Pheochromocytoma natural history, complications and prognosis: Difference between revisions

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{{Pheochromocytoma}}  
{{Pheochromocytoma}}  
{{CMG}}; {{AE}} {{AAM}}
{{CMG}}; {{AE}} {{AAM}} {{MAD}}
 
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==Overview==
==Overview==
The prognosis of pheochromocytoma is generally good but metastatic pheochromocytoma has a 5-year survival rate of approximately 45%.<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> Massive release of [[catecholamines]] can causes damage to myocytes.
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, Complication and Prognosis==
===Natural History===
===Natural History===
* The symptoms of pheochromocytoma usually develop in the fifth decade of life.
* Common symptoms are with [[tachycardia]], [[hypertension]], [[headache]], and [[sweating]].
* If left untreated, [[hyperglycemia]] and  [[Hypertensive crisis|hypertensive emergency]]. It may lead to [[heart failure]] and[[Stroke|cerebrovascular strokes]].
* 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>


===Complication===
===Complications===
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" />
Common complications of pheochromocytoma include:
Other complications may 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]]
*[[Hyperglycemia]] due to opposition of [[insulin]] effect by high doses of [[adrenaline]] secreted by the [[tumor]].
*[[Refractory hypertension]]
*[[Malignant hypertension]] that may cause [[Cerebrovascular event|cerebrovascular]] accidents such as:
*[[Malignant hypertension]]
**[[Intracranial hemorrhage]]
*[[Metastasis]] to [[lymph nodes]], [[bones]], [[lungs]], and [[liver]]
**[[Acute coronary syndrome]]
**[[Aortic dissection]]
**[[Heart failure]]
*[[Metastasis]] to:
**[[Lymph node|Lymph nodes]]  
**[[Bones]]  
**[[Lung|Lungs]]  
**[[Liver]]


===Prognosis===
===Prognosis===
five-year survival rates that range widely from 12 to 84 percent [3,5-15]. Some of this variability has to do with differing definitions of malignancy.
* 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>
* Prognosis and survival rate varies with the location of the primary [[tumor]], sites of [[Metastasis|metastases]], [[Tumor]] burden, and rate of progression.
* [[Metastasis]] to the [[brain]] and [[liver]] has a worse [[prognosis]] than other [[metastases]].


Among malignant tumors, the survival rate may depend on the primary tumor site and sites of metastases. Outcomes are most variable for patients with malignant paragangliomas of the skull base and neck, most of which are nonsecretory. The following represents the range of findings:
'''Post-surgical prognosis'''
 
* Factors associated with a favourable [[prognosis]] include:
●In one single institution series of 19 patients with malignant skull base and neck paraganglioma treated between 1970 and 2005, the five-year survival rate was 84 percent, despite the fact that 14 had distant metastases [10].
** Small [[tumor]] size
 
** Short duration of surgery
●On the other hand, in another report of 86 cases of malignant head and neck paraganglioma reported to the National Cancer Institute Surveillance, Epidemiology, and End Results (SEER) database from 1973 to 2009, the five-year survival rate was 65 percent overall; for those with regionally confined metastases (n = 47), it was 82 percent, compared with 41 percent for those with distant metastases (n = 39) [15]. Outcomes were more favorable for carotid body tumors than for malignant tumors at other sites (five-year survival 87 versus 48 percent).
** [[Systolic blood pressure]] < 160 mmHg
 
** 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>
●An earlier report of data on 59 cases of malignant paraganglioma of the skull base and neck extracted from the National Cancer Database demonstrated a five-year survival rate of 77 percent for regionally-confined metastases, but only 12 percent for patients with distant metastatic disease [3].
* 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>
●patients with metastatic pheochromocytoma five-year survival rates are 34 to 60 percent, averaging approximately 50 percent [5-9,11-14,16]. Even 10-year survival rates of 25 percent are reported [16]. However, others report that outcomes are poorer with pheochromocytomas compared with paraganglioma, regardless of functionality [17]. In this study, pheochromocytomas presented more often with distant metastases and with larger tumors; overall survival at five years was 58 percent compared with 80 percent for paraganglioma.
 
Prognosis is impacted by tumor burden, location of metastases, and rate of progression; patients with brain, liver, and lung metastases tend to have a worse prognosis than do those with isolated bone lesions [18].
* Approximately 10% recur after being resected.


==References==
==References==
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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.