Pheochromocytoma natural history, complications and prognosis: Difference between revisions

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{{CMG}}; {{AE}} {{AAM}} {{MAD}}
{{CMG}}; {{AE}} {{AAM}} {{MAD}}
==Overview==
==Overview==
Pheochromocytoma is an [[adrenaline]] secreting tumor, usually develop in the fifth decade of life. Symptoms start with tachycardia, hypertension, headache and sweating.
Pheochromocytoma is an [[adrenaline]] secreting [[tumor]], that usually develops in the fifth decade of life. Symptoms start with [[tachycardia]], [[hypertension]], [[headache]] and [[sweating]]. Massive release of [[catecholamines]] may cause [[hyperglycemia]], [[malignant hypertension]] and [[metastasis]]. The [[prognosis]] of pheochromocytoma is generally good but metastatic pheochromocytoma has a 5-year survival rate of approximately 50%.
Massive release of [[catecholamines]] can cause [[hyperglycemia]], malignant hypertension and [[metastasis]]. The prognosis of pheochromocytoma is generally good but metastatic pheochromocytoma has a 5-year survival rate of approximately 50%<ref name="cancergov">National Cancer Institute. Physician Data Query Database 2015. http://www.cancer.gov/types/pheochromocytoma/hp/pheochromocytoma-treatment-pdq#link/_25_toc</ref>
==Natural History, Complications and Prognosis==
==Natural History, Complication and Prognosis==
===Natural History===
===Natural History===
* Pheochromocytoma is the adrenaline secreting tumor, usually develop in the fifth decade of life.  
* The symptoms of pheochromocytoma usually develop in the fifth decade of life.
*Fifty percent of pheochromocytomas in children are solitary intra-adrenal lesions, 25% are present bilaterally, and 25% are extra-adrenal.
* Common symptoms are with [[tachycardia]], [[hypertension]], [[headache]], and [[sweating]].
*Most catecholamine-secreting tumors are sporadic.
* If left untreated, [[hyperglycemia]] and  [[Hypertensive crisis|hypertensive emergency]]. It may lead to [[heart failure]] and[[Stroke|cerebrovascular strokes]].  
*However, 30% of patients got the tumor as a part of the familial disease, the catecholamine-secreting tumors are more likely to be bilateral.<ref name="pmid28324046">{{cite journal| author=Pamporaki C, Hamplova B, Peitzsch M, Prejbisz A, Beuschlein F, Timmers HJLM et al.| title=Characteristics of Pediatric vs Adult Pheochromocytomas and Paragangliomas. | journal=J Clin Endocrinol Metab | year= 2017 | volume= 102 | issue= 4 | pages= 1122-1132 | pmid=28324046 | doi=10.1210/jc.2016-3829 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28324046  }}</ref>
* If [[malignant]], It can [[metastasize]] to [[lymph nodes]], [[bones]], [[lungs]], and [[liver]]. <ref name="pmid28324046">{{cite journal| author=Pamporaki C, Hamplova B, Peitzsch M, Prejbisz A, Beuschlein F, Timmers HJLM et al.| title=Characteristics of Pediatric vs Adult Pheochromocytomas and Paragangliomas. | journal=J Clin Endocrinol Metab | year= 2017 | volume= 102 | issue= 4 | pages= 1122-1132 | pmid=28324046 | doi=10.1210/jc.2016-3829 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28324046  }}</ref>
Symptoms start with tachycardia, hypertension, headache and sweating. If left untreated it causes hyperglycemia, [[Hypertensive Emergencies|hypertension emergency]] that causes heart failure, [[Stroke|cerebrovascular strokes]]. If malignant, It can metastasize to [[lymph nodes]], [[bones]], [[lungs]], and [[liver]].
 
===Complications===
Common complications of pheochromocytoma include:
* Damage to [[cardiac myocytes]] due to either a compromise of the [[coronary microcirculation]] or the direct toxic effects of [[catecholamines]] on [[cardiac myocytes]].<ref name="Goldman_327">{{Harvnb|Goldman|2011|pp=327}}</ref>
*[[Hyperglycemia]] due to opposition of [[insulin]] effect by high doses of [[adrenaline]] secreted by the [[tumor]].
*[[Malignant hypertension]] that may cause [[Cerebrovascular event|cerebrovascular]] accidents such as:
**[[Intracranial hemorrhage]]
**[[Acute coronary syndrome]]
**[[Aortic dissection]]
**[[Heart failure]]
*[[Metastasis]] to:
**[[Lymph node|Lymph nodes]]  
**[[Bones]]  
**[[Lung|Lungs]]  
**[[Liver]]


===Complication===
* The massive release of [[catecholamines]] in pheochromocytoma can cause damage to [[myocytes]].<ref name="Goldman_327">{{Harvnb|Goldman|2011|pp=327}}</ref> This damage may be due to either a compromise of the [[coronary microcirculation]] or the direct toxic effects of catecholamines on myocytes.<ref name="Goldman_327" />
Other complications may include:
*[[Hyperglycemia]] due to opposing insulin effect by high doses of adrenaline secreted by the tumor.
*[[Malignant hypertension]] that may cause cerebrovascular accidents such as intracranial hemorrhage, coronary syndrome, aortic dissection and heart failure.
*[[Metastasis]] to [[lymph nodes]], [[bones]], [[lungs]], and [[liver]].
===Prognosis===
===Prognosis===
* Pheochromocytoma prognosis is good if treated.
* The [[prognosis]] of pheochromocytoma is generally good with treatment. The 5-year survival rate in patients with [[metastatic]] pheochromocytoma is approximately 50%.<ref name="cancergov">National Cancer Institute. Physician Data Query Database 2015. http://www.cancer.gov/types/pheochromocytoma/hp/pheochromocytoma-treatment-pdq#link/_25_toc</ref>  
* Approximately 10% recur after being resected.
* Prognosis and survival rate varies with the location of the primary [[tumor]], sites of [[Metastasis|metastases]], [[Tumor]] burden, and rate of progression.
* Patients with metastatic pheochromocytoma five-year survival rates are approximately 50%.<ref name="cancergov" />Survival rate may depend on the primary tumor site and sites of metastases.
* [[Metastasis]] to the [[brain]] and [[liver]] has a worse [[prognosis]] than other [[metastases]].
* Tumor burden, the location of metastases, and rate of progression are the main factors affecting prognosis. Metastases in the brain, the liver has a worse prognosis than other metastases.
 
* '''Prognosis of surgery'''
'''Post-surgical prognosis'''
**Good prognosis patients include small tumor size, short duration of surgery, systolic blood pressure less than 160 mmHg, and low levels of urinary catecholamines.<ref name="pmid20336320">{{cite journal| author=Murphy MM, Witkowski ER, Ng SC, McDade TP, Hill JS, Larkin AC et al.| title=Trends in adrenalectomy: a recent national review. | journal=Surg Endosc | year= 2010 | volume= 24 | issue= 10 | pages= 2518-26 | pmid=20336320 | doi=10.1007/s00464-010-0996-z | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20336320  }}</ref>
* Factors associated with a favourable [[prognosis]] include:
**Postoperative hypotension can be avoided by adequate fluid replacement.
** Small [[tumor]] size
**Recurrence is more in patients with familial pheochromocytoma and extra-adrenal tumors.<ref name="pmid11297571">{{cite journal| author=Plouin PF, Duclos JM, Soppelsa F, Boublil G, Chatellier G| title=Factors associated with perioperative morbidity and mortality in patients with pheochromocytoma: analysis of 165 operations at a single center. | journal=J Clin Endocrinol Metab | year= 2001 | volume= 86 | issue= 4 | pages= 1480-6 | pmid=11297571 | doi=10.1210/jcem.86.4.7392 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11297571  }}</ref>
** Short duration of surgery
**Familial pheochromocytomas have a high incidence of bilateral disease.
** [[Systolic blood pressure]] < 160 mmHg
**Partial [[adrenalectomy]] is recommended for these patients with bilateral pheochromocytomas to prevent permanent glucocorticoid deficiency.
** Low levels of [[urinary]] [[catecholamines]].<ref name="pmid20336320">{{cite journal| author=Murphy MM, Witkowski ER, Ng SC, McDade TP, Hill JS, Larkin AC et al.| title=Trends in adrenalectomy: a recent national review. | journal=Surg Endosc | year= 2010 | volume= 24 | issue= 10 | pages= 2518-26 | pmid=20336320 | doi=10.1007/s00464-010-0996-z | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20336320  }}</ref>
**Complete bilateral [[adrenalectomy]] is recommended for MEN2 patients. There is a high rate of recurrence than in sporadic cases. Follow up for a long duration after surgery is needed. Most patients should have annual biochemical screening.
* Approximately 10% recur after being [[Resection|resected]].  
* Recurrence is more common in patients with familial pheochromocytoma and extra-[[Adrenal gland|adrenal]] [[tumors]].<ref name="pmid11297571">{{cite journal| author=Plouin PF, Duclos JM, Soppelsa F, Boublil G, Chatellier G| title=Factors associated with perioperative morbidity and mortality in patients with pheochromocytoma: analysis of 165 operations at a single center. | journal=J Clin Endocrinol Metab | year= 2001 | volume= 86 | issue= 4 | pages= 1480-6 | pmid=11297571 | doi=10.1210/jcem.86.4.7392 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11297571  }}</ref>


==References==
==References==

Latest revision as of 20:21, 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

Pheochromocytoma is an adrenaline secreting tumor, that usually develops in the fifth decade of life. Symptoms start with tachycardia, hypertension, headache and sweating. Massive release of catecholamines may cause hyperglycemia, malignant hypertension and metastasis. The prognosis of pheochromocytoma is generally good but metastatic pheochromocytoma has a 5-year survival rate of approximately 50%.

Natural History, Complications and Prognosis

Natural History

Complications

Common complications of pheochromocytoma include:

Prognosis

Post-surgical prognosis

References

  1. ↑ Pamporaki C, Hamplova B, Peitzsch M, Prejbisz A, Beuschlein F, Timmers HJLM; et al. (2017). "Characteristics of Pediatric vs Adult Pheochromocytomas and Paragangliomas". J Clin Endocrinol Metab. 102 (4): 1122–1132. doi:10.1210/jc.2016-3829. PMID 28324046.
  2. ↑ Goldman 2011, pp. 327
  3. ↑ National Cancer Institute. Physician Data Query Database 2015. http://www.cancer.gov/types/pheochromocytoma/hp/pheochromocytoma-treatment-pdq#link/_25_toc
  4. ↑ Murphy MM, Witkowski ER, Ng SC, McDade TP, Hill JS, Larkin AC; et al. (2010). "Trends in adrenalectomy: a recent national review". Surg Endosc. 24 (10): 2518–26. doi:10.1007/s00464-010-0996-z. PMID 20336320.
  5. ↑ Plouin PF, Duclos JM, Soppelsa F, Boublil G, Chatellier G (2001). "Factors associated with perioperative morbidity and mortality in patients with pheochromocytoma: analysis of 165 operations at a single center". J Clin Endocrinol Metab. 86 (4): 1480–6. doi:10.1210/jcem.86.4.7392. PMID 11297571.
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