Pheochromocytoma natural history, complications and prognosis: Difference between revisions
Jump to navigation
Jump to search
Akshun Kalia (talk | contribs) No edit summary |
|||
| Line 3: | Line 3: | ||
{{CMG}}; {{AE}} {{AAM}} {{MAD}} | {{CMG}}; {{AE}} {{AAM}} {{MAD}} | ||
==Overview== | ==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]] | 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 and Prognosis== | ||
===Natural History=== | ===Natural History=== | ||
* Pheochromocytoma is the [[adrenaline]] secreting [[tumor]], that usually develop in the fifth decade of life. | * Pheochromocytoma is the [[adrenaline]] secreting [[tumor]], that usually develop in the fifth decade of life. | ||
*Fifty percent of pheochromocytomas in children are solitary intra-[[Adrenal gland|adrenal]] lesions, 25% are present bilaterally, and 25% are extra-[[Adrenal gland|adrenal]]. | *Fifty percent of pheochromocytomas in children are solitary intra-[[Adrenal gland|adrenal]] lesions, 25% are present bilaterally, and 25% are extra-[[Adrenal gland|adrenal]]. | ||
* | *Majority of [[catecholamine]]-secreting [[Tumor|tumors]] are sporadic. | ||
*However, 30% of patients get the [[tumor]] as a part of the familial disease. These [[catecholamine]]-secreting [[Tumor|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> | *However, 30% of patients get the [[tumor]] as a part of the familial disease. These [[catecholamine]]-secreting [[Tumor|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> | ||
Symptoms start with [[tachycardia]], [[hypertension]], [[headache]], and [[sweating]]. If left untreated, it leads to [[hyperglycemia]] and [[Hypertensive crisis|hypertensive emergency]] that causes [[heart failure]] and[[Stroke|cerebrovascular strokes]]. If [[malignant]], It can [[metastasize]] to [[lymph nodes]], [[bones]], [[lungs]], and [[liver]]. | Symptoms start with [[tachycardia]], [[hypertension]], [[headache]], and [[sweating]]. If left untreated, it leads to [[hyperglycemia]] and [[Hypertensive crisis|hypertensive emergency]] that causes [[heart failure]] and[[Stroke|cerebrovascular strokes]]. If [[malignant]], It can [[metastasize]] to [[lymph nodes]], [[bones]], [[lungs]], and [[liver]]. | ||
Revision as of 15:27, 12 October 2017
|
Pheochromocytoma Microchapters |
|
Diagnosis |
|---|
|
Treatment |
|
Case Studies |
|
Pheochromocytoma natural history, complications and prognosis On the Web |
|
American Roentgen Ray Society Images of Pheochromocytoma natural history, complications and prognosis |
|
FDA on Pheochromocytoma natural history, complications and prognosis |
|
CDC on Pheochromocytoma natural history, complications and prognosis |
|
Pheochromocytoma natural history, complications and prognosis in the news |
|
Blogs on Pheochromocytoma natural history, complications and prognosis |
|
Risk calculators and risk factors for Pheochromocytoma natural history, complications and prognosis |
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
- Pheochromocytoma is the adrenaline secreting tumor, that 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.
- Majority of catecholamine-secreting tumors are sporadic.
- However, 30% of patients get the tumor as a part of the familial disease. These catecholamine-secreting tumors are more likely to be bilateral.[1]
Symptoms start with tachycardia, hypertension, headache, and sweating. If left untreated, it leads to hyperglycemia and hypertensive emergency that causes heart failure andcerebrovascular strokes. If malignant, It can metastasize to lymph nodes, bones, lungs, and liver.
Complications
- The massive release of catecholamines in pheochromocytoma can cause damage to cardiac myocytes.[2] This damage may be due to either a compromise of the coronary microcirculation or the direct toxic effects of catecholamines on cardiac myocytes.[2]
Other complications may include:
- Hyperglycemia due to opposition of insulin effect by high doses of adrenaline secreted by the tumor.
- Malignant hypertension that may cause cerebrovascular accidents such as:
- Metastasis to:
Prognosis
- The prognosis of pheochromocytoma is good with treatment.
- Approximately 10% recur after being resected.
- Without treatment, patients with metastatic pheochromocytoma have a five-year survival rate of approximately 50%.[3] Survival rate may depend on the primary tumor site and sites of metastases.
- Tumor burden, the location of metastases and rate of progression are the main factors affecting prognosis. Metastasis to the brain and liver has a worse prognosis than other metastases.
Post-surgical prognosis
- Features leading to a good prognosis include small tumor size, short duration of surgery, systolic blood pressure less than 160 mmHg, and low levels of urinary catecholamines.[4]
- Postoperative hypotension can be avoided by adequate fluid replacement.
- Recurrence is more in patients with familial pheochromocytoma and extra-adrenal tumors.[5]
- Familial pheochromocytomas have a high incidence of bilateral disease.
- Partial adrenalectomy is recommended for these patients with bilateral pheochromocytomas to prevent permanent glucocorticoid deficiency.
- Complete bilateral adrenalectomy is recommended for MEN2B patients. There is a high rate of recurrence compared to sporadic cases. Follow-up for a long duration after surgery is required. Most patients should undergo annual biochemical screening.
References
- ↑ 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.
- ↑ 2.0 2.1 Goldman 2011, pp. 327
- ↑ National Cancer Institute. Physician Data Query Database 2015. http://www.cancer.gov/types/pheochromocytoma/hp/pheochromocytoma-treatment-pdq#link/_25_toc
- ↑ 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.
- ↑ 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.