A report on Pheochromocytoma

Normal remnant adrenal gland (left) with a pheochromocytoma (right) involving the adrenal medulla
Adrenal gland; the medulla (center, red) is the origin of the adrenal gland
There are two adrenal glands, highlighted in yellow, on top of each of the kidneys
Structure of epinephrine
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FDG PET – the tumor is appreciated as the dark structure in the patient's left chest. Darkened structures at head of patient is brain, in the abdomen are the kidneys, in the pelvis is the bladder. These are normal.
Histopathology on the resected tumor confirms the diagnosis, by typical features as shown.
Patient receiving radiation therapy to the region of the head and neck. Full facial mold is in-place to protect areas where they do not want exposure
Top: Purple lesions are metastatic disease detected with DOTATATE imaging. Bottom: Same patient. Purple lesions are metastatic disease detected with FDG PET
Likelihood of diagnosis when an adrenal-nodule is identified; pheochromocytoma is in yellow near the top-right corner
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The Zebra has become a powerful symbol in the pheochromocytoma advocacy community and represents the rare medical cases that are more likely to be misdiagnosed

Rare tumor of the adrenal medulla composed of chromaffin cells, also known as pheochromocytes.

- Pheochromocytoma
Normal remnant adrenal gland (left) with a pheochromocytoma (right) involving the adrenal medulla

53 related topics with Alpha

Overall

Skeletal formula of propranolol, the first clinically successful beta blocker

Beta blocker

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Beta blockers, also spelled β-blockers, are a class of medications that are predominantly used to manage abnormal heart rhythms, and to protect the heart from a second heart attack after a first heart attack (secondary prevention).

Beta blockers, also spelled β-blockers, are a class of medications that are predominantly used to manage abnormal heart rhythms, and to protect the heart from a second heart attack after a first heart attack (secondary prevention).

Skeletal formula of propranolol, the first clinically successful beta blocker
Dichloroisoprenaline, the first beta blocker

Phaeochromocytoma, in conjunction with α-blocker

The biosynthesis of adrenaline involves a series of enzymatic reactions.

Adrenaline

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Hormone and medication which is involved in regulating visceral functions .

Hormone and medication which is involved in regulating visceral functions .

The biosynthesis of adrenaline involves a series of enzymatic reactions.

Increased adrenaline secretion is observed in pheochromocytoma, hypoglycemia, myocardial infarction and to a lesser degree in essential tremor (also known as benign, familial or idiopathic tremor).

Adrenal gland. (Medulla labeled at bottom right.)

Chromaffin cell

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Chromaffin cells, also called pheochromocytes (or phaeochromocytes), are neuroendocrine cells found mostly in the medulla of the adrenal glands in mammals.

Chromaffin cells, also called pheochromocytes (or phaeochromocytes), are neuroendocrine cells found mostly in the medulla of the adrenal glands in mammals.

Adrenal gland. (Medulla labeled at bottom right.)
Adrenaline (Epinephrine)
Noradrenaline (Norepinephrine)
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Tumors arising from these cell are called paragangliomas or pheochromocytomas.

Catechol

Catecholamine

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Monoamine neurotransmitter, an organic compound that has a catechol (benzene with two hydroxyl side groups next to each other) and a side-chain amine.

Monoamine neurotransmitter, an organic compound that has a catechol (benzene with two hydroxyl side groups next to each other) and a side-chain amine.

Catechol

Extremely high levels of catecholamine can also be caused by neuroendocrine tumors in the adrenal medulla, a treatable condition known as pheochromocytoma.

Skeletal formula of noradrenaline

Norepinephrine

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Organic chemical in the catecholamine family that functions in the brain and body as both a hormone and neurotransmitter.

Organic chemical in the catecholamine family that functions in the brain and body as both a hormone and neurotransmitter.

Skeletal formula of noradrenaline
Norepinephrine degradation. Metabolizing enzymes are shown in boxes.
Norepinephrine (labeled "noradrénaline" in this drawing) processing in a synapse. After release norepinephrine can either be taken up again by the presynaptic terminal, or broken down by enzymes.
Schema of the sympathetic nervous system, showing the sympathetic ganglia and the parts of the body to which they connect.
Brain areas containing noradrenergic neurons.
Chemical structure of octopamine, which serves as the homologue of norepinephrine in many invertebrate species

A pheochromocytoma is a rarely occurring tumor of the adrenal medulla, caused either by genetic factors or certain types of cancer.

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Alpha blocker

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Alpha-blockers, also known as α-blockers or α-adrenoreceptor antagonists, are a class of pharmacological agents that act as antagonists on α-adrenergic receptors (α-adrenoceptors).

Alpha-blockers, also known as α-blockers or α-adrenoreceptor antagonists, are a class of pharmacological agents that act as antagonists on α-adrenergic receptors (α-adrenoceptors).

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Schematic of G Protein Coupled Receptor Signaling, representing Gi GPCR signaling, Gs GPCR signaling, and Gq GPCR signaling.
An image of a patient with pheochromocytoma. In patients with this disease, a catecholamine-secreting tumor is formed, and causes excess CNS stimulation, such as excess sweating and tachycardia. Nonselective alpha blockers, such as phenoxybenzamine or phentolamine, can be used to mitigate this disease.
Benign prostate hyperplasia, a disease in which urinary retention becomes an issue. Alpha-1 blockers can be used, but it can result in side effects such as increased urination and retrograde ejaculation.
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While there are limited clinical α-blocker uses, in which most α-blockers are used for hypertension or benign prostatic hyperplasia, α-blockers can be used to treat a few other diseases, such as Raynaud's disease, congestive heart failure (CHF), pheochromocytoma, and erectile dysfunction.

Medulla labeled at bottom right.

Adrenal medulla

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Part of the adrenal gland.

Part of the adrenal gland.

Medulla labeled at bottom right.
In H&E staining the adrenal medulla (on the pointer) stains lighter than the adrenal cortex.

Pheochromocytoma (most common), a catecholamine-secreting tumor of the adrenal medulla

Automated arm blood pressure meter showing arterial hypertension (shown by a systolic blood pressure 158 mmHg, diastolic blood pressure 99 mmHg and heart rate of 80 beats per minute)

Hypertension

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Long-term medical condition in which the blood pressure in the arteries is persistently elevated.

Long-term medical condition in which the blood pressure in the arteries is persistently elevated.

Automated arm blood pressure meter showing arterial hypertension (shown by a systolic blood pressure 158 mmHg, diastolic blood pressure 99 mmHg and heart rate of 80 beats per minute)
Determinants of mean arterial pressure
Illustration depicting the effects of high blood pressure
Rates of hypertension in adult men in 2014.
Diagram illustrating the main complications of persistent high blood pressure
Image of veins from Harvey's Exercitatio Anatomica de Motu Cordis et Sanguinis in Animalibus
Graph showing, prevalence of awareness, treatment and control of hypertension compared between the four studies of NHANES

Pheochromocytoma may cause abrupt episodes of hypertension accompanied by headache, palpitations, pale appearance, and excessive sweating.

Micrograph of a carotid body tumour (a type of paraganglioma).

Paraganglioma

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Rare neuroendocrine neoplasm that may develop at various body sites .

Rare neuroendocrine neoplasm that may develop at various body sites .

Micrograph of a carotid body tumour (a type of paraganglioma).
Mediastinal paraganglioma. The cut surface of a 3.9 × 3.5 × 2.5 cm tumor is triangular, with a bulging peripheral portion and a somewhat fibrotic center. It was surrounded by the heart, left lower lobe of the lung, aorta, esophagus, and diaphragm, and had been 1.8 cm in diameter 7 years before.
Micrograph of a carotid body tumor with the characteristic Zellballen. H&E stain.
Micrograph of a carotid body tumor
Glomus jugulare tumor
Ectopic functional paraganglioma (glomus jugulare) in a patient with VHL. T2 weighted MRI at the same location demonstrates a high signal mass consistent with a paraganglioma. Extra adrenal paragangliomas can be found in VHL (arrow).
S100 immunostain highlighting the sustentacular cells in a paraganglioma
Digital subtraction arteriogram of carotid body tumor and jugular paraganglioma

When the same type of tumor is found in the adrenal gland, they are referred to as a pheochromocytoma.

The adrenal glands lie above the kidneys.

Adrenal gland

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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.

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.

The adrenal glands lie above the kidneys.
Adrenal glands, anterior (left) and posterior (right) surface.
Section of human adrenal gland under the microscope, showing its different layers. From the surface to the center: zona glomerulosa, zona fasciculata, zona reticularis, medulla. In the medulla, the central adrenomedullary vein is visible.
Different hormones are produced in different zones of the cortex and medulla of the gland. Light microscopy at magnification × 204.
Steroidogenesis in the adrenal glands – different steps occur in different layers of the gland
Negative feedback in the HPA axis
Characteristic skin hyperpigmentation in Addison's disease
Incidences and prognoses of adrenal tumors.

Pheochromocytomas are tumors of the adrenal medulla that arise from chromaffin cells.