Pheochromocytoma history and symptoms: Difference between revisions

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{{Pheochromocytoma}}
{{Pheochromocytoma}}
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==Overview==
==Overview==
The hallmarks of [[pheochromocytoma]] is recurrent episodes of sympathetic symptoms. The most common [[Symptom|symptoms]] of pheochromocytoma include episodes of [[palpitations]], [[sweating]] and [[headaches]]. Less common [[symptoms]] include [[cardiomyopathy|cardiomypathy]], episodic [[hypotension]], [[symptoms]] of other [[Multiple endocrine neoplasia|multiple endocrine neoplasias]] associated diseases such as that of [[medullary thyroid cancer]] and [[hyperparathyroidism]].


A '''pheochromocytoma''' ('''phaeochromocytoma''' outside of the US) is a [[neuroendocrine tumor]] of the [[Adrenal medulla|medulla]] of the [[adrenal gland]]s (originating in the [[chromaffin cell]]s) or extra-adrenal chromaffin tissue which failed to involute after birth,<ref>{{cite book |author=Boulpaep, Emile L.; Boron, Walter F. |title=Medical physiology: a cellular and molecular approach |publisher=Saunders |location=Philadelphia |year=2003 |pages=1065 |isbn=0-7216-3256-4 |oclc= |doi=}}</ref> which secretes excessive amounts of [[catecholamine]]s, usually [[epinephrine]] and [[norepinephrine]].
==History and Symptoms==
Extra-adrenal [[paragangliomas]] (often described as extra-adrenal pheochromocytomas) are closely related, though less common, tumors that originate in the [[ganglion|ganglia]] of the [[sympathetic nervous system]] and are named based upon the primary anatomical site of origin.  
The hallmark of [[pheochromocytoma]] is recurrent episodes of [[Palpitation|palpitations]], [[sweating]], and [[headache]].  
==History and symptoms==
The [[signs]] and [[symptoms]] of a pheochromocytoma are those of [[sympathetic nervous system]] [[hyperactivity]], including:


*[[Elevated heart rate]]
===History===
*[[Elevated blood pressure]], including paroxysmal (sporadic, episodic) high blood pressure, which sometimes can be more difficult to detect; another clue to the presence of pheochromocytoma is [[orthostatic hypotension]] (a fall in [[systolic blood pressure]] greater than 20 [[mmHg]] or a fall in [[diastolic blood pressure]] greater than 10 [[mmHg]] on making the patient stand)
Patients with [[pheochromocytoma]] may have a positive history of:
*[[Palpitations]]
 
*[[Anxiety]] often resembling that of a [[panic attack]]
*Recurrent attacks of [[palpitations]]
*[[Diaphoresis]]
*[[Headache]] and [[Perspiration|sweating]]
*[[Headaches]]
*Paroxysmal changes in [[blood pressure]]
*[[Pallor]]
*[[Family history]] of [[pheochromocytoma]]
*[[Weight loss]]
*Family history of syndromes- [[MEN2|MEN 2A]], [[Multiple endocrine neoplasia type 2|MEN 2B]], [[NF1]], [[Von Hippel-Lindau disease|VHL]]
*Localized [[amyloid]] deposits found microscopically
*Elevated blood glucose level (due primarily to catecholamine stimulation of [[lipolysis]] (breakdown of stored fat) leading to high levels of [[free fatty acids]] and the subsequent inhibition of glucose uptake by muscle cells. Further, stimulation of beta-adrenergic receptors leads to glycogenolysis and gluconeogenesis and thus elevation of blood glucose levels).


A pheochromocytoma can also cause resistant [[arterial hypertension]]. A pheochromocytoma can be fatal if it causes [[malignant hypertension]], or severely [[high blood pressure]]. This hypertension is not well controlled with standard blood pressure medications.
===Common symptoms===
Common symptoms of [[pheochromocytoma]] are secondary to [[sympathetic nervous system]] hyperactivity. [[Symptoms]] usually subside in less than one hour and they may include:


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.
*[[Palpitations]] especially in epinephrine producing tumors.<ref name="pmid8325290">{{cite journal| author=Bravo EL, Gifford RW| title=Pheochromocytoma. | journal=Endocrinol Metab Clin North Am | year= 1993 | volume= 22 | issue= 2 | pages= 329-41 | pmid=8325290 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8325290  }}</ref>
*[[Anxiety]] often resembling that of a [[panic attack]]
*[[Sweating]]
*[[Headaches]] 
*Paroxysmal attacks of [[hypertension]] but some patients have normal [[blood pressure]]
*Pheochromocytoma may be [[asymptomatic]] and discovered incidentally after [[Screening (medicine)|screening]] for [[MEN, type 2|MEN]] patients


Tumors may grow very large, but most are smaller than 10 cm.
=== Less common symptoms ===
==Tumor Location==
Less common symptoms of pheochromocytoma include:
In adults, 90% tumors are located unilaterally and are solitary, and 10% are located outside the adrenal gland. In children 50% are adrenal, while 25% are bilateral and 25% are extraadrenal. The common extradrenal locations are the abdomen, thorax and urinary bladder.
* [[Cardiomyopathy]]: [[dyspnea on exertion]], [[leg swelling]], painful [[hepatomegaly]].<ref name="pmid19158054">{{cite journal| author=Kassim TA, Clarke DD, Mai VQ, Clyde PW, Mohamed Shakir KM| title=Catecholamine-induced cardiomyopathy. | journal=Endocr Pract | year= 2008 | volume= 14 | issue= 9 | pages= 1137-49 | pmid=19158054 | doi=10.4158/EP.14.9.1137 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19158054  }}</ref>
* Episodic [[hypotension]].<ref name="pmid8076587">{{cite journal| author=Bravo EL| title=Evolving concepts in the pathophysiology, diagnosis, and treatment of pheochromocytoma. | journal=Endocr Rev | year= 1994 | volume= 15 | issue= 3 | pages= 356-68 | pmid=8076587 | doi=10.1210/edrv-15-3-356 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8076587  }}</ref>
* Pheochromocytoma patients may show symptoms of other diseases associated with syndromes such as:
**[[Multiple endocrine neoplasia type 2|MEN 2]]
*** [[Hyperparathyroidism]]
*** [[Marfan's syndrome|Marfanoid features]]
*** [[Medullary thyroid cancer]]
**[[Von Hippel-Lindau Disease|Von Hippel-Lindau disease]]
***[[Angiomatosis]]
***[[Hemangioblastoma]]s
***[[Renal cell carcinoma]]
***[[Cyst|Cysts]]/[[Tumor|tumors]] of the [[pancreas]]. <ref name="pmid10458336">{{cite journal| author=Walther MM, Reiter R, Keiser HR, Choyke PL, Venzon D, Hurley K et al.| title=Clinical and genetic characterization of pheochromocytoma in von Hippel-Lindau families: comparison with sporadic pheochromocytoma gives insight into natural history of pheochromocytoma. | journal=J Urol | year= 1999 | volume= 162 | issue= 3 Pt 1 | pages= 659-64 | pmid=10458336 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10458336  }}</ref>


==References==
=References=
{{Reflist|2}}
{{Reflist|2}}


[[Category:Endocrinology]]
[[Category:Endocrinology]]
[[Category:Oncology]]
[[de:Phäochromozytom]]
[[es:Feocromocitoma]]
[[fr:Phéochromocytome]]
[[it:Feocromocitoma]]
[[he:פאוכרומוציטומה]]
[[nl:Feochromocytoom]]
[[ja:褐色細胞腫]]
[[pl:Guz chromochłonny nadnerczy]]
[[sv:Feokromocytom]]


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Latest revision as of 20:45, 28 July 2020

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Ahmad Al Maradni, M.D. [2]Mohammed Abdelwahed M.D[3]

Overview

The hallmarks of pheochromocytoma is recurrent episodes of sympathetic symptoms. The most common symptoms of pheochromocytoma include episodes of palpitations, sweating and headaches. Less common symptoms include cardiomypathy, episodic hypotension, symptoms of other multiple endocrine neoplasias associated diseases such as that of medullary thyroid cancer and hyperparathyroidism.

History and Symptoms

The hallmark of pheochromocytoma is recurrent episodes of palpitations, sweating, and headache.

History

Patients with pheochromocytoma may have a positive history of:

Common symptoms

Common symptoms of pheochromocytoma are secondary to sympathetic nervous system hyperactivity. Symptoms usually subside in less than one hour and they may include:

Less common symptoms

Less common symptoms of pheochromocytoma include:

References

  1. Bravo EL, Gifford RW (1993). "Pheochromocytoma". Endocrinol Metab Clin North Am. 22 (2): 329–41. PMID 8325290.
  2. Kassim TA, Clarke DD, Mai VQ, Clyde PW, Mohamed Shakir KM (2008). "Catecholamine-induced cardiomyopathy". Endocr Pract. 14 (9): 1137–49. doi:10.4158/EP.14.9.1137. PMID 19158054.
  3. Bravo EL (1994). "Evolving concepts in the pathophysiology, diagnosis, and treatment of pheochromocytoma". Endocr Rev. 15 (3): 356–68. doi:10.1210/edrv-15-3-356. PMID 8076587.
  4. Walther MM, Reiter R, Keiser HR, Choyke PL, Venzon D, Hurley K; et al. (1999). "Clinical and genetic characterization of pheochromocytoma in von Hippel-Lindau families: comparison with sporadic pheochromocytoma gives insight into natural history of pheochromocytoma". J Urol. 162 (3 Pt 1): 659–64. PMID 10458336.

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