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{{Incidentaloma}}
{{Incidentaloma}}
{{CMG}}; {{AE}}  
{{CMG}}; {{AE}} {{MAD}}


==Overview==
==Overview==
The mainstay of treatment for [disease name] is [therapy].
The mainstay of treatment for [[Adrenal gland|adrenal]] incidentaloma is surgery but pre-operative medical management is needed for functional masses. Peri-operative medical management of patients with sub-clinical [[Cushing's syndrome]] includes [[glucocorticoid]] therapy during [[surgery]]. Such patients can safely undergo surgical resection of their [[tumor]] and have their [[cortisol]] levels measured post-operatively. Preoperative medical management of patients with [[pheochromocytoma]] includes preoperative treatment to control [[hypertension]] during surgery and [[hypotension]] after it. Three medical regimens for preoperative management of [[pheochromocytoma]]: Combined [[Alpha blocker|alpha]] and [[beta-adrenergic blockers]], [[Calcium channel blocker|calcium channel blockers]], and [[Metyrosine]]. Pre-operative medical management of patients with [[Hyperaldosteronism|aldosteronoma]] include medical therapy with [[mineralocorticoid]] [[Receptor antagonist|receptor antagonists]] should be reserved for those who are unable or unwilling to undergo surgery.


==Medical Therapy==
==Medical Therapy==
The mainstay of treatment for adrenal incidentaloma is surgery but preoperative medical management is needed for functional [[Mass|masses]].


=== Perioperative management of patients with SCS ===
==== Peri-operative medical management of patients with subclinical [[Cushing's syndrome]]:  ====
* Patients with Cushing’s syndrome or SCS have adequate circulating glucocorticoids and therefore do not require glucocorticoid therapy during surgery.  
*There are two approaches to deal with cases of [[Cushing's syndrome|Cushing's syndrome:]]<ref name="pmid203752102">{{cite journal| author=Chiodini I, Morelli V, Salcuni AS, Eller-Vainicher C, Torlontano M, Coletti F et al.| title=Beneficial metabolic effects of prompt surgical treatment in patients with an adrenal incidentaloma causing biochemical hypercortisolism. | journal=J Clin Endocrinol Metab | year= 2010 | volume= 95 | issue= 6 | pages= 2736-45 | pmid=20375210 | doi=10.1210/jc.2009-2387 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20375210  }}</ref><ref name="pmid20823463">{{cite journal| author=Nieman LK| title=Approach to the patient with an adrenal incidentaloma. | journal=J Clin Endocrinol Metab | year= 2010 | volume= 95 | issue= 9 | pages= 4106-13 | pmid=20823463 | doi=10.1210/jc.2010-0457 | pmc=2936073 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20823463  }}</ref><ref name="pmid193269852">{{cite journal| author=Marko NF, Gonugunta VA, Hamrahian AH, Usmani A, Mayberg MR, Weil RJ| title=Use of morning serum cortisol level after transsphenoidal resection of pituitary adenoma to predict the need for long-term glucocorticoid supplementation. | journal=J Neurosurg | year= 2009 | volume= 111 | issue= 3 | pages= 540-4 | pmid=19326985 | doi=10.3171/2008.12.JNS081265 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19326985  }}</ref>
* Such patients can safely undergo surgical resection of their tumor, have their cortisol levels measured in the morning of postoperative d 1
** Patients do not require [[glucocorticoid]] therapy during surgery. Such patients can safely undergo surgical resection of their [[tumor]] and have their [[cortisol]] levels measured post-operatively. 
* be started on hydrocortisone 30 mg in the morning and 10 mg in the early afternoon until the result of the cortisol level becomes available (73).  
** This would provide an early evaluation of surgical success. [[Hydrocortisone]] may be given to the patient 30 mg in the morning and 10 mg in the afternoon until the result of the [[cortisol]] level becomes available.<ref name="pmid19326985">{{cite journal| author=Marko NF, Gonugunta VA, Hamrahian AH, Usmani A, Mayberg MR, Weil RJ| title=Use of morning serum cortisol level after transsphenoidal resection of pituitary adenoma to predict the need for long-term glucocorticoid supplementation. | journal=J Neurosurg | year= 2009 | volume= 111 | issue= 3 | pages= 540-4 | pmid=19326985 | doi=10.3171/2008.12.JNS081265 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19326985  }}</ref> 
* This would provide an early and accurate evaluation of surgical success in patients with underlying Cushing’s syndrome or SCS.
** Another approach would be to cover all patients with [[glucocorticoids]] peri-operatively.<ref name="pmid20375210">{{cite journal| author=Chiodini I, Morelli V, Salcuni AS, Eller-Vainicher C, Torlontano M, Coletti F et al.| title=Beneficial metabolic effects of prompt surgical treatment in patients with an adrenal incidentaloma causing biochemical hypercortisolism. | journal=J Clin Endocrinol Metab | year= 2010 | volume= 95 | issue= 6 | pages= 2736-45 | pmid=20375210 | doi=10.1210/jc.2009-2387 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20375210  }}</ref>
* Another approach would be to cover all patients with glucocorticoids perioperatively and evaluate their HPA axis at a later date (71, 74).
'''Unilateral adrenal masses''' 


All patients with documented pheochromocytoma and adrenocortical cancer should undergo prompt surgical intervention because untreated pheochromocytoma may result in significant cardiovascular complications.Alpha blockade should be given before patients undergo adrenalectomy.
==== Pre-operative medical management of patients with pheochromocytoma ====
* All patients undergoing surgery need preoperative treatment to control [[hypertension]] during surgery and [[hypotension]] after it.<ref name="pmid20823463" /><ref name="pmid27390021">{{cite journal| author=Fassnacht M, Arlt W, Bancos I, Dralle H, Newell-Price J, Sahdev A et al.| title=Management of adrenal incidentalomas: European Society of Endocrinology Clinical Practice Guideline in collaboration with the European Network for the Study of Adrenal Tumors. | journal=Eur J Endocrinol | year= 2016 | volume= 175 | issue= 2 | pages= G1-G34 | pmid=27390021 | doi=10.1530/EJE-16-0467 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27390021  }}</ref>
* According to Endocrine Society’s 2014 clinical practice guidelines, there are three medical regimens for preoperative management of [[pheochromocytoma]]:<sup>[[Pheochromocytoma medical therapy#cite note-pmid248931352-1|[1]]][[Pheochromocytoma medical therapy#cite note-pmid14766711-2|[2]]]</sup>
** Combined [[Alpha blocker|alpha]] and [[beta-adrenergic blockers]]
** [[Calcium channel blocker|Calcium channel blockers]]
** [[Metyrosine]]


Patients with adrenocortical cancer or lesions suspicious for adrenocortical cancer should also undergo prompt adrenalectomy as their disease may progress rapidly.  
==== [[Alpha blocker|Alpha adrenoceptor blocker]] ====
* It is used to counteract [[hypertension]] and the [[Beta blockers|beta-1 adrenoceptor antagonist]] [[atenolol]] to reduce [[cardiac output]]. They can block the sudden release of [[adrenaline]] during surgical stress and prevent [[hypertensive crisis]]. The patient is ready for surgery after 10 to 14 days of initiation of [[Alpha-adrenergic blocking agent|alpha-adrenergic blockade]]. Patients should take high [[sodium]] diet to prevent [[orthostatic hypotension]] due to [[Alpha blocker|alpha blockers]]. After adequate [[Alpha-adrenergic blocker|alpha-adrenergic blockade]] has been achieved, a [[beta-adrenergic blocker]] is initiated 3 days before surgery.<ref name="pmid18617683">{{cite journal| author=Adler JT, Meyer-Rochow GY, Chen H, Benn DE, Robinson BG, Sippel RS et al.| title=Pheochromocytoma: current approaches and future directions. | journal=Oncologist | year= 2008 | volume= 13 | issue= 7 | pages= 779-93 | pmid=18617683 | doi=10.1634/theoncologist.2008-0043 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18617683  }}</ref>
'''[[Beta blockers|Beta-adrenergic blocker]]'''
* It should never be started first because unopposed [[Alpha-adrenergic agonist|alpha-adrenergic]] receptor stimulation can lead to the brisk increase in [[blood pressure]]. It should be used with caution due to the risk of [[heart failure]], [[pulmonary edema]], and [[asthma]].<ref name="pmid17989126">{{cite journal| author=Pacak K| title=Preoperative management of the pheochromocytoma patient. | journal=J Clin Endocrinol Metab | year= 2007 | volume= 92 | issue= 11 | pages= 4069-79 | pmid=17989126 | doi=10.1210/jc.2007-1720 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17989126  }}</ref>


Patients with aldosterone-producing adenomas should be offered surgery to cure aldosterone excess (
==== [[Calcium channel blocker]] ====
* It is used to control [[blood pressure]] pre-operatively and an [[intravenous injection]] is given intra-operatively.
* Its main use is controlling [[blood pressure]] in case of failed [[Alpha blocker|alpha]] and [[beta blockers]] regimen or unaccepted side effects in that regimen.<ref name="pmid10363888">{{cite journal| author=Goldstein RE, O'Neill JA, Holcomb GW, Morgan WM, Neblett WW, Oates JA et al.| title=Clinical experience over 48 years with pheochromocytoma. | journal=Ann Surg | year= 1999 | volume= 229 | issue= 6 | pages= 755-64; discussion 764-6 | pmid=10363888 | doi= | pmc=1420821 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10363888  }}</ref>


Some patients with documented subclinical Cushing's syndrome should be selected for surgery based upon the clinical parameters discussed above [47].  
==== [[Metyrosine]] ====
* It is the last medical line of treatment. It inhibits [[catecholamine]] synthesis.
* It is used in case of failure of other medical lines of treatment or in patients who cannot tolerate them.
* Clinicians use combined treatment in difficult cases and if [[radiofrequency ablation]] for [[metastatic]] foci will be used.
* Side effects of [[Metyrosine]] include:<ref name="pmid15761546">{{cite journal| author=Bravo EL| title=Pheochromocytoma: current perspectives in the pathogenesis, diagnosis, and management. | journal=Arq Bras Endocrinol Metabol | year= 2004 | volume= 48 | issue= 5 | pages= 746-50 | pmid=15761546 | doi=/S0004-27302004000500021 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15761546  }}</ref>
**[[Crystalluria|Crystalluria]]
**[[Extrapyramidal symptom|Extrapyramidal]] manifestations
**High cost.
=====Pre-operative medical management of patients with primary hyperaldosteronism=====


Adrenal masses with either suspicious imaging phenotype or size larger than 4 cm should be considered for resection because a substantial fraction will be adrenocortical carcinomas [2,14]. The clinical scenario and patient age frequently guide the management decisions in patients who have adrenal incidentalomas that fall on either side of the 4 cm diameter cutoff. As an example, most clinicians would advise resecting a uniform hypodense (9 Hounsfield unit [HU]) 3.7 cm adrenal incidentaloma in a 23-year-old woman, whereas most clinicians would choose serial imaging follow-up in an 83-year-old woman with a uniform hypodense (9 HU) 4.7 cm adrenal incidentaloma. Surgical resection should be considered in this example of a young woman because adrenal cortical nodularity is, in part, a function of aging and such a finding in a 23-year-old is not normal. In addition, the alternative would be long-term imaging follow-up, which is associated with clinically significant expense, worry, and risks. Before surgery, all patients should undergo appropriate testing for functional tumors.  
*Medical therapy with [[mineralocorticoid receptor]] [[antagonists]] should be reserved for those who are unable or unwilling to undergo surgery.<ref name="pmid26934393">{{cite journal| author=Funder JW, Carey RM, Mantero F, Murad MH, Reincke M, Shibata H et al.| title=The Management of Primary Aldosteronism: Case Detection, Diagnosis, and Treatment: An Endocrine Society Clinical Practice Guideline. | journal=J Clin Endocrinol Metab | year= 2016 | volume= 101 | issue= 5 | pages= 1889-916 | pmid=26934393 | doi=10.1210/jc.2015-4061 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26934393  }}</ref><ref name="pmid17492946">{{cite journal| author=Young WF| title=Primary aldosteronism: renaissance of a syndrome. | journal=Clin Endocrinol (Oxf) | year= 2007 | volume= 66 | issue= 5 | pages= 607-18 | pmid=17492946 | doi=10.1111/j.1365-2265.2007.02775.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17492946  }}</ref><ref name="pmid20823463" />
 
**[[Adrenalectomy]] in patients with a documented unilateral source of [[Primary hyperaldosteronism|primary aldosteronism]] is more cost effective compared with lifelong medical therapy.
Adrenal myelolipoma is a benign tumor composed of mature fat and interspersed hematopoietic elements that resemble bone marrow. On computed imaging, the presence of large amounts of macroscopic fat in an adrenal mass is diagnostic of a myelolipoma (image 5) [52]. Although adrenal myelolipomas may grow over time, they can usually be followed without surgical excision. However, when larger than 6 cm in diameter or when causing local mass-effect symptoms, surgical removal should be considered.
**[[Laparoscopic surgery|Laparoscopic]] [[adrenalectomy]] compared to an open procedure is associated with a shorter hospital stay, fewer complications, and faster recovery.
 
**Resection of the [[adrenal tumor]] will result in resolution of [[hypokalemia]] and improvement in [[hypertension]] in almost all patients.
'''Bilateral adrenal masses''' 
 
The management of bilateral adrenal masses is different from that for unilateral masses.
 
In cases of subclinical bilateral macronodular adrenal hyperplasia (BMAH), size is not an indication for surgery, as some can be as large as 5 to 10 cm with insufficient hormone production to necessitate surgery. Patients with bilateral adrenal masses should be investigated for congenital adrenal hyperplasia [53].
 
'''Adrenalectomy''' 
 
Adrenalectomy for patients with aldosteronomas, pheochromocytoma, cortisol-secreting tumors, and adrenal incidentalomas is safe and effective [54]. An adrenalectomy may be done laparoscopically, endoscopically via the posterior approach, or as an open procedure. Laparoscopic adrenalectomy, compared with open adrenalectomy, is associated with less pain, shorter hospitalization time, less blood loss, and faster recovery [55]. The laparoscopic approach is used for most adrenal masses.
 
In patients with known or suspected adrenal carcinoma, the laparoscopic approach should only be considered if the adrenal mass is <10 cm and does not appear to be locally invasive [56,57].
 
An open adrenalectomy is recommended for all large (>10 cm) adrenal masses, including those benign imaging features, as the adrenal mass may be diagnosed as malignant on a definitive histologic review [56,58-61].


====== Post-operative management of patients with primary hyperaldosteronism======
*Postoperative management in patients with primary [[hyperaldosteronism]] includes:<ref name="pmid16932426">{{cite journal| author=Mattsson C, Young WF| title=Primary aldosteronism: diagnostic and treatment strategies. | journal=Nat Clin Pract Nephrol | year= 2006 | volume= 2 | issue= 4 | pages= 198-208; quiz, 1 p following 230 | pmid=16932426 | doi=10.1038/ncpneph0151 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16932426  }}</ref>
**[[Potassium]] supplementation and [[mineralocorticoid]] receptor antagonists should be stopped post-operatively with close monitoring of serum [[potassium]].
**A temporary state of [[hypoaldosteronism]] may also develop in some patients with [[Primary hyperaldosteronism|primary aldosteronism]] post-operatively and can be managed by increasing salt intake.


==References==
==References==

Latest revision as of 16:09, 9 November 2017

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

Overview

The mainstay of treatment for adrenal incidentaloma is surgery but pre-operative medical management is needed for functional masses. Peri-operative medical management of patients with sub-clinical Cushing's syndrome includes glucocorticoid therapy during surgery. Such patients can safely undergo surgical resection of their tumor and have their cortisol levels measured post-operatively. Preoperative medical management of patients with pheochromocytoma includes preoperative treatment to control hypertension during surgery and hypotension after it. Three medical regimens for preoperative management of pheochromocytoma: Combined alpha and beta-adrenergic blockers, calcium channel blockers, and Metyrosine. Pre-operative medical management of patients with aldosteronoma include medical therapy with mineralocorticoid receptor antagonists should be reserved for those who are unable or unwilling to undergo surgery.

Medical Therapy

The mainstay of treatment for adrenal incidentaloma is surgery but preoperative medical management is needed for functional masses.

Peri-operative medical management of patients with subclinical Cushing's syndrome:

  • There are two approaches to deal with cases of Cushing's syndrome:[1][2][3]
    • Patients do not require glucocorticoid therapy during surgery. Such patients can safely undergo surgical resection of their tumor and have their cortisol levels measured post-operatively.
    • This would provide an early evaluation of surgical success. Hydrocortisone may be given to the patient 30 mg in the morning and 10 mg in the afternoon until the result of the cortisol level becomes available.[4]
    • Another approach would be to cover all patients with glucocorticoids peri-operatively.[5]

Pre-operative medical management of patients with pheochromocytoma

Alpha adrenoceptor blocker

Beta-adrenergic blocker

Calcium channel blocker

Metyrosine

Pre-operative medical management of patients with primary hyperaldosteronism
Post-operative management of patients with primary hyperaldosteronism

References

  1. ↑ Chiodini I, Morelli V, Salcuni AS, Eller-Vainicher C, Torlontano M, Coletti F; et al. (2010). "Beneficial metabolic effects of prompt surgical treatment in patients with an adrenal incidentaloma causing biochemical hypercortisolism". J Clin Endocrinol Metab. 95 (6): 2736–45. doi:10.1210/jc.2009-2387. PMID 20375210.
  2. ↑ 2.0 2.1 2.2 Nieman LK (2010). "Approach to the patient with an adrenal incidentaloma". J Clin Endocrinol Metab. 95 (9): 4106–13. doi:10.1210/jc.2010-0457. PMC 2936073. PMID 20823463.
  3. ↑ Marko NF, Gonugunta VA, Hamrahian AH, Usmani A, Mayberg MR, Weil RJ (2009). "Use of morning serum cortisol level after transsphenoidal resection of pituitary adenoma to predict the need for long-term glucocorticoid supplementation". J Neurosurg. 111 (3): 540–4. doi:10.3171/2008.12.JNS081265. PMID 19326985.
  4. ↑ Marko NF, Gonugunta VA, Hamrahian AH, Usmani A, Mayberg MR, Weil RJ (2009). "Use of morning serum cortisol level after transsphenoidal resection of pituitary adenoma to predict the need for long-term glucocorticoid supplementation". J Neurosurg. 111 (3): 540–4. doi:10.3171/2008.12.JNS081265. PMID 19326985.
  5. ↑ Chiodini I, Morelli V, Salcuni AS, Eller-Vainicher C, Torlontano M, Coletti F; et al. (2010). "Beneficial metabolic effects of prompt surgical treatment in patients with an adrenal incidentaloma causing biochemical hypercortisolism". J Clin Endocrinol Metab. 95 (6): 2736–45. doi:10.1210/jc.2009-2387. PMID 20375210.
  6. ↑ Fassnacht M, Arlt W, Bancos I, Dralle H, Newell-Price J, Sahdev A; et al. (2016). "Management of adrenal incidentalomas: European Society of Endocrinology Clinical Practice Guideline in collaboration with the European Network for the Study of Adrenal Tumors". Eur J Endocrinol. 175 (2): G1–G34. doi:10.1530/EJE-16-0467. PMID 27390021.
  7. ↑ Adler JT, Meyer-Rochow GY, Chen H, Benn DE, Robinson BG, Sippel RS; et al. (2008). "Pheochromocytoma: current approaches and future directions". Oncologist. 13 (7): 779–93. doi:10.1634/theoncologist.2008-0043. PMID 18617683.
  8. ↑ Pacak K (2007). "Preoperative management of the pheochromocytoma patient". J Clin Endocrinol Metab. 92 (11): 4069–79. doi:10.1210/jc.2007-1720. PMID 17989126.
  9. ↑ Goldstein RE, O'Neill JA, Holcomb GW, Morgan WM, Neblett WW, Oates JA; et al. (1999). "Clinical experience over 48 years with pheochromocytoma". Ann Surg. 229 (6): 755–64, discussion 764-6. PMC 1420821. PMID 10363888.
  10. ↑ Bravo EL (2004). "Pheochromocytoma: current perspectives in the pathogenesis, diagnosis, and management". Arq Bras Endocrinol Metabol. 48 (5): 746–50. doi:/S0004-27302004000500021 Check |doi= value (help). PMID 15761546.
  11. ↑ Funder JW, Carey RM, Mantero F, Murad MH, Reincke M, Shibata H; et al. (2016). "The Management of Primary Aldosteronism: Case Detection, Diagnosis, and Treatment: An Endocrine Society Clinical Practice Guideline". J Clin Endocrinol Metab. 101 (5): 1889–916. doi:10.1210/jc.2015-4061. PMID 26934393.
  12. ↑ Young WF (2007). "Primary aldosteronism: renaissance of a syndrome". Clin Endocrinol (Oxf). 66 (5): 607–18. doi:10.1111/j.1365-2265.2007.02775.x. PMID 17492946.
  13. ↑ Mattsson C, Young WF (2006). "Primary aldosteronism: diagnostic and treatment strategies". Nat Clin Pract Nephrol. 2 (4): 198–208, quiz, 1 p following 230. doi:10.1038/ncpneph0151. PMID 16932426.

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