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Pheochromocytoma

**{{{Name|Pheochromocytoma}}}**
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(ICD-O8700) |

 ICD9        = 255.6|

}} A pheochromocytoma (also phaeochromocytoma, English spelling) is a tumorof the medullaof the adrenal glands originating in the chromaffincells, which secretes excessive amounts of catecholamines, usually epinephrineand norepinephrine. Extra-adrenal paragangliomas(often described as extra-adrenal pheochromocytomas) are closely related, though less common, tumors that originate in the gangliaof the sympathetic nervous systemand are named based upon the primary anatomic site of origin.

Inhaltsverzeichnis

1 Inheritance
2 Features
3 Diagnosis
- 3.1 Differential diagnosis
4 Treatment
5 External links

Inheritance

Up to 25% of pheochromocytomas may be familial. Mutations of the genes VHL, RET, NF1, SDHB and SDHD are all known to cause familial pheochromocytoma/extra-adrenal paraganglioma.

Features

The signsand symptomsof a pheochromocytoma are those of sympathetic nervous system hyperactivity:

elevated heart rate
elevated blood pressure
palpitations
anxietyoften resembling that of a panic attack
diaphoresis
headaches

A pheochromocytoma can also cause resistant arterial hypertension. A pheochromocytoma can be fatal if it causes malignant hypertension, or severely high blood pressure.

Diagnosis

The diagnosis can be established by measuring catecholamine and metanefrine in plasma or urine. One diagnostic test used in the past for a pheochromocytoma is to administer clonidine(Catapres®), a centrally-acting alpha-2 agonist used to treat high blood pressure. Clonidine mimics catecholamines in the brain, causing it to reduce the activity of the sympathetic nerves controlling the adrenal medulla. A healthy adrenal medulla will respond to clonidine by reducing catecholamine production; the lack of a response is evidence of pheochromocytoma. Another test is for the clinician to press gently on the adrenal gland. A pheochromocytoma will often release a burst of catecholamines, with the associated signs and symptoms quickly following.

Pheochromocytomae occur most often during young-adult to mid-adult life. Less than 10% of pheochromocytomas are malignant(cancerous).

These tumors can form a pattern with other endocrine gland cancers which is labelled multiple endocrine neoplasia(MEN). Pheochromocytoma may occur in patients with MEN 2a and MEN 2b.

Differential diagnosis

The differential diagnosisof pheochromocytoma includes:

Anxiety disorders
Carcinoid tumor
Essential hypertension
Hyperthyroidism
Insulinoma
Paroxysmal supraventricular tachycardia
Renovascular hypertension

Treatment

Surgical resectionof the tumor is the treatment of first choice.

External links

OMIM171300
NIH/Medline
eMedicine
National Cancer Institutefr:Phéochromocytome

nl:Feochromocytoom sv:Feokromocytom

This article is licensed under the GNU Free Documentation License.
It uses material from the http://en.wikipedia.org/wiki/Pheochromocytoma Wikipedia article Pheochromocytoma.

All text is available under the terms of the GNU Free Documentation License