Pheochromocytoma
| Classification according to ICD-10 | |
|---|---|
| C74.1 | Malignant neoplasm of the adrenal medulla |
| D35.0 | Benign neoplasm of the adrenal gland |
| D44.1 | Adrenal gland growth of unsafe or unfamiliar behavior |
| E27.5 | Overactive adrenal medulla |
| ICD-10 online (WHO version 2019) | |
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The pheochromocytoma (from Greek phaios "dark", chroma "color", neo-Latin cytus "cell", -oma "tumor") is a disease of the chromaffin cells of the adrenal medulla with an incidence of 1 / 100,000 people / year. It is a tumor that produces catecholamines ( noradrenaline , adrenaline, and metanephrines ) which cause high blood pressure . Malignant pheochromocytomas also produce dopamine. 85% of it is located in the adrenal medulla, but also occurs in the nerve tissues of the thoracic and abdominal trunk (paraganglioma), but here it almost exclusively produces noradrenaline. Pheochromocytoma very rarely occurs, for example, in the urinary bladder and other tissues derived from neural crest cells. About 10% of pheochromocytomas are malignant ; different percentages can be found in the literature.
The pheochromocytoma occurs in isolation or in the context of a MEN-2 syndrome (multiple endocrine neoplasias), in von Hippel-Lindau syndrome type 2 and in neurofibromatosis type 1 (Recklinghausen's disease).
Initial description
The first description of a pheochromocytoma goes back to Felix Fränkel in 1886, who reported the case of 18-year-old Minna Roll from Wittenweier, who died within ten days in the Freiburg University Hospital . An adrenal and angiosarcoma has been described pathologically in both kidneys . In the preparation of the preparation, the chromate- containing fixation solution according to Müller was used, which only stained the tumor cells brownish, which was referred to in 1912 by the Berlin pathologist Ludwig Pick as chromate brown , or phaeochrom (Greek). He was the first to use the term pheochromocytoma. In a more recent study, surviving relatives of the first patient were able to demonstrate that she suffered from a multiple endocrine neoplasia MEN-2.
Symptoms
The patient complains mainly about one (by releasing stress hormones related) paroxysmal hypertension (paroxysmal hypertension) or permanent hypertension (persistent hypertension, often in children). Headache , dizziness , rapid heartbeat and sweating occur during phases of increased blood pressure . Other signs include pale skin, increased blood sugar (hyperglycaemia), leukocytosis and weight loss. Fainting, nausea, vomiting, increased tears and saliva may occur in attacks.
diagnosis
Left: from the front. Right: from behind.
The diagnosis is based on the suspicious clinical symptoms. Particularly suspicious are sporadic attacks of high blood pressure that do not respond to conventional drug therapies. Since a pheochromocytoma represents a very serious risk for the affected patient, reliable laboratory diagnostics must be carried out.
A number of methods are available for laboratory tests. These are primarily the determination of catecholamines or their breakdown products from urine or plasma. The determination of vanillin-mandelic acid is no longer indicated today because of its low sensitivity. It is better to determine the catecholamines (adrenaline, noradrenaline) from the 24-hour urine collection or from plasma samples or to determine the metanephrines (metanephrine and normetanephrine) from the 24-hour urine. The disadvantage of the aforementioned laboratory methods, however, is that neither a positive nor a negative result enables a reliable statement.
This is only possible through the quantitative determination of the free metanephrines in the plasma. If these are not increased, a pheochromocytoma can be ruled out with certainty. The diagnostic sensitivity of this method is almost 100%, which has been shown in several international studies.
If, on the other hand, the metanephrines in the plasma are increased even in repeated cases, there is a strong suspicion of a pheochromocytoma. Recently, the quantitative determination of free plasma metanephrines with a routine determination ( RIA or ELISA method) can be carried out in any well-equipped laboratory. The plasma collection may only be carried out after a 20-minute rest phase while lying down, as otherwise there will be too many false-positive results.
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CT of the upper abdomen.
The * marks the pheochromocytoma. |
SPECT with MIBG in a comparable cut.
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If the determination of the plasma metanephrine repeatedly produces a positive result, further localization diagnostics must be carried out. This is done using imaging methods such as computed tomography and sonography or MRI imaging. With CT it must be taken into account that catecholamines can be released when iodine contrast media enters , which is not the case with MRI. The nuclear medicine method of MIBG scintigraphy (metaiodobenzylguanidine) is mainly used to exclude pheochromocytomas outside the adrenal gland. This substance is mainly deposited in the affected chromaffin cells of the pheochromocytoma. The newest and most reliable form of the nuclear medicine method is the so-called DOPA-PET for pheochromcytomas . It is currently (as of 2012) only offered in a few centers in Germany. In addition, tumors of a possible MEN syndrome must be looked for in any case.
Differential diagnosis
Other tumors of the adrenal gland (e.g. ganglioneuroma ) must be excluded from the differential diagnosis . Pheochromocytoma can be ruled out in 98% of patients with episodic hypertension. Then there is often severe paroxysmal arterial hypertension , which is also known as pseudophaeochromocytoma .
A variety of other medical conditions can lead to anfallsartigem hypertension and needs in the differential diagnosis be included: anxiety disorder , panic disorder , hyperthyroidism , beta-adrenergic hyperdynamic circulation disorder , cluster headache , migraine , hypertensive encephalopathy , coronary heart disease , renal artery stenosis , stroke , brain tumor , brain injury , brain hemorrhage , compression of the medulla oblongata , seizure disorders , carcinoid , drugs such as cocaine , ergot alkaloids , diethylamine , amphetamines , tyrosine in combination with monoamine oxidase inhibitors , impaired baroreceptor - reflex and Munchausen syndrome .
therapy
Surgical therapy involves surgical resection of the tumor. At least one week before the procedure, an alpha-blocker (e.g. phenoxybenzamine ) is used to lower blood pressure . Then you add an unselective beta blocker . This administration takes place because large amounts of catecholamines are released during the surgical removal of the tumor, which without appropriate blockers could be life-threatening. The alpha blockers must always be given before the beta blockers, otherwise a hypertensive crisis threatens. If beta-blockers are given first in a pheochromocytoma, the vasodilatory effect mediated by the β2 receptor disappears and the blood pressure rises (stimulation of the α1 receptors causes vasoconstriction ).
In the case of a unilateral pheochromocytoma, a total removal of the adrenal gland from this side is carried out; in the context of a MEN syndrome , only the adrenal medulla is removed on both sides. In 80% of patients, catecholamine levels and blood pressure normalize postoperatively.
If metastases develop , systemic radionuclide therapy ( MIBG therapy ) or polychemotherapy are carried out. Other options are interferons and octreotide .
literature
- Graeme Eisenhofer: Biochemical Diagnosis of Pheochromocytoma - Is it Time to Switch to Plasma-Free Metanephrines? In: J Clin Endocrinol Metab , 88 (2), pp. 550-552, PMID 12574178 .
- Heinz Lüllmann et al .: Pharmacology and Toxicology. 16th edition, Thieme Verlag, Stuttgart 2006, ISBN 3-13-368516-3 .
- Lois Jovanovic, Genell J. Subak-Sharpe: Hormones. The medical manual for women. (Original edition: Hormones. The Woman's Answerbook. Atheneum, New York 1987) From the American by Margaret Auer, Kabel, Hamburg 1989, ISBN 3-8225-0100-X , p. 319 f. and 383.
Web links
- MSD manual. Medical Edition: Pheochromocytoma .
- Further literature: www.pheochromocytoma.org
Individual evidence
- ↑ H. Lipsky, G. Leitner: The pheochromocytoma of the urinary bladder. In: Current Urology. 20, 1989, p. 342, doi: 10.1055 / s-2008-1061238 .
- ↑ S. Vyas, N. Kalra, SK Singh, MM Agarwal, AK Mandal, N. Khandelwal: pheochromocytoma of urinary bladder. In: Indian journal of nephrology. Volume 21, number 3, July 2011, pp. 198-200, doi: 10.4103 / 0971-4065.78072 , PMID 21886982 , PMC 3161440 (free full text).
- ^ HPH Neumann et al .: Evidence of MEN-2 in the original description of classic pheochromocytoma . In: New England Journal of Medicine , 2007, 57 (13), p. 1311
- ↑ Carsten Kunz, Winfried Kunz, Mechthild Seel: Compact knowledge of nursing . Schlütersche, 2004, ISBN 978-3-87706-759-8 , p. 345.
- ↑ JW Lenders, K. Pacak, MM Walther, WM Linehan, M. Mannelli, P. Friberg, HR Keizer, DS Goldstein, G. Eisenhofer: Biochemical diagnosis of pheochromocytoma: which test is best? In: Journal of the American Medical Association . Volume 287, Number 11, March 2002, pp. 1427-1434, PMID 11903030 . Full text (PDF) .
- ^ SJ Mann: Severe paroxysmal hypertension (pseudopheochromocytoma): understanding the cause and treatment. In: Archives of internal medicine. Vol. 159, Number 7, April 1999, pp. 670-674, PMID 10218745 (review). PDF .
- ↑ Tim Scholz et al .: Current Treatment of Malignant Pheochromocytoma . In: The Journal of Clinical Endocrinology & Metabolism , 2007, Vol. 92, No. 4, pp. 1217-1225.
