Pheochromocytoma. A pheochromocytoma (from Greek phaios "dark", chroma "color", kytos "cell", -oma "tumor") or phaeochromocytoma (PCC) is a neuroendocrine tumor of the medulla of the adrenal glands (originating in the chromaffin cells), or extra-adrenal chromaffin tissue that failed to involute after birth and secretes high amounts of catecholamines, mostly norepinephrine, plus epinephrine to a lesser extent. Extra-adrenal paragangliomas (often described as extra-adrenal pheochromocytomas) are closely related, though less common, tumors that originate in the ganglia of the sympathetic nervous system and are named based upon the primary anatomical site of origin.
Signs and symptoms The signs and symptoms of a pheochromocytoma are those of sympathetic nervous system hyperactivity, including: A pheochromocytoma can also cause resistant arterial hypertension. A pheochromocytoma can be fatal if it causes malignant hypertension, or severely high blood pressure. Adrenal tumor. The term adrenal tumor or adrenal mass can refer to one of several benign and malignant neoplasms of the adrenal gland, several of which are notable for their tendency to overproduce endocrine hormones.
Adrenal cancer specifically refers to malignant adrenal tumors, which include neuroblastoma, adrenocortical carcinoma, and a minority of adrenal pheochromocytomas. Most adrenal pheochromocytomas and all adrenocortical adenomas are benign tumors, which do not metastasize or invade nearby tissues, but which may still cause significant health problems by giving rise to hormonal imbalances.
Hyperaldosteronism. Hyperaldosteronism, also aldosteronism, is a medical condition wherein too much aldosterone is produced by the adrenal glands, which can lead to lowered levels of potassium in the blood (hypokalemia).
Types In endocrinology, the terms 'primary' and 'secondary' are used to describe the abnormality (e.g., elevated aldosterone) in relation to the defect, i.e., the tumor's location. Primary Primary aldosteronism (hyporeninemic hyperaldosteronism) was previously thought to be most commonly caused by an adrenal adenoma, termed Conn's syndrome. However, recent studies have shown that bilateral idiopathic adrenal hyperplasia is the cause in up to 70% of cases. Features Investigations High serum aldosteroneLow serum reninHigh-resolution CT abdomen Management Adrenal adenoma: surgeryBilateral adrenocortical hyperplasia: aldosterone antagonist, e.g., spironolactone.
Pheochromocytoma. Addison's disease. The condition arises from problems with the adrenal gland, primary adrenal insufficiency, and can be caused by damage by the body's own immune system, certain infections, or various rarer causes.
Addison's disease is also known as chronic primary adrenocortical insufficiency, to distinguish it from acute primary adrenocortical insufficiency, most often caused by Waterhouse–Friderichsen syndrome. Addison's disease should also be distinguished from secondary and tertiary adrenal insufficiency, which are caused by deficiency of ACTH (produced by the pituitary gland) and CRH (produced by the hypothalamus), respectively.
Despite this distinction, Addisonian crises can happen in all forms of adrenal insufficiency. Addison’s disease is named after Thomas Addison, a graduate of the University of Edinburgh Medical School who first described the condition in 1849. The adjective "Addisonian" is used to describe features of the condition, as well as patients suffering from Addison’s disease. Congenital adrenal hyperplasia. Congenital adrenal hyperplasia (CAH) are any of several autosomal recessive diseases resulting from mutations of genes for enzymes mediating the biochemical steps of production of cortisol from cholesterol by the adrenal glands (steroidogenesis). Most of these conditions involve excessive or deficient production of sex steroids and can alter development of primary or secondary sex characteristics in some affected infants, children, or adults. Signs and symptoms The symptoms of CAH vary depending upon the form of CAH and the sex of the patient.
Symptoms can include: Due to inadequate mineralocorticoids: vomiting due to salt-wasting leading to dehydration and death Due to excess androgens: Genetics The gene for 21-hydroxylase is found on 6p21.3 as part of the HLA complex. 21-hydroxylase deficiency results in a unique mutation with two highly homologous near-copies in series consisting of an active gene (CYP21A) and an inactive pseudogene (CYP21P). Waterhouse–Friderichsen syndrome. Waterhouse–Friderichsen syndrome (WFS) or hemorrhagic adrenalitis or Fulminant meningococcemia, is defined as adrenal gland failure due to bleeding into the adrenal glands, caused by severe bacterial (or rarely viral) infection (most commonly the meningococcus Neisseria meningitidis). Signs and symptoms WFS is the most severe form of meningococcal septicemia.
The onset of the illness is nonspecific with fever, rigors, vomiting, and headache. Soon a rash appears; first macular, not much different from the rose spots of typhoid, and rapidly becoming petechial and purpuric with a dusky gray color. Low blood pressure (hypotension) is the rule and rapidly leads to septic shock. Causes Multiple species of bacteria can be associated with the condition: Meningococcus is another term for the bacterial species Neisseria meningitidis; blood infection with said species usually underlies WFS.
Absent adrenal gland. Conn's syndrome. Conn syndrome is an aldosterone-producing adenoma.
Conn's syndrome is named after Jerome W. Conn (1907–1994), the American endocrinologist who first described the condition at the University of Michigan in 1955. Cushing's syndrome. Not to be confused with Cushing's triad, a disease state resulting from increased intracranial pressure.
Cushing's syndrome describes the signs and symptoms associated with prolonged exposure to inappropriately high levels of the hormone cortisol. This can be caused by taking glucocorticoid drugs, or diseases that result in excess cortisol, adrenocorticotropic hormone (ACTH), or CRH levels. Adrenal insufficiency.