Adrenal Gland Tumors And Their

Managment
Presented By
Dr.Faisal Zia
Post Graduate Resident
Leady Reading Hospital Peshawar

Outline
Anatomy of Adrenal Glands
Short Review Of Physiology
Classification Of Tumors Of Adrenal Glands
Symptoms/Signs
Diagnosis
Management Strategies

Anatomy of Adrenal Glands

Vascular Supply Of Adrenal Gland

-The Arterial supply of each adrenal
gland may arise from three main
sources: superior adrenal arteries
(branches from the inferior
phrenic arteries), middle adrenal
arteries (direct visceral branches
from the aorta) and inferior
adrenal arteries (branches from
the ipsilateral renal artery.
The short right adrenal vein drains
directly into the vena cava. On the
left, the adrenal vein is long
compared with the right and is

 Functionally Adrenal Glands Has two Parts

1. Adrenal Cortex
2. Adrenal Medulla
Adrenal Cortex is Characterized by Zonal Configuration
i.e
a. Zona Glumerulosa ---- Aldosterone ( Na+, k+
Homeostasis)
b. Zona Fasiculata ------ Cortisol ( Stress Hormone)
c. Zona Reticularis ----- Dehydroepiandrosterone
(Androgen precursor)
Adrenal Medulla
The Medulla secretes Epinephrine (80%),
Norepinephrine(19%), and Dopamine (1%).

A. Classification Of Adrenal Tumors

1. Adrenal Cortical Tumors
2. Adrenal Medullay Tumours
1. AdrenoCortical Tumors
a. Benign
b. Malignant

Adenoma/Nodular hyperplasia
Carcinoma

Adrenocortical

2. Medullay tumours
Benign

Malignant

GanglioNeuroma, Pheochromocytoma Neuroblastoma

Other Tumours are :
Myelolipoma
Adrenal Mets

AdrenoCortical Adenoma
Adenomas are the most common neoplasms arising
from the adrenal gland
The incidence of adenomas rises with age.
Adrenal adenomas are by definition benign and the vast
majority are metabolically silent (Non-Functional i-e
Non-hormone producing) But Some May produce
Hormone producing Signs and Symtoms depend Upon
Zone Involved.
May be Unilateral Or Bilateral

Clinical features
Depend upon Type of Adenoma i.e
1. Small Size, Non Hormone producing are usually Asymptomatic, Incidently
diagnosed In CT/MRI Done for Some Other reason where they r Called
Incidentaloma.
2. Large Size, Non functioning Tumours Frequently Present with Abdominal
Discomfort or Back Pain, however with increasing use of Abdominal imaging
they are detected early now
3. Hormone Producing Adenomas will produce Signs/Symtoms of The Hormones
Produced. I-e
Cushing Syndrome If hypersecretion of Cortisol Occurs
Conns Syndrome If hypersecretion of Aldosteron occurs

 Cushing Syndrome
Weight Gain/Central Obesity
Diabetes
Hirsutism
Hypertension
Skin changes (Striae, Facial
Plethora,
ecchymosis,
acne)
Muscle weakness
Menstrual
irregulariety/Impotence
Depression/Mania
Osteoporosis
Hypokalemia

 Conns Syndrome
Headache
Hypertension
Muscle Weakness
Cramps
Intermittent Paralysis
Polyuria
Polydipsia
Nocturia

Diagnosis
Complete History
Clinical Examination
Biochemical evaluation

Morning and Midnight Plasma Cortisol Measurement

Dexamethasone Suppression Test
24 hr Urinary Cortisol measurement
Serum Potassium, Plasma aldosterone and Plasma Renin Activity
Serum Dehydroepiandrosterone or 17 B-hydroxyestradiol (Virilising or feminizing tumour).

Abdominal Imaging
CT Scan :
Is the Initial procedure and will localize Adenoma in approx 90% of Patients

MRI Scan
Equally effective in distinguishing adrenocortical adenoma from Carcinoma

Adrenal vein Catheterization

If no adenoma is visualized, adrenal vein sampling for aldosterone and cortisol will correctly
differentiate adenoma from hyperplasia in virtually all cases.
Similarly Samples From Both the Adrenal Vein And Determing Aldosterone to Cortisol Ratio ACR will
differentiate between unilateral and bilateral disease

Treatment
Any Non-Functioning Adrenal Tumour Greater tha 4cm in
diameter and smaller tumours that increase in size over
time should Undergo Surgical resection.
Non-Functioning Tumours Smaller than 4cm should be
followed up after 6, 12 and 24 months by Imaging and
Hormonal evaluation.
Options for Functioning Tumors
Medical Management
Dietary Sodium Restriction
Spironolactone ------ Conns syndrome
Metyrapone or KetoConazole ------ reduce Steroid Synthesis ------Cushing syndrome

Surgical Management

 Pre-Op Management
Conns and Cushing Related Disease (Hypertension, Diabetes) and
Biochemical Abnormalities should be corrected.
Patients with Cushings Syndrome are at an increased risk of Hospital
Acquired Infection and thromboembolic and myocardial Complications.
Therefore, Prophylactic anticoagulation and the use of prophylactic
antibiotics are essential.

Post-Op Management
Supplementary Cortisol should be give after surgery. In total 15 mg h -1 is
required parentally for the first 12 hr followed by a daily dose of 100 mg for 3
days which is gradually reduced thereafter.
After Unilateral adrenalectomy, the contralateral suppressed Gland need Up2
1 year to recover adequate function.

Adrenocortical Carcinoma
A rare malignancy with an incidence of 0.5 to 2 per
million but generally poor prognosis.
Peaks in children in the first decade of life and adults in
the fourth to fifth decades of life.
Slight female predominance.
The majority of ACCs are unilateral.

Clinical features
Approx 60% present with evidence of Steroid hormone
excess i-e Cushing Syndrome. Else any Hormone Excess
can be the Presentation.
Patients with Non-Functioning tumours frequently
complain of Abdominal discomfort or Back pain caused
by Large tumours

Diagnosis
Complete History
Clinical Examination
Biochemical evaluation

Morning and Midnight Plasma Cortisol Measurement

Dexamethasone Suppression Test
24 hr Urinary Cortisol measurement
Serum Potassium, Plasma aldosterone and Plasma Renin Activity
Serum Dehydroepiandrosterone or 17 B-hydroxyestradiol (Virilising or feminizing
tumour).

Abdominal Imaging
CT Scan
Equally effective in distinguishing adrenocortical adenoma from
Carcinoma
MRI Scan
MRI Angiography ---- TO exclude tumour thrombus in the vena Cava which must
be excluded before Adrenalectomy.
As Distant Metastasis Is frequently Present So a CT scan of the Lung is
recommended

Staging Of Adrenocortical Ca
The World Health Organization Classification of 2004 is
based on the McFarlane classification and defines Four
Stages:
I.
II.
III.
IV.

Stage
Stage
Stage
Stage

=
=
=
=

Tumour < 5cm

Tumour >5 cm
Locally invasive tumours
Tumour with distant metasis

Managment
Complete Tumour Resection (R0) is associated with
favourable Outcome and should be attempted
Whenever Possible.
En bloc resection with removal of locally involved
organs is often required and incase of tumour thrombus
in the vena cava the assistance of a cardiac surgeon is
sometimes needed.
Tumour debulking plays a role in functioning tumours to
control hormone excess.
Laproscopic adrenalectomy is Associated with a high
incidence of local recurrence and cannot be
recommended.

 Adjuvent Chemotherapy
Patient should be treated Postoperatively with Mitotane alone
or in combination with Etoposide, Doxorubicin or Cisplatin in
30% and 50% of cases respectively.

Adjuvent Radiotherapy
May reduce the rate of local recurrence.

Follow Up
After surgery, Restaging every 3 months is required as the risk
of tumour relapse is high.

Prognosis
Depends On the stage of Disease and Complete Removal of
the tumour.
Patients with stage I or II disease have a 5-year survival rate of
25% whereas patient with stage III and stage IV disease have
5-year survival rate of 6% and 0% respectively.

Phaeochromocytoma (Adrenal
Paraganglioma)

Catecholamine-producing tumor derived from the sympathetic and

parasympathetic nervous system, usually located in the adrenal gland.
May also produce Calcitonin, ACTH, VIP, PTHrP
Prevalence in Hypertensive patients is 0.1 0.6% with over all prevalence of
0.05% In autopsy series.
Sporadic Phaechromocytoma occur after the fourth decade whereas patients with
hereditary form are diagnosed early.
Also known as the 10% Tumour i-e

10%
10%
10%
10%
10%

are inherited
Extraadrenal
Malignant
Bilateral
Occur in Children

Hereditary Phaechoromocytoma are Usually Associated with Syndromes like

Multiple Endocrine Neoplasia Type 2
Medullay thyroid Carcina
Primary Hyperparathyroi
Phaechromoctoma

Familial paraganglioma Syndrome

Von Hippel-Lindau Syndrome
Neurofibromatosis Type I

Clinical features
Can be continuous or Intermittent.
Headache
Palpitation
Sweating
Hypertension ( Paroxysmal or continuous)
Pallor
Weight loss
Nausea
Hyperglycemia
Psychological effects
Paroxysms may be precipitated by physical training, induction of
Anesthesia and Drugs like TCA, Metochlopramide, Opiates or Contrast
media

Diagnosis
Complete History
Clinical Examination
Biochemical evaluation
Determination of Adrenaline, Noradrenaline, Metanephrine and
normetanephrine levels in a 24-hr Urine Collection. Level exceeding Normal
range by 2- 40 times will be found in affected patients.
Determination of Plasma free Metanephrine and Normetanephine Levels has
a high sensitivity
Biochemical tests Should be performed at least twice

Imaging
For Localization of Tomour and/Or Metastases MRI is preferred because
Contrast Media used for CT scans can provoke Paroxysms.
123I-MIBG (meta-Iodobenzyl guanidine) single- photon emission computerized
tomography SPECT will identify about 90% of primary Tumours. And is
essential for the detection of Multiple Extra-Adrenal Tumours and Metastases.

Management
Laparoscopic resection.
If tumour Larger than 810cm or radiological signs of malignancy are detected, then an
Open Approach Should be Considered

PreOperative Care
Once Diagnosed, An alpha adrenergic Blocker (Phenoxybenzamine) is used to
block Catecholamine excess and its consequences during Surgery.
A dose of 20 mg of Phenoxybenzamine initially, should be increased daily by
10mg until a daily dose of 100160 mg is achieved and the patient reports
symptomatic postural Hypotension.
Additional Beta-Blockade is required if tachycardia or arrhythmias develops, and
this should not be introduced until the patient is alpha Blocked.

PerOperative Care
In Some patients, a dramatic changes in heart rate and Blood pressure may occur
and require sudden administration of Pressor or Vasodilator agents. A central
Venous Catheter and invasive arterial monitoring are essential.
Special attention is required when the adrenal vein is ligated as a sudden Drop in
Blood Pressure may Occur.
Rarely, Infusion of large volume of Fluid or even administration of Adrenaline can
be necessary.

 Post Operative Care

Patient should be Observed for 24 hr in the ICU as Hypovolemia and
hypoglycemia may Occur.

Follow Up
Biochemical cure should be confirmed by an assessment of
catecholamine 2-3 weaks Postoperatively.
Lifelong Yearly Biochemical tests should be performed to identify
recurrent, metastatic or metachronous phaeochromocytoma.

Malignant phaechromocytoma
App 10% of Phaechromocytoma are malignant
Rate is higher in Extra-adrenal tumours (Paraganliomas).
Diagnosis of Malignancy Implies Mets to Lymph Node, bone and liver.
About 8% apparently benign tumour subsequently develop metastasis.

Treatment
Surgical excision Is only chance of Cure.
Even in patient with metastatic disease, tumour debulking can be considered
to reduce the tumour burden and to control the catecholamine excess.
Symtomatic treatment can be obtained with alpha-blockers.
Mitotane should be started as Adjuvent Or Pallistive treatment.
Treatment with 131I-MIBG or combination chemotherapy has resulted In a
partial response in 30% and an improvement of symtoms in 80% of patients.
The natural History is highly Variable with a 5 year survival rate of < 50%.

NeuroBlastOMA
A Malignant tumour Derived from Sympathetic nervous system in the Adrenal
Medulla (38%) or from any site along the sympathetic chain in the paravertebral
sites of the abdomen (30%), chest (20) and rarely, the neck or Pelvis.
Predominantly Newborn Infants and Young Children (<5 years of age) are
affected
Metastatic disease is present in 70% of patients.

Clinical Features
Symtoms are caused by tumour growth or bone metastases.
Patients present with a mass in the abdomen, neck or chest, proptosis, bone
pain, painless bluish skin metastases, weakness or paralysis.
The catecholamine exces is asymptomatic and an excess ACTH or VIP may
Occur.

 Diagnosis
Biochemical Evaluation
Urinary excretion (24 hr Urine) of VMA, Homovanillic acid, Dopamine and Noradrenaline, an
increased levels are present in about 80% of patients.

Accurate staging requires CT/MRI of the Chest and Abdomen, a bone scan, bone
marrow aspiration and core Bioipsies as well as an MIBG scan.

Treatment
Prognosis and Treatment plan can be predicted by the tumour stage and the age at
diagnosis.
Patients are classified as low, intermediate or high risk.
Low Risk
Patients are treated by surgery alone ( the Addition of 612 weeks of chemotherapy is optional.
3 year survival rate of 90%.

Intermediate Risk
Patients are treated by surgery with Adjuvent Multi-agent chemotherapy i.e carboplatin,
cyclophosphamide, etoposide, doxorubicin.
3 year survival rate of 70--90%.

High risk
Patient receive High dose Multi-agent chemotherapy followed by surgical resection in responding
tumours and myeloablative stem cell rescue.
3 year survival rate of 30%.

Ganglioneuroma
Benign adrenal Neoplasm of neural crest tissue i.e adrenal
medulla, paravertebral sympathetic plexus.
Found in all age groups More Common Before 60 year of age.
Most often they are identified incidentally by CT or MRI Performed
for other indications.
Treatment is by Surgical excision, Laparoscopic when
adrenalectomy is indicated.

Myelolipoma
Myelolipoma is a rare lesion of the adrenal gland that contains
hematopoietic elements and mature adipose tissues.
Surgery is indicated only for extremely large or symptomatic
lesions.

SURGERY OF THE ADRENAL GLAND

Laparoscopic or Retroperitoneoscopic Adrenalectomy had become the Gold
Standard in the resection of Adrenal Tumours, except for tumours with signs
of malignancy.
The more popular approach is the laparoscopic Transperitoneal approach,
which offers a better view of the adrenal region and may be easier to learn.
Advantage of the retroperitoneoscopic approach is the minimal dissection
required by this extra-abdominal procedure.
In case of small, bilateral tmours or in patients with hereditary tumour
syndromes a subtotal resection is warranted.
The mortality rate ranges from 0 to 2% in specialized centres.
An Open Approach should be considered if radiological signs, distant
metastases, large tumours (>8-10cm) or a distinct hormonal pattern suggest
malignancy. The surgical access in such cases should be thoracoabdominal.

 Laparoscopic Adrenalectomy
Knowledge of the anatomy of the adrenal region is essential as anatomical landmarks guide the
surgeon during operation. If these landmarks are respected, injury to the vena cava or renal vein,
the pancreatic tail or the spleen can be avoided.
Carefull haemostasis is essential as small amount of blood can impair the surgeons view.
To prevent spillage, direct grasping of the adrenal tissue/tumour has to be avoided.

Laparascopic Transperitoneal Approach

Althought this Approach is possible with the patient Supine, it is usually better to place the patient
in lateral decubitus Position, Head up and the table broken to open the renal angle, so that gravity
acts as a natural retractor.

Right Adrenalectomy
Three or four right subcostal ports are commonly used.
The right triangular ligament is divided and the liver retracted medially to expose the kidney and
IVC. A vertical incision medial to the upper pole of the kidney exposes the Gland.
The Dissection continues at the level of the periadrenal fat using careful coagulation and is
finished by complete or subtotal removal of he adrenal gland.

Left Adrenalectomy
With the patient positioned On his or her right side, three left subcostal ports usually suffice.
The splenic flexure is mobilized and the lienorenal ligament divided. The spleen then displaces
forward under gravity to expose the kidney and adrenal gland.
The resection is completed by the transection of the adrenal gland at the level of the periadrenal
fat.

 Retroperitoneoscopic adrenalectomy
The patient is positioned in the lateral decubitus or the prone jack-knife
position as for the open retroperitoneal approach.
The first port is placed under direct vision into the retroperitoneum and
a retroperitoneal space is created by balloon insufflation. Completeness
of adrenal gland excision can be a particular problem with the posterior
retroperitoneal approach.
Recurrent cushings syndrome has been reported in upto 20% of cases.

 Open Adrenalectomy
Through a thoracoabdominal approach, it is almost exclusively performed when a
malignant adrenal tumour is suspected.
A transverse Upper abdominal incision is satisfactory; alternatively, it can be a curved
incision, convex upwards. A subcostal incision is suitable for a unilateral adrenalectomy.
On the right Side the Hepatic Flexure of the colon is Mobilised and the right liver lobe is
cranially retracted to achieve an optimal exposure of the inferior vena cava and the
adrenal gland.
On the left side the adrenal gland can be exposed after mobilization of the splenic flexure
of the colon, through the transverse mesocolon or through the gastrocolic ligament.
The remaining dissection is the same as in laparoscopic adrenalectomy.
A resection of regional lymph nodes Is recommended in malignant adrenal tumours and
should include resection of the tissue between the renal pedicle and the diaphragm
Open Loin And Posterior Approach
Any of the loin Approaches for renal surgery can be used. The lateral decubitus position is used
for a loin incision and the prone jack-knife position for the posterior lumbotomy incision.
Posterior inicisions have an advantage if both adrenal glands must be explored, as the patient
does not need to be turned.