Difference between revisions of "Adrenal myelolipoma"

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{{CMG}}; {{AE}}; {{RAK}}
 
{{CMG}}; {{AE}}; {{RAK}}
  
{{SK}}  
+
{{SK}}Adrenal myelolipoma; Myelolipoma of adrenal gland; Adrenal myelolipomata; Myelolipoma
  
 
==Overview==
 
==Overview==
Adrenal myelolipoma is a  benign adrenal neoplasm predominantly composed of mature adipose tissue and intermixed myeloid tissue. They comprise 6-16% of adrenal incidentaloms and are the second most common cause after adrenal adenomas.  
+
Adrenal myelolipoma is a  benign tumor of the adrenal gland. It mainly consists of mature adipose tissue and intermixed myeloid tissue. Adrenal myelolipoma are the second most common cause of adrenal incidentillomas and make up almost 10% of  it. Rarely, myelolipomas are encountered outside the adrenal glands and are termed as extra adrenal myelolipomoas.
  
 
==Historical Perspective==
 
==Historical Perspective==
Adrenolipoma was first discovered by Gierke in 1905.
+
Adrenal myelolipoma was first discovered by Gierke in 1905.<ref name="pmid21927708">{{cite journal| author=Cha JS, Shin YS, Kim MK, Kim HJ| title=Myelolipomas of both adrenal glands. | journal=Korean J Urol | year= 2011 | volume= 52 | issue= 8 | pages= 582-5 | pmid=21927708 | doi=10.4111/kju.2011.52.8.582 | pmc=3162227 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21927708  }}</ref>
  
 
==Classification==
 
==Classification==
  
Myelolipomas are classified into 4 type based on their clinicopathologic patterns:  
+
Myelolipomas are classified into 4 types based on their [[clinical]] and [[Pathology|pathologic]] patterns:  
* Isolated adrenal myelolipoma
+
*[[Isolated]] adrenal myelolipoma
* Adrenal myelolipoma with acute hemorrhage
+
*[[Adrenal]] myelolipoma with acute [[hemorrhage]]
 
* Extra-adrenal myelolipoma
 
* Extra-adrenal myelolipoma
* Myelolipoma associated with other adrenal diseases.
+
* Myelolipoma associated with other [[Adrenal disease|adrenal diseases]]
{| class="wikitable"
 
|+
 
!Clinicopathologic patterns
 
!Description
 
!Symptoms
 
|-
 
|'''Isolated Adrenal Myelolipoma'''
 
|
 
* Myelolipomas occurring in an otherwise normal adrenal gland
 
* Most common pattern of presentation
 
|
 
* Asymptomatic
 
* Incidentally identified
 
|-
 
|'''Adrenal Myelolipoma with Acute Hemorrhage'''
 
|
 
* Isolated larger lesions (typically over 4 cm in size)
 
|
 
* Retroperitoneal hemorrhage
 
|-
 
|'''Extra-adrenal Myelolipoma'''
 
|
 
* Myelolipomas occurring outside the adrenal gland
 
** Retroperitoneum (presacral or perirenal)
 
|
 
* Patients usually have no endocrine disorder or acute haemorrhage.
 
|-
 
|'''Adrenal Myelolipoma with Associated Adrenal Disease'''
 
|
 
* Associated with endorcrine disorder
 
** Hypercortisolism (Cushing syndrome) and 21-hydroxylase deficiency with adrenal hyperplasia
 
|
 
* Reported to present with an underlying endocrine disorder.
 
|}
 
  
 
==Pathophysiology==
 
==Pathophysiology==
  
*[[Myelolipomas]] are usually less than 4 cm in size occasionally measuring more than 10 cm in size.
+
==== Gross pathology ====
* Extra-adrenal sites for myelolipomas include the [[retroperitoneum]], [[thorax]], and [[pelvis]].
+
 
* Usually [[unilateral]] however they can also involve both adrenals.
+
*Adrenal myelolipomas are usually less than 4 cm in size; however, they could grow more than 10 cm in size sometimes.<ref name="pmid29164520">{{cite journal| author=Decmann Á, Perge P, Tóth M, Igaz P| title=Adrenal myelolipoma: a comprehensive review. | journal=Endocrine | year= 2018 | volume= 59 | issue= 1 | pages= 7-15 | pmid=29164520 | doi=10.1007/s12020-017-1473-4 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29164520  }}</ref>
 +
** Myelolipomas can occur in extra-adrenal sites such as the [[retroperitoneum]], [[thorax]], and [[pelvis]].
 +
**Usually [[unilateral]] however they can also involve both adrenals.
 
* One hypothesis suggests that stimuli, such as necrosis, [[inflammation]], [[infection]], or stress could cause adrenocortical cell metaplasia
 
* One hypothesis suggests that stimuli, such as necrosis, [[inflammation]], [[infection]], or stress could cause adrenocortical cell metaplasia
* If chronically present these stimulants lead to the development of neoplasms.
+
** If chronically present these stimulants lead to the development of neoplasms.
* This hypothesis is supported by the increased incidence of the lesion in the advanced years of life
+
** This hypothesis is supported by the increased incidence of the lesion in the advanced years of life
*On gross pathologic examination, a cut section of a myelolipoma has a variegated appearance consisting of bright yellow areas of fat, dark red areas of hematopoietic myeloid tissue, and areas with intermixed red and yellow components.
+
*On gross pathologic examination, a cut section of a myelolipoma has  
 +
**Variegated appearance consisting of bright yellow areas of fat
 +
**Dark red areas of hematopoietic myeloid tissue
 +
**Areas with intermixed red and yellow components.
 
*On histopathologic examination, myelolipomas are predominantly composed of fatty areas with interspersed hematopoietic tissue components.
 
*On histopathologic examination, myelolipomas are predominantly composed of fatty areas with interspersed hematopoietic tissue components.
*** These fatty elements and hematopoietic areas may be clearly separated, or they are often intermixed.
+
**These fatty elements and hematopoietic areas may be clearly separated, or they are often intermixed.
*** Tissue analysis often reveals a variable amalgamation of myeloid and erythroid cells, megakaryocytes, and occasionally lymphocytes.
+
**Tissue analysis often reveals a variable amalgamation of myeloid and erythroid cells, megakaryocytes, and occasionally lymphocytes.
*** In an isolated adrenal myelolipoma, a peripheral rim of normal adrenal cortical tissue can be commonly identified distinctly from the mass.
+
**In an isolated adrenal myelolipoma, a peripheral rim of normal adrenal cortical tissue can be commonly identified distinctly from the mass.
*** Rarely the myelolipomas can contain osteoid tissue in addition to the myeloid tissue.
+
**Rarely the myelolipomas can contain osteoid tissue in addition to the myeloid tissue.
*** The hemorrhagic areas may be partly replaced by fibrotic tissue or may undergo calcification <br />
+
**The hemorrhagic areas may be partly replaced by fibrotic tissue or may undergo calcification
 +
 
 +
==== Microscopic pathology ====
 +
 
 +
* Histological examination demonstrates:
 +
** Variable amounts of mature [[adipocytes]] (with distended lipid vacuoles) similar to [[bone marrow]]
 +
** Variable amounts of hematopoietic cells (including cells from [[myeloid]], [[erythroid]] and [[Megakaryocytes|megakaryocytic]] cells lines)
 +
*
  
<br />
 
 
==Causes==
 
==Causes==
  
 
The exact causes of Adrenal myelolipoma remain unknown. However, Adrenal myelolipoma is often associated with conditions that can be considered as adrenal stimulants such as:
 
The exact causes of Adrenal myelolipoma remain unknown. However, Adrenal myelolipoma is often associated with conditions that can be considered as adrenal stimulants such as:
  
* Cushing's disease
+
*[[Cushing's disease]]
* Obesity
+
*[[Obesity CT|Obesity]]
* Hyperlipidemia
+
*[[Hyperlipoproteinemia|Hyperlipidemia]]
* Hypertension
+
*[[Hypertension]]
* Diabetes
+
*[[Diabetes Mellitus|Diabetes]]
 
* Stressful lifestyle
 
* Stressful lifestyle
 
* Unbalanced diet
 
* Unbalanced diet
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==Differentiating adrenal myelolipoma from Other Diseases==
 
==Differentiating adrenal myelolipoma from Other Diseases==
  
 +
Adrenal myelolipoma should be differentiated from other adrenal masses such as:
 +
 +
* [[Retroperitoneal liposarcoma]]
 +
* Fat containing [[adrenocortical carcinoma]]
 +
* [[Teratoma|Adrenal teratoma]]
 +
* Renal [[angiomyolipoma]] (AML)
  
 
==Epidemiology and Demographics==
 
==Epidemiology and Demographics==
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* Adrenolipoma affects men and women equally.
 
* Adrenolipoma affects men and women equally.
 
* Adrenolipomas are usually recognized in adults, either incidentally at ultrasound or computed topography or may present with vague abdominal symptoms if complicated by hemorrhage.
 
* Adrenolipomas are usually recognized in adults, either incidentally at ultrasound or computed topography or may present with vague abdominal symptoms if complicated by hemorrhage.
 
+
*Adrenal myelolipomas are the second most common adrenal incidentalomas comprising of 6-16% of adrenal incidentalomas.
 
+
*They have an approximate autopsy prevalence of 0.08% to 0.2%.
 +
*They are usually recognized in adulthood with a medican age of diagnosis around 51 years. There is no gender predilection.
 +
*There may be a slight right sided predilection as reported by some studies.<ref name="pmid28143811">{{cite journal| author=Lam AK| title=Lipomatous tumours in adrenal gland: WHO updates and clinical implications. | journal=Endocr Relat Cancer | year= 2017 | volume= 24 | issue= 3 | pages= R65-R79 | pmid=28143811 | doi=10.1530/ERC-16-0564 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28143811  }}</ref>
  
 
==Risk Factors==
 
==Risk Factors==
 
There are no established risk factors for Adrenal myelolipoma.
 
There are no established risk factors for Adrenal myelolipoma.
 
 
 
==Screening==
 
==Screening==
 
There is insufficient evidence to recommend routine screening for Adrenal myelolipoma.
 
There is insufficient evidence to recommend routine screening for Adrenal myelolipoma.
  
 
==Natural History, Complications, and Prognosis==
 
==Natural History, Complications, and Prognosis==
 +
 +
*Functional adrenolipomas are associated with endocrine disorders such as:<ref name="radiopaedia">http://radiopaedia.org/articles/adrenal-myelolipoma</ref>
 +
**[[Cushing syndrome]]
 +
**[[Congenital adrenal hyperplasia]]
 +
**[[Conn syndrome]]
 +
 +
*Large adrenolipomas  can cause [[retroperitoneal haemorrhage]] or [[infarction]]
  
 
==Diagnosis==
 
==Diagnosis==
 
===Diagnostic Study of Choice===
 
===Diagnostic Study of Choice===
The diagnosis of Adrenal myelolipoma is made when  
+
The diagnosis of Adrenal myelolipoma is made when a biopsy shows 
  
 
===History and Symptoms===
 
===History and Symptoms===
The majority of patients with Adrenal myelolipoma are asymptomatic.
+
The most common presenting [[Symptom|symptoms]] for adrenal myelolipomas are as follows:<ref name="pmid28613782">{{cite journal| author=| title=StatPearls | journal= | year= 2019 | volume=  | issue=  | pages=  | pmid=28613782 | doi= | pmc= | url= }}</ref>
 +
 
 +
*[[Abdominal pain]]
 +
* Hypochondrial pain
 +
*[[Flank pain]]
 +
*[[Abdominal mass]]
 +
 
 +
Rarely, dyspnea, back pain, fever, weight loss, and virilization can be the presenting symptoms of adrenal myelolipomas.  
  
 
===Physical Examination===
 
===Physical Examination===
  
Common physical examination findings of Adrenal myelolipoma include  
+
Common physical examination findings of Adrenal myelolipoma include:
  
 +
===Laboratory Findings===
  
===Laboratory Findings===
+
There are no associated laboratory findings among the majority of patients with adrenolipoma, some laboratory findings may include:
  
 +
* Elevated [[cortisol]] level (suggestive of [[Cushing syndrome]])
 +
* Elevated aldosteron level (suggestive of [[Conn's syndrome]])
  
 
===Electrocardiogram===
 
===Electrocardiogram===
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===X-ray===
 
===X-ray===
 
There are no x-ray findings associated with Adrenal myelolipoma.
 
There are no x-ray findings associated with Adrenal myelolipoma.
 +
===Echocardiography or Ultrasound===
 +
Adrenal myelolipoma has the typical appearance of a hyperechoic mass with intermixed hypoechoic regions.<ref name="pmid28866749">{{cite journal| author=Campbell MJ, Obasi M, Wu B, Corwin MT, Fananapazir G| title=The radiographically diagnosed adrenal myelolipoma: what do we really know? | journal=Endocrine | year= 2017 | volume= 58 | issue= 2 | pages= 289-294 | pmid=28866749 | doi=10.1007/s12020-017-1410-6 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28866749  }}</ref>
  
 
+
* Echogenicity is mainly determined by the variable proportions of the constituent elements in the lesion. The areas of intermixed fatty and myeloid tissue are the most echogenic, whereas regions of pure fat may appear hypoechoic.
===Echocardiography or Ultrasound===
+
* Because of inhomogeneous architecture, myelolipomas often have heterogeneous echogenicity.
There are no echocardiography/ultrasound  findings associated with Adrenal myelolipoma.
+
* Calcification foci appear hyperechoic with acoustic shadowing.
 +
* Myelolipoma has vague and no appreciable margins due having almost the same echogenicity as surrounding retroperitoneal fat.
 +
* Hemorrhage alters the sonographic picture, with hemorrhagic areas appearing hypoechoic compared with fat
  
 
===CT scan===
 
===CT scan===
Line 138: Line 142:
 
===Other Imaging Findings===
 
===Other Imaging Findings===
 
There are no other imaging findings associated with Adrenal myelolipoma.
 
There are no other imaging findings associated with Adrenal myelolipoma.
 
 
 
===Other Diagnostic Studies===
 
===Other Diagnostic Studies===
 
There are no other diagnostic studies associated with Adrenal myelolipoma.
 
There are no other diagnostic studies associated with Adrenal myelolipoma.
Line 146: Line 148:
 
===Medical Therapy===
 
===Medical Therapy===
 
There is no treatment for Adrenal myelolipoma; the mainstay of therapy is supportive care.
 
There is no treatment for Adrenal myelolipoma; the mainstay of therapy is supportive care.
 
 
 
===Surgery===
 
===Surgery===
  

Latest revision as of 20:30, 30 September 2019

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

Synonyms and Keywords: Adrenal myelolipoma; Myelolipoma of adrenal gland; Adrenal myelolipomata; Myelolipoma

Overview

Adrenal myelolipoma is a  benign tumor of the adrenal gland. It mainly consists of mature adipose tissue and intermixed myeloid tissue. Adrenal myelolipoma are the second most common cause of adrenal incidentillomas and make up almost 10% of it. Rarely, myelolipomas are encountered outside the adrenal glands and are termed as extra adrenal myelolipomoas.

Historical Perspective

Adrenal myelolipoma was first discovered by Gierke in 1905.[1]

Classification

Myelolipomas are classified into 4 types based on their clinical and pathologic patterns:

Pathophysiology

Gross pathology

  • Adrenal myelolipomas are usually less than 4 cm in size; however, they could grow more than 10 cm in size sometimes.[2]
  • One hypothesis suggests that stimuli, such as necrosis, inflammation, infection, or stress could cause adrenocortical cell metaplasia
    • If chronically present these stimulants lead to the development of neoplasms.
    • This hypothesis is supported by the increased incidence of the lesion in the advanced years of life
  • On gross pathologic examination, a cut section of a myelolipoma has
    • Variegated appearance consisting of bright yellow areas of fat
    • Dark red areas of hematopoietic myeloid tissue
    • Areas with intermixed red and yellow components.
  • On histopathologic examination, myelolipomas are predominantly composed of fatty areas with interspersed hematopoietic tissue components.
    • These fatty elements and hematopoietic areas may be clearly separated, or they are often intermixed.
    • Tissue analysis often reveals a variable amalgamation of myeloid and erythroid cells, megakaryocytes, and occasionally lymphocytes.
    • In an isolated adrenal myelolipoma, a peripheral rim of normal adrenal cortical tissue can be commonly identified distinctly from the mass.
    • Rarely the myelolipomas can contain osteoid tissue in addition to the myeloid tissue.
    • The hemorrhagic areas may be partly replaced by fibrotic tissue or may undergo calcification

Microscopic pathology

Causes

The exact causes of Adrenal myelolipoma remain unknown. However, Adrenal myelolipoma is often associated with conditions that can be considered as adrenal stimulants such as:

Differentiating adrenal myelolipoma from Other Diseases

Adrenal myelolipoma should be differentiated from other adrenal masses such as:

Epidemiology and Demographics

  • The incidence of adrenolipoma is approximately 0.8-4 per 100,000 individuals worldwide.
    • The increased prevalence of adrenal myelolipomas is due to increased detection because of wider accessibility of computed tomography (CT) and magnetic resonance imaging (MRI) scans.
  • Adrenolipoma affects men and women equally.
  • Adrenolipomas are usually recognized in adults, either incidentally at ultrasound or computed topography or may present with vague abdominal symptoms if complicated by hemorrhage.
  • Adrenal myelolipomas are the second most common adrenal incidentalomas comprising of 6-16% of adrenal incidentalomas.
  • They have an approximate autopsy prevalence of 0.08% to 0.2%.
  • They are usually recognized in adulthood with a medican age of diagnosis around 51 years. There is no gender predilection.
  • There may be a slight right sided predilection as reported by some studies.[3]

Risk Factors

There are no established risk factors for Adrenal myelolipoma.

Screening

There is insufficient evidence to recommend routine screening for Adrenal myelolipoma.

Natural History, Complications, and Prognosis

Diagnosis

Diagnostic Study of Choice

The diagnosis of Adrenal myelolipoma is made when a biopsy shows

History and Symptoms

The most common presenting symptoms for adrenal myelolipomas are as follows:[5]

Rarely, dyspnea, back pain, fever, weight loss, and virilization can be the presenting symptoms of adrenal myelolipomas.

Physical Examination

Common physical examination findings of Adrenal myelolipoma include:

Laboratory Findings

There are no associated laboratory findings among the majority of patients with adrenolipoma, some laboratory findings may include:

Electrocardiogram

There are no ECG findings associated with Adrenal myelolipoma.

X-ray

There are no x-ray findings associated with Adrenal myelolipoma.

Echocardiography or Ultrasound

Adrenal myelolipoma has the typical appearance of a hyperechoic mass with intermixed hypoechoic regions.[6]

  • Echogenicity is mainly determined by the variable proportions of the constituent elements in the lesion. The areas of intermixed fatty and myeloid tissue are the most echogenic, whereas regions of pure fat may appear hypoechoic.
  • Because of inhomogeneous architecture, myelolipomas often have heterogeneous echogenicity.
  • Calcification foci appear hyperechoic with acoustic shadowing.
  • Myelolipoma has vague and no appreciable margins due having almost the same echogenicity as surrounding retroperitoneal fat.
  • Hemorrhage alters the sonographic picture, with hemorrhagic areas appearing hypoechoic compared with fat

CT scan

Abdominal CT scan may be helpful in the diagnosis of Adrenal myelolipoma. Findings on CT scan suggestive of/diagnostic of Adrenal myelolipoma include

MRI

There are no MRI findings associated with Adrenal myelolipoma.

Other Imaging Findings

There are no other imaging findings associated with Adrenal myelolipoma.

Other Diagnostic Studies

There are no other diagnostic studies associated with Adrenal myelolipoma.

Treatment

Medical Therapy

There is no treatment for Adrenal myelolipoma; the mainstay of therapy is supportive care.

Surgery

Surgery is not the first-line treatment option for patients with Adrenal myelolipoma. Surgery is usually reserved for patients with either

  • Surgery is the mainstay of treatment of large adrenolipomas. Management of adrenal myelolipoma is decided based upon the size of lesion and presence of symptoms.
  • Small lesions measuring less than 5 cm, and those who are asymptomatic are usually monitored via imaging over a period of one to two years.
  • Symptomatic tumors or myelolipomas larger than 7 cm should undergo elective surgical excision.
    • The approach is based on the reported incidence of life-threatening emergencies caused by spontaneous rupture and hemorrhage within large lesions.
    • Conventional or endoscopic access may be chosen according to the size of the tumor.
    • Mini-invasive and endoscopic techniques are best utilized for smaller-sized lesions, depending on the expertise of the operator.
    • Conventional methods including transabdominal, lumbar, subcostal or posterior access laparotomy operations have all been described in the literature.
    • An extraperitoneal approach is preferable as it leads to quicker recovery of the patient and lesser postoperative complications.
    • The midline approach is indicated for masses larger than 10 cm or in cases where there are adhesions and infiltration of the surrounding structures.
    • Follow up is mandatory regardless of which surgical method has been employed.


Primary Prevention

There are no established measures for the primary prevention of Adrenal myelolipoma.

Secondary Prevention

There are no established measures for the secondary prevention of Adrenal myelolipoma.

References

  1. Cha JS, Shin YS, Kim MK, Kim HJ (2011). "Myelolipomas of both adrenal glands". Korean J Urol. 52 (8): 582–5. doi:10.4111/kju.2011.52.8.582. PMC 3162227. PMID 21927708.
  2. Decmann Á, Perge P, Tóth M, Igaz P (2018). "Adrenal myelolipoma: a comprehensive review". Endocrine. 59 (1): 7–15. doi:10.1007/s12020-017-1473-4. PMID 29164520.
  3. Lam AK (2017). "Lipomatous tumours in adrenal gland: WHO updates and clinical implications". Endocr Relat Cancer. 24 (3): R65–R79. doi:10.1530/ERC-16-0564. PMID 28143811.
  4. http://radiopaedia.org/articles/adrenal-myelolipoma
  5. "StatPearls". 2019. PMID 28613782.
  6. Campbell MJ, Obasi M, Wu B, Corwin MT, Fananapazir G (2017). "The radiographically diagnosed adrenal myelolipoma: what do we really know?". Endocrine. 58 (2): 289–294. doi:10.1007/s12020-017-1410-6. PMID 28866749.

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