Introduction :

A tumor begins when healthy cells change and grow out of control, forming a
mass. A tumor can be cancerous or benign. A cancerous tumor is malignant,
meaning it can grow and spread to other parts of the body. A benign tumor
means the tumor can grow but will not spread.

An adrenal gland tumor can sometimes produce too much of a hormone.

When it does, the tumor is called a 'functioning tumor." An adrenal gland tumor
that does not produce hormones is called a "nonfunctioning tumor."

Definition :

Adrenal tumors are cancerous or noncancerous growths on the adrenal glands.

 An adrenal tumor or adrenal mass is any benign or malignant neoplasms

of the adrenal gland, several of which are notable for their tendency to
overproduce endocrine hormones.

Anatomy of Adrenal Glands :

The right suprarenal gland

 Is pyramid shaped.
 Caps the upper pole of the right kidney.

 Relations :
 Anterior : Right lobe of the live and inferior vena cava.
 Posterior : diaphagram
 The left suprarenal gland
 Is crescentic in shape
 Extends along the medical border of the left kidney from the upper
pole to the hilus.
 Relations :
 Anterior : Pancreas, lesser sac. and stomach.
 Posterior : Diaphragm
 Vascular supply of Adrenal Gland :
 The Arterial supply of each adrenal gland may cause, 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 venin is long compared with the right and joined by the
inferior phrenic venin prior to draining into the left renal vein.
 Functionally adrenal glands has two parts :
1. Adrenal cortex
2. Adrenal Medulla
Adrenal Cortex : Adrenal cortex is characterized by zonal configuration i.e.

a. Zona Glumerulosa – Aldosterone (Na+, K+, Homeostasis)

b. Zoma Fasiculata – Cortison (Stress Hormones)
c. Zoma Reticular – Do hydroeplandrosterone, (Androgen, procursar)

Adrenal Medullo :

The Medulla secretes Epinophrine (80%)

Norepinophrine (19%) and Dopmine (1%)

Histology :

Two distinctive parts : 1. Cortex

2. Medulla

Cortex :
 Hesodermal origin
 Three distinct layer : Glomerulasa : Hineralcarticoids.
Fasciculata : Glucocarticoids
Reticularis : Sex hormonas

(2) Module :
 Neuroectodermal in origin (neural crest)
 Secrete catecholamines : Epinophrione and norepinephrione
 Glomerulosa : Well – outlined cells aggregated into small clusters and short
trabaculae.
 Fasciculata : broad band made up of large cells with distinct membranes
arranged in two cell-wide cards. Cytooplasm is vacuolated /clear.
 Reticularis : haphazard arrangement of cells with granular cytoplasm.
Lipotusein may be present.
Classification of Adrenal Tumors

1. Adrenal cortical tumors

2. Adrenal medullary tumors

1) Adrenal Cortical Tumors

a) Benign b) Malignant
Adenoma/Nodular hyperplasia Adrenolartical Carcinoma
2) Medullar Tumors :
a) Benign b) Malignant
Ganglioneuroma Neuroblastoma.
Pheorhromocytoma

3) Others tumors are :

 Myelolipoma
 Adrenal Mets
A. Adrenocortical Adenoma

 Adenomas are the most common neooplasmg arising from the adrenal
gland.
 The incidence of adenomas rises with age.
 Adrenal adenomas are the definition benign and the vast majority are
metabolicallysilent (Non Functional i.e. Non-hormone producing). But
some may produce hormone producing signs and symptoms depend upon
zone involved may be unilateral or bi-lateral.

Clinical Features :

1. Small Size, non-Hormone producing are usually asymptomatic, ioncidently

diagnosed in CT/NRI done for some others reason where they are called
incidentaloma.

2. Large size, Non functioning Tumors; Frequency 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/symptoms of the

hormones produced i.e.

 Cushing syndrome if hypersecretation of cortisone occurs.

 Conn's syndrome of hypersecretion of Aldexteron occurs.

Crushing Syndrome

 Weight gain / Control obesity

 Diabetes
 Hirsuitism
 Hypertension
 Skin Changes (Straie, Facial POlethora, Eecymosss, acene)
 Muscle weakness
  Menstrual irregularity /importance
 Depression/Mania
 Osteoporosis
 Hypokalemia

Conn's 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 Cartisol measurement
 Serum potassium, plasma aldosterone and plasma rennin activity.
 Serum dehydropiandrosterone or 17B hydroxyes stradior (Veiling or
feminizing tumor)

Abdominal Imaging
 CT Scan :
 Is the initial procedure and will localize Adenoma in approx 90% of
patients.
 NRI Scan
  Equally effective in distinguishing adrenocartical adenoma from
carcinoma.
 Adrenal Vein catheterization
 If no adenoma is visualized, adrenal vein sampling for aldesterone and
cortisol will correctly differentiate adenoma from hyperplasia in
viritually ill cases.
 Similarly samples from both the adrenal vein and determining
aldosterone to cortisol ratio ACR will differentiate between unilateral
and bilateral disease.

Treatment
 Any Non-functioning Adrenal Tumor greater than 4 cm in diameter and
smaller tumors that increase in size over times should undergo surgical
resection.
 Non-functionniog tumors smaller than 4 cm should be followed up after 6,
12 and 24 months by imaging and Hormonal evaluation.
 Options for functioning tumors –
o Medical Management
 Dietary Sodium restriction
 Spironolactone - Conn's Syndrome
 Metyrapone or Ketoconazole – reduce steroid synthesis –
Cushing syndrome.
o Surgical Management
 Unilateral Adenoma – Unilateral Adrenalectomy
 Bilateral Disease – Bilateral Adrenalectomy

Pre-Operative Management
 Conn's 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 myrardial complications.
 Therefore, prophylactic anticoagulation and the use of prophylactic
antibiotics are essential.

Post-Operative Management
 Supplementary cortisol should be give after surgery. In total 15mg/h is
required parentally for the first 12 hr. followed by a daily dose of100 mg
for 3 days which is gradually reduced thereafter.
 After unilateral adrenalectomy, the contralateral suppressed Gland need
upto 1 year to recover adequate function.

B. Adrenocortical Carcinoma
 A rare malignancy with an incidence of 0.5 to 2 per million but generally
poor programs.
 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 Aces are unilateral.

 Clinical Features :
 Approx.60% present with evidence of steroid hormone excess i.e. cushing
syndrome. Else hormone excess can be the presentation.
 Patients with non-functioning tumors frequently complain of abdominal
discomfort or back pain caused by large tumours.
 Diagnosis
 Complete history
 Clinical examination
 Biochemial evaluation
 Morning and midnight plasma cortisol measurement.
 Dexamethasone suppression test.
 24 hr. urinary cortisol measurement
  Serum potassium, plasma aldosterone and plasma rennin
activity.
 Serum dehydroeplandrosterone or 17 B-hydroxyesteradiol
(virilising or feminizing tumor)
 Abdominal Imaging –
 CT Scan – equally effective in distinguishing
adrenocortical adenoma from carcinoma.
 MRI scan
 MRI Angiography – To exclude tumor thrombus in the
vena cava which must be excluded before
Adrenalection.
 As distant metastasis is frequently present to 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. Stage = Tumour <5 cm
II. Stage = Tumour >5 cm
III. Stage = Locally invasive tumors
IV. Stage = Tumour with distant metasis

 Management
 Complete tumour resection (RD) is associated with favourable outcome
and should be attempted whenever possible.
 Enbloc 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 contol
hormone excess.
 Laproscopic adrenalectomy is associated with a high incidence of local
recurrence sand cannot be recommended.
 Adjuvent Chemotherapy
 Patient should be treated post-operatively with imitatano alone or ion
combination with elaposide, 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 tumor
relapse is high.
 Prognosis
 Depends on the stage of disease and complete removal of the tumor.
 Patients with Stage – I or II disease have a 5-year survival rate of
25% whereas patient with stage III stage IV disease have 5-year
survival rate of 6% and 0% respectively.

C. Phaeochromocytoma (Adrenal Paaganglioma)

 Catecholamine – producing tumor derived from the sympathetic and
parasympathetic nervous system, usually located in the adrenal gland.
 May also produce calcltonin, ACTH, VIP, PTHrP.
 Prevalence in hypertensive patients is0.1 - 0.6% with over all prevalence of
0.05% in autopsy series.
 Sporadic phaechromocytoma occur after the fourth decade whereas
patients with hereditary from are diagnosed early.
 Also known as the 10% tumour si.e.
 10% are inherited.
 10% extraadrenal
 10% Malignant
 10% Bilateral
 10% occur in children
 Hereditary phaecharomocytoma are usually associated with symptoms like
  Multiple Endocrine Neoplasia Type – 2
 Medullary thyroid carcinoma
 Primary hyperparthoid
 Phaechromactoma
 Familial paraganglioma syndrome.
 Von Hippel – Lindau Syndrome
 Neurofibromatosis Type – I

Clinical Features
 Continuous / 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 likeTCA, Metochlopramide, Opiates or contrast
media.

Diagnosis :
 Complete history
 Clinical Examination
 Biochemical Evaluation
 Determination of Adrenaline,Naradrenaline, Matenephrine and
non-metanepohrine levels in a 24 hr. urine collection level exceeding
normal range by 2-40 times be found in affected patients.
  Determination of plasma free metamephrine and non-metanephrine
levels has a high sensitivity.

 Biochemical tests should be performed at least twice.

 Imaging
 For localization of tumor and/or Metastases MRI is preferred because
contrast media used for CT scans can provoke paroxysms.

 IMJBG (meta-Iodobenzyl guanidine) single – photon emission

computerized tomography SPECT will identifyabout 90% of primary
tumors. And is essential for the detection of multiple Extra – Adrenal
Tumours and Matastases

 Management
 Laparoscopic resection
 It tumour larger than 8 – 10 cm or radiological signs of malignancy are
detected, then an open approach should be considered.

Pre-operative care -
 Once diagnosed, Analpha adrenergic blocker (phenoxybenzemine) is used
to block catecholamine excess and its consequences during surgery.

 A dose of 20 mg of phenoxybenzamine initially, should be increased daily

by 10 mg, until a daily dose of 100-160 mg is achieved and the patient
reports symptomatic postural hypotension.

 Additional Beta-Blocker is required if tachycardia or arrhythmias develops,

and this should not be introduced until the patient is alpha blocked.

Post-Operative 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 administrationi of

adrenaline can be necessary.

 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 weeks postoperatively.
 Lifelong, yearly biochemical tests should be performed to identify
recurrent, metastatic or metachronous phaeochromocytoma

Malignant Phachromocytoma
 App 10% phaochromocytoma are malignant.
 Rate is higher in extra-adrenal tumours (Paragnliomas)
 Diagnosis of Malignancy implies mets to lymph node, bone and liver.
 About 8% apparently benign tumour subseque4ntly 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 Adjuventor pallistive treatment.

 Treatment with 131

I-MIB G or combination chemotherapy has resulted in
partial response in 30% and an improvement of symptoms 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 a long 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 :
 Symptoms are caused by tumour growth or bone metastases.
 Patients present with a mass in the abdomen, neck or chest, proptosis, bone
pain, painless blush skin metastases,m weakness or paralysis.
 The catecholamine excess is asymptomatic and an excess ACTH or VIP
may occur.

 Diagnosis
Biochemical Evaluation :
 Urinary excretion (24 hr. urine) of VHA, Homovanillie acid, Dopamine and
noradrenaline, an increased levels are present in about 80% of patients.
 Accurate staging requires CT/NRI of the chest and abdomen, a bone scan,
bone marrow aspiration and core bioipsies as well as an MIBG can.
Treatment :
 Prognosis and treatment plan can be predicted by the tumour stage and the
age of diagnosis.
 Patients are classified as low, intermediate or high risk.

Low Risk
 Patients are treated by surgery alone (the Addition of 6-12 weeks of
chemotherapy is optimal).
 3 year survival rate of 90%.

Intermediate Risk
 Patients are treated by surgery with adjuvant 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 myeloblastive 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 years of age.
 Most often they are identified incidentally by CT or HRI performed for
other indicators.
 Treatment is by surgical excision, laparoscopic when adrenaectomy 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 Adrenalector 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 many be easier to
learn.

 Advantage of the retroperitoneoscopic approach is the minimal dissection

required by this extra-abdominal procedure.

 In case of small, bilateral tumours 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 – 10 cm) or a distinct hormonal pattern
suggest malignancy. The surgical access in such cases should be
thoracobdominal.

Laparoscopic Adrenalectomy

 Knowledge of the anatomy of the adrenal region is essential as anatomical

landmarks guide the surgeon during operation. If these land marks are
respected, injury to the vena cava or renal vein, the pancreatic tail or the
spleen can be avoided.
  Careful hasesmostatis is essential as small amount of blood can impair the
surgeon's view.

 To prevent spillage, direct grasping of the adrenal tissue/tumour has to be

avoided.

Laparascopic Transperitoneal Approach

 Although 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 the gravity acts as natural retractor.

Right Adrenalectomy
 Three or four right subcostal posts 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 of at the level of the periadrenal fat using careful
coagujlation and is finished by complete or subtotal removal of the adrenal
gland.

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

Retroperitoneoscopic adrenalectomy :
  The patient is positioned in the lateral docubitus or the prone jack-knife
position as for the open retroperitoneal approach.
 The first part is placed under direct vision into the retroperitoneum and a
retroperitoneal space is created by ballon insulflation. Completeness of
adrenal gland excision can be a particular problem with the posterior
retroperitoneal approach.
 Recurrent cushing's 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 mobilized 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 modes 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 incisions have an advent age if both adrenal gland must be
explored, as the patient does not need to be turned.

Anterior Approach (Transabdominal)

 Provides wide-exposure and access to both adrenals.
 Allows staging of adrenal malignancies.
 The preferred route for resection of large, potentially invasive adrenal
tumors
 In patients with extremely large (>10 to 15 cm) adrenocartical carcinomas,
the bilateral subcostal incision can be extended in the upper midline to the
xiphoid (Mercedes incision) to allow improved exposure and mobilization
of the liver.

Conclusion :
Adrenal turmours are cancerous or noncancerous growths on the adrenal glands.
The cause of most adrenal tumours is unknown risk factors for adrenal tumors
can include carney complex, lifraumeni syndrome, multiple endocrine neoplasia.
Adrenal tumors may be removed surgically.