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adrenal.pptx
1. Adrenal Anatomy , Tumours&
Workup of Patients
Presentation- Vikash Singh
Moderator: Dr Palaniappan
2. Anatomy
The adrenal glands are paired retroperitoneal organs composed of
cortex and medulla and located above the kidney.
Gross examination reveals
Cortex- spiculated,mustard yellow coloured
Medulla- central , brown colour
Weight ~ 4-5 gram
Size
Length 4-6 cm
Width 2-3 cm
Right adrenal is triangular in shape and left adrenal crescent in shape.
3.
4.
5. Relations
The right and left adrenal glands are located within Gerota fascia at the levels
of the 11th and 12th ribs.
Medial aspect of the right adrenal gland is often retrocaval.
Right Adrenal Left Adrenal
Anteriorly Liver Stomach,Pancreas[body]
,splenic vessels
Posteriorly Diaphragm Diaphragm
Superiorly Diaphragm Spleen
Inferolaterally Rt Kidney Lt Kidney
Medially IVC Aorta
9. Arterial Supply
• 3 arterial sources of flow:
• Branches from Inferior Phrenic Artery Superior Adrenal A.
• Direct visceral branches from Aorta Middle Adrenal A.
• Branches from I/L Renal Artery Inferior Adrenal A.
The main adrenal arteries branch to form a subcapsular plexus
• From subcapsular plexus
• Some branches continue directly to medulla
• Others form sinusoids to cortex
10. Venous Drainage:
• Medullary veins coalesce to form adrenal vein
Right adrenal vein
• Short
• Drains directly into post IVC
Left adrenal vein
• Long as compared to right adrenal vein
• Joined by Inferior Phrenic vein prior to draining into Left Renal Vein
• The overlapping of both arterial and venous anatomy makes partial
adrenalectomy possible with little risk of subsequent adrenal infarction
13. Tumors of Adrenal Cortex
Classified according to
Functional or non functional status
Benign or malignant features
Adreno-cortical tumors include-
Aldosteronoma[Conn Syndrome]
Cortisol producing adenoma[Cushing syndrome]
Virilizing/feminizing adrenal tumors
Adrenocortical carcinoma
Adrenal Metastases
Nonfunctioning adrenal tumors[Incidentaloma]
14. Diagnosis
Involves screening, confirmatory testing, and subtype differentiation.
Indications for Primary Aldosteronism Screening
Severe hypertension (≥160/≥110)
Hypertension with hypokalemia
Adrenal Incidentaloma with hypertension
Resistant hypertension (three or more oral agents with poor control)
Early-onset hypertension (<20) or stroke (<50 years)
Unexplained hypokalemia (spontaneous or diuretic induced)
Whenever considering Secondary causes of hypertension (i.e., pheochromocytoma or
renovascular
disease)
Evidence of target organ damage disproportionate to degree of hypertension
15. Screening Test
• Prior to screening
• Hypokalemia should be corrected
• All contraindicated medications to be discontinued
• Screening for primary aldosteronism begins by obtaining a morning (between 8
and 10 AM)
plasma aldosterone concentration (PAC) and plasma renin activity (PRA)
• The National Institutes of Health (NIH) Consensus Statement (2002) on the
management of the clinically inapparent adrenal mass suggests cut-offs of
• > 30 for ARR [Aldosterone renin ratio]
• > 20 ng/dL for PAC
• All positive tests must be confirmed with further testing
16. Confirmatory Test
Of the patients with positive screening tests, only 50% to 70% will be diagnosed
with PAL on confirmatory testing
• Of the confirmatory tests available
Following evaluate suppression of aldosterone following sodium loading
• Fludrocortisone suppression test
• Oral sodium loading test
• Intravenous saline infusion test
Following evaluate suppression of ARR following administration of an ACE inhibitor.
• Captopril suppression test
17. Subtype Differentiation
Subtype differentiation of PAL is essential in selecting appropriate
therapy, because surgical therapy is only successful for select subtypes
Adrenal CT scan
Radiographic characteristics of aldosterone-producing adenomas
unilateral low-density non-enhancing lesion of less than 10 Hounsfield units (HU)
Average size of 1.6 to 1.8 cm
Normal appearing contralateral adrenal gland.
~ 20% of aldosterone-producing adenomas = < 1 cm in size. Therefore lateralization is not based on
CT findings alone
Adrenal MRI
MRI does not offer an advantage in delineating primary aldosteronism subtypes, and is a/w
increased cost
Routine use is not currently recommended
18. Lateralisation Studies
•
To establish lateralization of aldosterone secretion in surgical candidates, adrenal vein
sampling should be performed.
• Percutaneous access to the femoral vein is obtained, and catheter tips are positioned to
collect samples
from three sites: the right adrenal vein, left adrenal vein, and inferior vena cava (IVC).
• Samples are evaluated for aldosterone and cortisol concentrations.
• Lateralization of aldosterone secretion is determined by comparing the aldosterone to
cortisol ratios of
the dominant to nondominant sides.
• Aldosterone is considered to be lateralized if ratio is greater than 2-4 : 1, depending on
the use of
ACTH stimulation
Alternative studies when AVS is inconclusive due to sampling error
• Nuclear Scintigraphy
• Postural stimulation testing
• Measurement of cortisol metabolites
19. Cushing Syndrome
Definition
Hypercortisolism secondary to excessive production of glucocorticoids by the
adrenal cortex is defined as Cushing syndrome
Incidence: 2 - 5 cases/million people/yr
SUBTYPES
20. ACTH-Independent Cushing Syndrome
Diagnosis
Screening Test:
24-hour Urinary free cortisol
This test is considered the gold standard diagnostic test.
Direct measure of free bioavailable cortisol
Normal cutoff value of 50 mcg/ 24 hr urine volume is used.
Low dose dexamethasone suppression test
Patients take 1 mg of dexamethasone, a synthetic steroid, at 11 pm and cortisol level is measured at 8 am the
next day.
In normal patients cortisol should be below 5 mcg/dl.
Late-night salivary cortisol
This is the latest diagnostic test for Cushing’s.
Elevated cortisol levels b/w 11 PM & midnight = earliest indication of disease.
Cortisol level should not exceed 145 ng/dl.
In adrenal tumors ACTH level is low and CT/MRI of the adrenal almost always identifies the
tumor.
21. Adreno cortical Carcinoma
It is a rare malignancy with incidence of 0.5 to 2 per million.
Bimodal age distribution,peaks in 1st and 4th -5th decade.
Majority of cases are sporadic and unilateral.
Functional breakdown of ACC
Functional 50%-80%
Cushing Syndrome 33%-53%
Cushing Syndrome + Virilization 20%-24%
Virilization alone 10%-20%
Feminization 6%-10%
Hyperaldosteronism 2.5%-5%
Non functional 20%-50%
22. Diagnosis, Functional Evaluation
Type of Adrenal Hypersecretion Functional Evaluation
Glucocorticoid excess Low- Dose dexamethasone suppression test
Or late night salivary
Or 24 hour urinary cortiisol
Sexual steroids& steroid precursors DHEA-S
17-OH- Progesterone
Androstenedione
Testerone
17B – esterediol( only men & post – menopausal
women
Catecholamine excess Serum or urinary metanephrines
Mineralocorticoid excess Aldosterone to renin ratio(in patients with
hypertension & hypokalemia
23. Imaging
CT Scan : radiographic characteristics of ACC on CT imaging include
irregular borders, irregular enhancement, calcifications,
necrotic areas with cystic degeneration.
Mean attenuation on noncontrast CT scan in ACC is significantly higher (39 HU) compared with
adenomas(8 HU).
No contrast washout
For metastatic evaluation
Lymphadenopathy
Extension to IVC and other adjacent organs.
Distant metastases to lung and liver.
MRI:
ACCs appear isointense relative to the liver or spleen on T1-weighted images and demonstrate
intermediate to
increased intensity on T2-weighted images.
essential tool in detecting the presence of a tumor clot and delineating its extent.
24. Percutaneous needle biopsy
Primary indication for needle biopsy is in cases of unresectable, locally advanced, or
metastatic disease, to confirm the diagnosis prior to systemic medical therapy.
In cases of surgically resectable disease, the information obtained from biochemical and
radiographic evaluation should be enough to justify extirpation.
25. Treatment
Surgery
Approach: Open transabdominal or retroperitoneal approach is preferred
Complete surgical excision is essential in the management of adrenal cortical carcinomas and offers
the
best chance of cure.
For locally advanced disease : enbloc resection should be performed, whenever possible.
Cases of venous tumor thrombus involving the IVC may require vascular bypass techniques, IVC
replacement, and/or IVC interruption.
In cases of metastatic adrenal carcinoma, cytoreductive removal of the primary tumor and
debulking
metastatectomy should be considered if greater than 90% of the disease burden can be removed.
In patients who are not candidates for complete tumor debulking, surgery should not be performed
because it will delay systemic therapy.
Rate of recurrence – 60-80 % despite aggressive surgical resection.
26. Adrenal Incidentaloma
Adrenal incidentalomas are unsuspected
adrenal masses greater than 1 cm in diameter
identified on cross sectional imaging performed for
seemingly unrelated causes.
It is important to emphasize that “incidental”
does not mean “insignificant”.
Incidence : 1-4% patients undergoing abdominal
imaging studies.
29. Pheochromocytoma
Tumour of catecholamine-producing cells of adrenal medulla
Incidence: ~ 1 – 2/1,00,000 individuals
Classically, pheochromocytoma has been called the “10% tumour”: 10%
extra-adrenal, 10% familial, 10% bilateral, 10% paediatric, and 10%
malignant
However up to 25% of pheochromocytomas can be extraadrenal and
upto 30% cases familial.
Tumors arising from the right adrenal are more common, tend to be
larger, and recur more frequently than those that arise in the left gland
Pediatric pheochromocytoma is the most frequently encountered
endocrine neoplasm in children.
32. Pre-operative work up
History & exam:
• Age and obesity
• Is it a functional adrenal mass?
• What is the likelihood of malignancy?
• Past surgical/medical history - preventing
laparoscopy
• Primary or recurrence?
• Comorbidities like HTN, DM, etc
• Consent for Nephrectomy
33. Diagnosis and work up:
• Contrasted CT/ MRI (where is the site and what size is it?)
CROSS-SECTIONAL IMAGING (CT Scan)
• Pheochromocytomas appear as well-circumscribed lesions
• Given their rich vascularity and low lipid content,
Pheochromocytomas typically measure an attenuation > 10 HU on
NCCT (mean ≈35 HU)
• Although nonspecific, Pheochromocytomas, unlike adenomas, do not
exhibit rapid contrast washout on delayed imaging
MRI
• Similar to CT, magnetic resonance imaging is an excellent imaging
modality for characterizing adrenal lesions
• Classically “light bulb” sign is seen which was earlier believed to be
diagnostic of pheochromocytoma.
The right adrenal vein is a potentially perilous structure to manage, because it is short, wide, variable, and confluent with thin-walled, large capacitance vessels. A significant second adrenal vein may be found in up to 10%of patients. In the right AVOID BLEEDING, in the Left GET THE PLANE RIGHT!
