Adrenal_gland (Endocrinology) --_Anatomy.ppt

Adrenal anatomy

1. ADRENAL TUMOR
Vinay k jha

2. Adrenal gland --Anatomy
•Suprarenal gland
•2--in NO.
•Situated on posterior abdo. Wall
•Lies over upr. Pole of kidney behind peritoneum
•2–parts outer cortex , inner medulla
•Lies in epigastrium , at upr. Pole of kidney, in front of crus of
diaphragm, opposite vertebrl end of 11 ICS and 12 rib.

4. Cont.
•Measures–50 mm height, 30 mm
breadth,10mm thickness.
•1/3 0f kidney at birth, 1/30 of it in adult.
•Weight–5g ; Medulla form 1/10 of gland.
•Sheaths–immediately by areolar tissue
outer to it renal fascia .

5. CONT.
•Rt. Gland --triangular/piramidal.
•Apex , base, two surfaces, three borders
•Lt. Gland –semilunar .
•Two ends , two border, two surfaces.

6. CONT.
•Arterial supply–superior suprarenal artery
--middle suprarenal artery
--Inferior suprarenal artery
•Venous drainage—Rt suprarenal vein-IVC
Lt suprarenal vein-Lt renal vein.
•Lymphatic drainage-drains to lateral aortic nodes.
•Nerve supply–preganglionic sympathetic fibers.

7. CONT.
•Adrenal medulla belongs to chromaffin
tissue
•Cells stain yellow when treated with
chromium salt
•Apart from adrenal medulla chromaffin
tissue seen in relation to posterior
abdominal wall

8. Adrenal tumor
•Origin from adrenal cortex
adrenocortical adenoma
adrenocortical carcinoma
•Origin from adrenal medulla
neoplasm of sympathetic neuron
ganglioneuroma
neuroblastoma
neoplasm of chromaffin cells
phaeochromocytoma

9. Adrenal medulla--tumor
•Phaeochromocytoma –composed of
chromaffin cells which synthesize and
release catecholamine
•Give rise to surgically correctable forms of
hypertension
•Occasionally may be associated with
cushing syndrome

10. CONT.
•85% Arises in medulla
•Remainder in any extra adrenal
paraganglia
•90% occur sporadically
•10% Autosomal dominant , familial
•40-60 yrs age , slight female
preponderances
•In familial syndrome male preponderances

11. CONT.
•Familial –b/l (70%)
•Non familial –b/l (10-15 %)
•Frank malignancy more common in tumor
arising in extra adrenal site (20-40%) than
in adrenal (10%)

12. CONT.
•Morphology–Ranges from small
circumscribed confined to adrenal to large
hemorrhagic mass weight in kilogram
•Average 100 gm
•Fibrous trabecluae ,richly vascularized,
pass into tumor and produces lobular
pattern

13. CONT.
•Remnant of adrenal can be seen
•Cut section yellow , large lesion ,
hemorrhagic ,necrotic, and typically efface
adrenal gland
•Histologically quite variable polygonal to
spindle shaped chromaffin cells , clustered
with supporting cells into small nets or
alveoli by a rich vascular network

14. CONT.
•Cytoplasm finely granular appearance
•Cellular and nuclear pleomorphism often
present, giant cells often seen
•Mitotic figure rare and does not imply
malignancy
•Vascular and capsular invasion may be
seen in benign lesion
•Diagnosis of malignancy exclusively on
presence of metastases

15. Clinical feature
•hypertension-continuous in 50% and
paroxysmal in 50%
•Palpitation , sweating and headache
•Events which raises intra-abdo. Pressure
may precipitate attacks
•Labour or invasive procedure may ppt.
attack

16. CONT.
•Incidence of MI ,hypertensive
encephalopathy, CVA and catecholamine
cardiomyopathy are all raised
•Flushing , pallor, pupillary dilatation,
raynaud’s phenomenon, fever, tremor,
nausea, weakness, vertigo, anxiety

17. CONT.
•Generalized abdo. Pain, ileus, ischemic
enterocolitis and acute megacolon
•Glucose intolerance and severe
hyperglycemia

18. CONT.
•O/E; thin ,pale anxious patient
•Hypertension may be severe
•Postural drop in BP and tachycardia
•Abdo. Mass in 15% of patients
•MEN 2A, 2B

19. Investigation and diagnosis
•Establish catecholamine excess and
localize tumor
•24 hour urine collection in acidified
container and measurement of
metanephrine and VMA content of urine
•VMA In excess of 7mg/24hour
•Aderanaline ,noradranaline , and
dopamine also assessed by high pressure
liquid chromatography

20. CONT.
•In border line cases measurement of plasma
free catecholamine level may be helpful
•Provocation test –historical interst
•Localization-MRI localizing procedure of choice
, tumor displayed as low signal intensity T1 –
weighted images with a characteristic almost
unique , hyperintense image on T2 weighted
images

21. CONT.
•MRI is of particular value in localizing
extra-aderanal paragangliomas
•Contrast enhanced CT scan
•MIBG-123 I Metaiodobenzylguanidine has
been reported to have sensitivity 90% and
specificity –100% in tumor localization

22. TREATMENT
•Pre-operative control of hypertension
•Long acting alpha adrenergic blocker-
phenoxybenzamine
•Started 7-10 days prior to surgery-10 mg
bd, increasing by 10-20 mg /day until
hypertension is controlled (160 mg/day)
•Encourage liberal fluid and salt

23. CONT.
•3-4 Days prior to surgery Propranolol-40
mg/day
•ANAESTHESIA-for pheocromocytoma
•Central venous line and arterial lines
•ECG, IV access, urinary bladder
cathetrization
•Pulmonary wedge pressure for patient
with compromised cardiac function

24. CONT.
•Anaesthesia is best maintained by
isoflurane
•Halothane –unwanted cardiac arrythmia
•A range of pressure regulating agent
(phentolamine, sodium nitroprusside,
noradrenaline and dopamine) should be
available
•Maintain and monitor BP on minute to
minute basis throughout procedure

25. CONT.
•IV lignocaine (50-100 mg) –controlling
arrythimia
•Cardioversion equipment –available
•Danger period –induction ,intubation, and
pre-operative handling of tumor
•Crucial period immediately after resection
of tumor when hypotension can occur

26. CONT.
•B/L adrenalectomy –hydrocortisone
100mg on induction and at time of tumor
removal
•

27. SURGERY
•Special consideration-minimal handling of
tumor
•Avoid rupture of tumor as it may result in
recurrence
•Complete haemostasis
•Close communication with anaesthetist
•Surgical removal is the treatment

28. Post-operative management
•Intensive care monitoring of arterial BP ,
CVP, urinary output
•Fluid requirement guided by above
parameters
•When hypotension persist , haemorrhage
rather than refractory vasodilatation
should be considered
•Avoid over transfusion –pulmonary
oedema

29. CONT.
•Blood glucose monitored particularly in 1
st
6 hrs as life threatening hypoglycemia can
occur
•Persistent hypertension –residual tumor,
metastatic disease, chronic renal damage
•Urinary catecholamine and VMA
measurement repeated post-resection
anually thereafter

30. CONT.
•Epidural anaesthesia is valuable
•Anticipate profound swing in BP either
direction
•For B/L operation during 1
st
24 hour --no
more than 300 mg hydrocortisone ,
dosage should be halved each day until a
maintenance dos hydrocotisone 30 mg is
reached
•Fludrocortisone 0.1 mg / added

31. CONT.
•Patient should always carry a card
stating dosage of corticosteroid being
received
•Any stress is indication to increase
dosage

32. Phaeochromocytoma in special
circumstances
•Unsuspected encountered during surgery
•If warning signs go unheeded , patient
may develop cardiac failure ,pulmo.
Oedema, cardiac arrest
•Alpha blocked instituted immediately
and the procedure terminated
•Then patient should undergo full Ix ,
preparation and localization prior to
excision of tumor at a later date

33. Malignant phaeochromocytoma
•Occur in 10% of phaeochromocytoma , rises to
40-50% in extra adrenal tumors
•Surgical resection remains principal therapeutic
measure
•Where possible recurrent disease should be
resected , debulked or ablated
•Chemotherapy not very effective
•Radiotherapy useful palliation particularly in
bony metastases
•Survival 35-40% at 5 year

34. IN PREGNANCY
•In pregnancy a potentially lethal condition
maternal mortality -40%,fetal mortality-40-56%
•May be mistaken for pre-eclampsia
•Catecholamine excess should be excluded in all
hypertensive pregnancy
•Mortality reduced greatly when diagnosed prior
to onset of labour -hypertensive paroxyms ppt.
by uterine contraction, anaesthesia or operation

35. CONT.
•Alpha and beta blockade 2-3 days pre-
operatively in 1
st
and 2
nd
trimester
surgical excision may be done
•In 3
rd
trimester caesarean section
followed by removal of tumor
•under same anaesthetic
•Vaginal delivery should be avoided at all
costs

36. In children
•When occurs in children there is
increased incidence of B/L, multiple and
extra-adrenal tumors --association wit
MEN 2 syndrome
•In contrast to adult tumor are less
commonly malignant and hypertension
more often sustained
•Surgical resection is usually followed by
excellent clinical result

37. Phaeochromocytoma and MEN -2A
and MEN -2B
•Multiple endocrine neoplasia (MEN) is a
syndrome characterized by medullary
thyroid cancer and phaeochromocytoma
•In MEN 2A variant primary
hyperparathyroidism also associated
•In MEN 2B , Patient have characteristic
facies , marfinoid habitus , skeletal
abnormalities and mucocutaneous
ganglioneuromas

38. CONT.
•Ggerm line mutation in RET proto-
oncogene are responsible for MEN 2A
and MEN 2B
•Gene is inherited as autosomal dominant
fashion with high penetrance and
variable expression
•All MEN 2 will develop MCT but
phaeochromocytoma variable

39. CONT.
•In MEN 2 when adrenal disease develop –
B/L, passing through a phase of
hyperplasia, to nodularity and multiple
phaeochromocytomas
•Confirm through measurement of urinary
catcholamines and their matabolites
•Once disease confirmed B/L
adrenelectomy should be performed

40. NEUROBLASTOMA
•Extra cranial solid tumor of childhood
•Occurs 1 in 10,000 live birth
•Originates in adrenal medulla or anywhere
in sympathetic nervous system
•Mostly sporadic
•Account for at least 15% of all childhood
cancer death
•25-30% arises in adrenal medulla

41. CONT.
•Minute nodule to large mass >1kg
•Insitu 40 times more frequent than overt
tumor
•Great proportion spontaneously
regresses leaving only a focus of fibrosis
or calcification
•Can sharply demarcated to infiltrative

42. CONT.
•On transsection soft , grey , brain like
tissue
•Larger tumor shows areas of necrosis ,
cystic softening , foci of calcification
•Histo.–small primitive cells with dark
nuclei , scant cytoplasm and poorly
defined cell border growing in solid or
sheets

43. CONT.
•Rosettes (Homer-Wright pseudo rosettes)
in which tumor cells are arranged about a
central space filled with fibrilar extension
of cells

44. C/F
•50% children present to hospital less
than 1 year age, 80-90% by 3 year
•Abdominal mass firm to hard , nodular
and fixed
•Sick , weight loss fever , abdominal
distension and anemia

45. Investigation
•VMA passed in urine
•X ray abdomen –fine stippled calcification
•X ray chest --to rule out secondary
•USG and CT can define mass , nature
and extent and can detect hepatic
metastasis
•MRI

46. TREATMENT
•Surgical excision mainstay of t/t
•Complete excision not possible tumor
debulking followed by radiotherapy and
or chemotherapy
•Prognosis <2 year age –2 year survival
77%
>2 year age –2 year survival
38%

47. CONT.
•Stage 1 --2 year survival 100%
•Stage 2--2 year survival 82%
•Stage 3 --2 year survival 42%
•stage 4 --2 year survival 30%

48. Ganglioneuroma
•Relatively benign
•Symptomless
•Grows to large size
•One of variety of retroperitoneal sarcoma
•Only -15% involve adrenal gland
•Rest along sympathetic chain
•Removed completely cure may be
expected

49. Adrenocortical neoplasm
•Adrenocortical adenoma-well
circumscribed, nodular 1-2.5 cm
•Expand aderanal
•Some large size shows area of
hemorrhage ,cystic degeneration and
calcification
•Capsule may be deficient at places

50. CONT.
•Functional adenoma –cortex atrophy
•Non functional adenoma –cortex normal
thickness
•Cut section–yellow to yellow brown
nuclei small, some degree of
pleomorphism may be seen

51. Cont.
•Can present with c/f of
•Cushing syndrome-when adenoma
arises in zona fasciculata and secrete
excess of gucocorticoids
•conn’s syndrome –when adenoma
arises from zona gomerulosa and hence
hyperaldosteronism
•Virilizing and feminizing syndrome when
tumour arises from inner zona reticularis

52. CONT.
•After proper Ix to confirm diagnosis
definitive t/t –surgical resection of
affected adrenal gland should be done

53. Adrenocortical carcinoma
•Rare neoplasm
•10% of all cushing’s disease
•Peak age -4—5 decade of life
•May occur at any age
•More likely to be functional than adenoma
•Associated with c/f of hyperaderanalism
•Highly malignant
•Large when discovered many exceed 20 cm
diameter

54. CONT.
•Cut section-yellow , frequently have
hemorrhage , cystic and necrotic areas
•Encapsulated
•Histologically -mild degree of atypia to
wildly anaplastic neoplasm
•Strong tendency to invade adrenal vein ,
vena cava and lymphatic

55. CONT.
•Aggressive malignancy with high
recurrence rate
•Functional(60%) and non functional
•Clinical picture depend on hormone
secreted
•Cushing’s syndrome ,
hyperaldosteronism,virilization
feminization or mixed picture

56. CONT.
•Surgical resection –main t/t, but patient
usually present late stage 3, 4 hence
mostly palliative only
•Overall 5 year survival 16%
stage 1 and 2 -->50%
stage 4 --0%
•Mitotane –unlikely to improve overall
survival

57. INCIDENTALOMA
•Discovered by chance wile doing Ix –USG, CT,
MRI for some other pathology
•Silent adrenal mass in 0.5—4.5%
•For clinician a diagnostic problem
•Nonfunctioning tumor <5cm diam. Can usually
be left but monitored regularly
•Essential step establish whether functional or
not
•Increased risk of malignancy tumor >5cm, and
hence should be resceted

58. Adrenalectomy
•Most commonly done for benign
unilateral adrenal adenoma –functional
•B/L done for B/L adrenal tumor or
hypertensive state asso. With certain type
of cushing`s syndrome
•Close cooperation between endocrinology
and anesthesiology colleague

59. •CONT.
•Pheochromocytoma -achieve
normotension before operation with
medication in 2-4 wks period
•Preoperative fluid loading –NS/ Dextrose
-24 hrs preoperatively

60. •As rule treat solitary tumor by total
removal of affected adrenal gland
•Laparoscopic removal increasingly used
for small tumor
•Open operation for removal of malignant
tumor, large tumors, difficulty such as
adhesions following operation

61. •Bring patient to best possible condition
before operation
•Thromboprophylaxis during preoperative
peroid
•Preoperative antibiotics

62. Technique
•Laparoscopic approach –transperitoneal
-retoperitoneal
•Transperitoneal –Rt. Side
•Elevate side to be operated -sand bag
and table tilting to position patient half
way between true lateral and fully supine
•Create pneumoperitoneum using veress
needle

63. CONT.
•Create 4 or 5 ports situated in subcostal
space between midline and axillary fold
•Retract liver with rigid steel rod or
Nathanson retractor
•Carefully dissect off peritoneum overlying
tumor and vena cava using diathermy and
ultrasonic scalpel

64. CONT.
•Key points-avoid damaging vena cava
-confirm anatomy , particularly
relation of tumor to renal vessels
•Dissection continued between vena cava
and gland or tumor to identify main renal
vein
•3 occluding clips apply and divide vein
leaving 2 clips on caval side.

65. CONT.
•Continue dissection close to tumor and
divide all other vessels with diathermy
•Gently mobilize tumor , leaving diaphragm
intact and free from diathermy intact
•Now remove tumor within a bag through a
widened port site

66. Left side
•Reverse position on operating table,
otherwise approach is identical to that for
Rt. Gland
•Gland can be exposed two way
•1) Bring spleen forward with tail of
pancreas , approach gland between
spleen and diaphragm

67. CONT.
•2) After retracting spleen laterally ,gland
can be approached above pancreas
•In either approach spleenic flexure must
be free from colon to gain access

68. CONT.
•RETROPERITONEAL approach
•Semiprone,
•Insert ports posteriorly into retroperitoneal
fat just below costal margin
•Create artificial space
•Gland identified
•Control vessels

69. OPEN APPROACH
•popular approach for large tumor ,
malignant disease
•Affected side uppermost
•Incision over 11
th
rib dissect rib and
elevate it as dissection proceed
•cut rib costal cartilage , sweep pleura
and peritoneum
•Kidney identified , deep fascia incised
carefully fat dissected

70. CONT.
•Feel and see large tumor
•Handle gland carefully
•Avoid haste
•Safely ligate vein
•If damage to vena cava occurs repair with
proline 5-0

71. Posterior approach
•for small tumor 5—6cm
•Prone
•Horizontal incision over 11
th
rib
•Excise rib
•Dissect gland same as lateral approach

72. Anterior transperitoneal approach
•B/L Disease
•Rooftop (cheveron) incision or a midline
incision
•Approach rt. Gland –kocher’s maneuver to
mobilize duodenum and retract liver upward
•Lt. access by incising linorenal ligament to
release spleenic flexure then incise lateral
colonic reflection , sweep viscera forward

77. Thank you