The document details adrenal tumors' anatomy, physiology, and management, highlighting various types including incidentalomas, adrenocortical carcinoma, pheochromocytoma, and neuroblastoma. It discusses diagnosis methods such as imaging, laboratory tests, and treatment approaches including surgery and medication. Additionally, it outlines the characteristics and clinical features associated with these tumors, emphasizing the importance of proper assessment and intervention.

1. z
ADRENAL TUMORS

2. z
ANATOMY
 Paired glands , weighing 4g
 Retroperitoneal
 Near upper poles of kidneys , within gerotas facial
 Right adrenal gland – between right love of liver and
diaphragm, partly behind IVC
 Left – superomedial to upper poles of left kidney ,
covered by pancreatic tail and spleen

3. z
BLOOD SUPPLY
 Multiple small arterial branches
from
 The aorta
 Inferior phrenic artery
 Renal artery
 Venous drainage
 Right – IVC
 Left – Renal vein

4. z
PHYSIOLOGY
 Zona glomerulosa – Aldosterone
 Zona fasciculata – cortisol regulated by ACTH
 Zona reticularis – Androgens – DHEA – under control of ACTH

5. z
INCIDENTALOMA
 An asymptomatic adrenal mass detected on imaging not performed for suspected
adrenal disease
 Etiology
 Benign and malignant tumors of cortex , medulla , extra adrenal origin
 Functional or non functional – determined through
 clinical assessment
 Dexamethasone suppression test
 measurement of urinary metanephrins
 Plasma Aldosterone renin ratio
 2% incidence

6. z
 Benign or Malignant
 Clinically cushings and virilising tumors – Malignant
 Non contrast CT – Benign tumors <10HU
 PET /MRI

7. z
INVESTIGATION AND MANAGEMENT
ALGORITHM FOR INCIDENTALOMA

8. z
CORTICAL TUMORS
 ALDOSTERONE PRODUCING ADENOMA( Primary hyperaldosteronism )
 Arises from Zona glomerulosa
 Max diameter 10 to 20mm
 Macroscopically – Well circumscribed , golden yellow in color ( canary tumor )
 Mutations in potassium coded channels , triggering calcium influx into
glomerulosa cells
 C/F
 HTN
 Hypokalemia
 Can be asymptomatic

9. z
 Diagnosis
 Non suppressed plasma Aldosterone and suppressed plasma renin
activity
 Radiologically – CT and PET
 Treatment
 Prior to surgery – HTN and hypokalemia to be corrected
 Surgery – Adrenalectomy

10. z
ADRENAL ADENOMA
 Most common cause of adrenal Cushings syndrome
 Well circumscribed, modular in appearance
 Results in hypercortisolim leading to suppressed ACTH ,
causing atrophy of fasciculata , reticularis not glomerulosa

11. z

12. z

13. z
DIAGNOSIS
 Late night salivary cortisol levels
 24 hr urinary free cortisol excretion - >3
times of upper limit
 Non suppression of morning cortisol levels
after overnight dexamethasone
suppression test
Radiologically – Adrenal CT or MRI

14. z DEXAMETHASONE SUPPRESSION TEST
 In this test, 1 mg of a synthetic glucocorticoid (dexamethasone)
is given at 11 p.m. and plasma cortisol levels are measured at 8
a.m. the following morning.
 Physiologically normal adults suppress cortisol levels to <3
μg/dL, whereas most patients with Cushing’s syndrome do not.
 In patients with a negative test but a high clinical suspicion, the
classic low-dose dexamethasone (0.5 mg every 6 hours for eight
doses, or 2 mg over 48 hours) suppression test or urinary
cortisol measurement should be performed.

15. z
TREATMENT
 Unilateral adenoma – Adrenalectomy
 Bilateral Adrenalectomy – in severe and symmetrically enlarged
adrenal
 Asymmetric disease – excision of larger gland

16. z
ADRENOCORTICAL CARCINOMA
 Rare aggressive malignancy from adrenal cortex
 Presents at late stage with poor prognosis
 Sporadic – majority , or as a part of tumor
syndromes ( MEN 1 )
 Incidence – 1 in 1 lakh
 Female preponderance
 Non functional > functional

17. z
 Clinical features
 Abdominal lump ,
 Abdominal or back pain
 Features of Cushing syndrome
 Pathology
 Large tumors with necrosis
 Capsular invasion
 Abnormal mitosis
 Investigations
 MRI
 CT
 24 hr uninary metanephrins
 Overnight dexamethasone suppression test

18. z
Treatment
 Indeterminate or probably malignant tumor < 6cm : laparoscopic
Adrenalectomy
 Indeterminate or probably Malignant>6cm : Open radical
resection with en bloc dissection of involved adjacent organs
 Metastatic disease
 Limited disease – open resection of primary tumor with adjuvant
treatment of mets
 Widespread metastatic disease – palliative treatment

19. z
ADRENAL MEDULLA TUMORS
 PHEOCHROMOCYCTOMA AND PARAGANGLIOMA (PPGL )
 Tumors arising from the neuroectoderm tissue of adrenal
medulla are pheochromocytomas( PCC )
 Those arising from extraadrenal parasympathetic ( carotid body
) and sympathetic ganglia( organ of zuckerkandl) are
paragangliomas ( PGL )
 70% sporadic , remaining –familial
 Rule of 10 – 10% Malignant, 10% familial , 10%bilateral ,10%
Extraadrenal,10%Children .

20. z
 Pathology
 Highly vascularised with greyish pink
on cut surface
 Areas of necrosis and hemorrhage
 PCC produce calcitonin, ACTH,
vasoactive intestinal polypeptide
(VIP) and parathyroid hormone-
related protein (PTHrP)

21. z
Diagnosis
 Biochemical –
 Plasma metanephrine levels
 24 hr urinary catecholamine levels
 Plasma dopamine levels – marker
of tumor burden in malignant
disease
 Radiological
 CT /MRI
 MIBG scan
 FDG PET

22. z
Treatment
 Medical Management
 Pre-op control of blood pressure with phenoxybenzamine ( Alpha
adrenergic receptor blocker ) 20mg initially followed by 10mg until
daily dose of 100 – 160 mg is reached
 Beta adrenergic blockers if tachycardia or arrhythmias present
 Post operatively – hypoglycemia and hypovolemic should be
checked
 Surgical Treatment
 Adrenalectomy

23. z
Neuroblastoma
 Most common and deadliest extracranial malignancy in children
 Derived from primitive nerve cells of sympathetic nervous
system of neural crest cells
 Clinical enigma
 Peak incidence – 5yrs of age
 Clinical features – lump across midline of abdomen , Diarrhea,
dehydratio due to VIP
 Metastatic lesions – blue berry muffin lesions , racoon eyes

24. z
 Investigations – MRI > CECT
 Treatment – Surgical Resection of primary tumor
Chemotherapy – Etoposide , Cisplatin

25. z
GANGLIONEUROMA
 Ganglioneuromas (GNs) are benign diferentiated tumours of neural
crest-derived cells in the autonomic nerves.
 Sporadic , assosciated with MEN 2b
 Pathology – admixture of ganglion and schwann cells
 Sites – Mediastinum , retroperitoneal, Adrenal gland
 Clinical features – benign , present as non secreting slow growing
tumors
 Usually asymptomatic, <30years , when large in size cause
pressure symptoms

26. z
 Diagnosis – CT /MRI – well defined with capsule and
calcification
 Treatment – Surgical excision – laparoscopic method preferred
 Excellent prognosis

27. z
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