Pheochromocytoma
| Pheochromocytoma/Phaeochromocytoma | |
|---|---|
 |
|
| High magnification micrograph of a pheochromocytoma, showing the nested arrangement of cells (Zellballen) and stippled chromatin. H&E stain. | |
| Classification and external resources | |
| Specialty | Oncology |
| ICD-10 | D35.0, C74.1 |
| ICD-9-CM | 227.0, 194.0, 255.6 |
| ICD-O | M8700/0 |
| OMIM | 171300 |
| DiseasesDB | 9912 |
| MedlinePlus | 000340 |
| eMedicine | med/1816 radio/552 ped/1788 |
| Patient UK | Pheochromocytoma |
| MeSH | D010673 |
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, that 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.
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 a hypertensive emergency, that is, severely high blood pressure that impairs one or more organ systems (formerly called "malignant hypertension"). This hypertension is not well controlled with standard blood pressure medications.
Not all patients experience all of the signs and symptoms listed. The most common presentation is headache, excessive sweating, and increased heart rate, with the attack subsiding in less than one hour.
Tumors may grow large, but most are smaller than 10 centimetres (4 in).
Up to 25% of pheochromocytomas may be familial. Mutations of the genes VHL, RET, NF1 (Gene 17 Neurofibromatosis type 1), SDHB and SDHD are all known to cause familial pheochromocytoma, therefore this disease may be accompanied by Von Hippel–Lindau disease, neurofibromatosis, or familial paraganglioma depending on the mutation.
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Wikipedia