hormone and medication which is involved in regulating visceral functions (e.g., respiration). It appears as a white microcrystalline granule. Adrenaline is normally produced by the adrenal glands a ...
(adrenaline) and
norepinephrine Norepinephrine (NE), also called noradrenaline (NA) or noradrenalin, is an organic compound, organic chemical in the catecholamine family that functions in the brain and human body, body as a hormone, neurotransmitter and neuromodulator. The ...
. This may lead to potentially life-threatening
high blood pressure Hypertension, also known as high blood pressure, is a long-term medical condition in which the blood pressure in the arteries is persistently elevated. High blood pressure usually does not cause symptoms itself. It is, however, a major ri ...
, or cardiac
arrhythmia Arrhythmias, also known as cardiac arrhythmias, are irregularities in the cardiac cycle, heartbeat, including when it is too fast or too slow. Essentially, this is anything but normal sinus rhythm. A resting heart rate that is too fast – ab ...
s, and numerous symptoms such as
headache A headache, also known as cephalalgia, is the symptom of pain in the face, head, or neck. It can occur as a migraine, tension-type headache, or cluster headache. There is an increased risk of Depression (mood), depression in those with severe ...
, palpitations, anxiety attacks,
sweat Perspiration, also known as sweat, is the fluid secreted by sweat glands in the skin of mammals. Two types of sweat glands can be found in humans: eccrine glands and Apocrine sweat gland, apocrine glands. The eccrine sweat glands are distribu ...
ing,
weight loss Weight loss, in the context of medicine, health, or physical fitness, refers to a reduction of the total body mass, by a mean loss of fluid, body fat (adipose tissue), or lean mass (namely bone mineral deposits, muscle, tendon, and other conn ...
, and
tremor A tremor is an involuntary, somewhat rhythmic muscle contraction and relaxation involving neural oscillations, oscillations or twitching movements of one or more body parts. It is the most common of all involuntary movements and can affect the h ...
. Diagnosis is most easily confirmed through urinary measurement of catecholamine
metabolite In biochemistry, a metabolite is an intermediate or end product of metabolism. The term is usually used for small molecules. Metabolites have various functions, including fuel, structure, signaling, stimulatory and inhibitory effects on enzymes, c ...
s such as VMA and metanephrines. Most pheochromocytomas are initially treated with anti-adrenergic drugs to protect against catecholamine overload, with surgery employed to remove the tumor once the patient is medically stable.


Incidentalomas

An adrenal ''
incidentaloma In medical or research imaging, an incidental imaging finding (also called an incidentaloma) is an unanticipated finding which is not related to the original diagnostic inquiry. As with other types of incidental medical findings, they may represe ...
'' is an adrenal tumor found by coincidence without clinical symptoms or suspicion. It is one of the more common unexpected findings revealed by computed tomography (CT), magnetic resonance imaging (MRI), or
ultrasonography Medical ultrasound includes diagnostic techniques (mainly imaging) using ultrasound, as well as therapeutic applications of ultrasound. In diagnosis, it is used to create an image of internal body structures such as tendons, muscles, joints, ...
. Adrenal incidentalomas often secrete cortisol and require thorough clinical evaluation. Management of incidentalomas include evaluation by a endocrinologist or endocrine surgeon. Tumors under 3 cm are generally considered benign and are only treated if there are grounds for a diagnosis of Cushing's syndrome or pheochromocytoma. Radiodensity helps with estimating the risk of malignancy in a tumor. A tumor of 10 Hounsfield units or less on an un-enhanced CT is likely a lipid-rich adenoma. On CT scan, benign
adenoma An adenoma is a benign tumor of epithelium, epithelial tissue with glandular origin, glandular characteristics, or both. Adenomas can grow from many glandular organ (anatomy), organs, including the adrenal glands, pituitary gland, thyroid, prosta ...
s typically are of low radiographic density (due to fat content) and show rapid washout of contrast medium (50% or more of the contrast medium washes out at 10 minutes). If the hormonal evaluation is negative and imaging suggests benign, follow-up should be considered with imaging at 6, 12, and 24 months and repeat hormonal evaluation yearly for 4 years. Adrenal-dedicated CT and MRI imaging can be performed to distinguish benign adenomas from potentially malignant lesions. Online calculators assist radiologists in calculating the washout of contrast in adrenal nodules on CT and chemical shift on MRI. Hormonal evaluations can also be used, which include: * 1-mg overnight dexamethasone suppression test to detect excess cortisol * 24-hour urinary specimen for measurement of fractionated metanephrines and catecholamines *
Blood plasma Blood plasma is a light Amber (color), amber-colored liquid component of blood in which blood cells are absent, but which contains Blood protein, proteins and other constituents of whole blood in Suspension (chemistry), suspension. It makes up ...
aldosterone Aldosterone is the main mineralocorticoid steroid hormone produced by the zona glomerulosa of the adrenal cortex in the adrenal gland. It is essential for sodium conservation in the kidney, salivary glands, sweat glands, and colon. It plays ...
concentration and plasma renin activity, ''if hypertension is present''


Metastasis to the Adrenals

Metastasis Metastasis is a pathogenic agent's spreading from an initial or primary site to a different or secondary site within the host's body; the term is typically used when referring to metastasis by a cancerous tumor. The newly pathological sites, ...
, or mets, to one or both adrenal glands is the most common form of malignant adrenal lesion, and the second most common adrenal tumor after benign adenomas. Primary tumors in are most commonly from
lung cancer Lung cancer, also known as lung carcinoma, is a malignant tumor that begins in the lung. Lung cancer is caused by genetic damage to the DNA of cells in the airways, often caused by cigarette smoking or inhaling damaging chemicals. Damaged ...
(39%),
breast cancer Breast cancer is a cancer that develops from breast tissue. Signs of breast cancer may include a Breast lump, lump in the breast, a change in breast shape, dimpling of the skin, Milk-rejection sign, milk rejection, fluid coming from the nipp ...
(35%), malignant melanoma, gastrointestinal tract cancer, pancreas cancer, and renal cancer. Clinical concern for mets to adrenal glands requires evaluation for adrenal insufficiency as well as evaluation for tumor markers that can determine involvement of other areas of metastatic disease.


Epidemiology

Adrenal tumors are common tumors in humans. The detection of adrenal incidentalomas has increased, likely due to improvement in imaging modalities. A population based study by Ebbehoj et. al. determined a 10x increase in adrenal tumors from 1995 to 2010, aligning with an increase in abdominal scans. Incidentalomas were also determined to be increasingly functional due to better evaluation methods for hormonal imbalances that the tumors may cause. In review of literature by Zeiger et al., it was also determined that about 80% of adrenal tumors found incidentally were non-functional adenomas, while the remaining had functional, malignant or metastatic tumors.


Treatment


Surgical

Diagnosis of adrenal tumors occur through lab and imaging studies. For adrenal carcinomas, the most effective treatment is
surgery Surgery is a medical specialty that uses manual and instrumental techniques to diagnose or treat pathological conditions (e.g., trauma, disease, injury, malignancy), to alter bodily functions (e.g., malabsorption created by bariatric surgery s ...
, however, surgery may not be feasible for many patients and the overall
prognosis Prognosis ( Greek: πρόγνωσις "fore-knowing, foreseeing"; : prognoses) is a medical term for predicting the likelihood or expected development of a disease, including whether the signs and symptoms will improve or worsen (and how quickly) ...
of the disease is poor.
Chemotherapy Chemotherapy (often abbreviated chemo, sometimes CTX and CTx) is the type of cancer treatment that uses one or more anti-cancer drugs (list of chemotherapeutic agents, chemotherapeutic agents or alkylating agents) in a standard chemotherapy re ...
,
radiation therapy Radiation therapy or radiotherapy (RT, RTx, or XRT) is a therapy, treatment using ionizing radiation, generally provided as part of treatment of cancer, cancer therapy to either kill or control the growth of malignancy, malignant cell (biology), ...
, and hormonal therapy may also be employed in the treatment of this disease. Because pheochromocytomas can cause imbalances in catecholamines that can be life-threatening, treatment and optimization before surgery is required. Close monitoring during the surgery and careful consideration for the anesthesia used during the surgery for a pheochromocytoma is also required to ensure that catecholamine release is avoided. Patients can also experience a drop in blood pressure or
hypotension Hypotension, also known as low blood pressure, is a cardiovascular condition characterized by abnormally reduced blood pressure. Blood pressure is the force of blood pushing against the walls of the arteries as the heart pumps out blood and is ...
after the tumor is removed, which is treated with adrenergic agonists and fluids. In pregnant patients, functional adrenal tumors can lead to complications in pregnancy, for example, if the tumor leads to symptoms of hypertension. Surgical management (adrenalectomy) has been determined to be safe for pregnant patients and fetuses. A 2018 Cochrane Systematic review compared two different types of surgery: laparoscopic retroperitoneal adrenalectomy and laparoscopic transperitoneal adrenalectomy in different types of adrenal tumors. Laparoscopic retroperitoneal adrenalectomy appeared to reduce late morbidity, time to oral fluid or food intake and time to ambulation, when compared to laparoscopic transperitoneal adrenalectomy. However there was uncertainty in these findings due to low-quality evidence, as well as inconclusive findings about effects of either surgery on all-cause mortality, early morbidity, socioeconomic effects, duration of surgery, operative blood loss, and conversion to open surgery. Lymphadenectomy or removal of surrounding lymph nodes is also recommended.


Non-Surgical Management

Non-Surgical Management of Adrenocortical Cancer: Although surgery is recommended for the best chance of survival without reoccurrence, Mitotane has also been used in the non-surgical management of adrenocortical carcinomas as adjuvant therapy. Mitotane is given orally via tablets and levels are monitored to ensure proper levels. Retrospective studies have analyzed the efficacy of mitotane and have determined that it should be used as adjuvant therapy in cases of patients with increased risk of reoccurrence. Mitotane can induce hormonal dysregulation including
adrenal insufficiency Adrenal insufficiency is a condition in which the adrenal glands do not produce adequate amounts of steroid hormones. The adrenal glands—also referred to as the adrenal cortex—normally secrete glucocorticoids (primarily cortisol), mineraloco ...
,
hypothyroidism Hypothyroidism is an endocrine disease in which the thyroid gland does not produce enough thyroid hormones. It can cause a number of symptoms, such as cold intolerance, poor ability to tolerate cold, fatigue, extreme fatigue, muscle aches, co ...
, and
hypogonadism Hypogonadism means diminished functional activity of the human gonad, gonads—the testicles or the ovary, ovaries—that may result in diminished biosynthesis, production of sex hormones. Low androgen (e.g., testosterone) levels are referred t ...
. Non-hormonal side effects of this medication include fatigue, GI symptoms, dyslipidemia, skin rash and mild leukopenia.


Future diagnostic tools

Blood circulating microRNAs (miRNA) has been investigated in the recent years for the potential as a less-invasive biomarker for adrenal diseases. Nine studies have so far investigated the occurrence of circulating miRNAs in blood from patients diagnosed with adrenocortical tumors. MiRNAs can be released into the blood stream by three different types of excretion. Cellular damage with passive release (necrosis, inflammation), active secretion in the form of extracellular vesicles (EV) (microvesicles, exosomes and apoptotic bodies) or in association with high density lipoproteins (HDL) and Argonaute (AGO) proteins. The majority of miRNAs found in blood are in complexes with AGO. Despite the promising results, the sensitivity of potential circulating miRNA markers for adrenocortical tumors appears to be variable. An increase in sensitivity could possibly be achieved by targeting only EV-associated miRNAs as the release of miRNAs into EVs are hypothesized to be a controlled process. EV-associated miRNAs could thereby act as more specific markers of malignancy. However, as there were significant differences in the results of the studies performed, methodological differences and low patient numbers could contribute to this discrepancy. This calls for further studies on larger cohorts with uniform methodological requirements to clarify the applicability of circulating miRNAs as biomarkers of prognosis and malignancy in patients diagnosed with adrenocortical tumors.


References


Further reading

* * *


External links

{{DEFAULTSORT:Adrenal Tumor Endocrine neoplasia Adrenal gland disorders