This document discusses adrenal anatomy, physiology, and diseases. It covers adrenal tumors including adenomas, adrenocortical carcinoma (ACC), and primary adrenal lymphoma. ACC is typically larger than 5 cm, irregular, and shows necrosis/calcification. Diagnosis involves imaging like CT/MRI and biochemical testing. Treatment of ACC involves surgery with the goal of a complete resection followed by mitotane therapy. Prognosis depends on stage, resection margin, and markers like estrogen receptor status. Primary adrenal lymphoma is rare but has a better prognosis than ACC. Radiotherapy may be used for local recurrence. Ongoing research evaluates new adjuvant therapies.

1. Adrenals and
Adrenal carcinoma
Review
Dr Shankar Ram HS
Department of Genito Urinary Surgery
TDMCH .

5. • Cortical vessel
• Medullary vessel
• Capsular vessel – Cortical- Medullary capillary
sinusoids – medullary vein
• Medulla – Dual supply
• Pericapsular adrenal arterial Plexus – emissary
vein – Central vein

6. Medulla - -- sympathetic ganglion
Chromaffin cells – post ganglionic symp neurons w/o axons &
dentrites

8. Adrenal rests 1 %
• Path of gonadal descent and celiac axis
• CAH- p/w palpable testes
• Compensatory hypertrophy

9. GFR ACE

12. • 29% of adenomas have attenuation values
higher than 10 HU (hyperattenuating) and
remain indeterminate (5).
• Chemical shift MR imaging can be performed
rapidly without contrast material injection
• strong correlation between the degree of
signal intensity (SI) decrease on out-of-phase
MR images and CT attenuation

13. • washout CT has excellent diagnostic
performance for hyperattenuating adrenal
adenoma
• lipid-poor adenomas with attenuation higher
than 30 HU at unenhanced CT are not well
diagnosed with chemical shift MR imaging

18. Cushigns syndrome
• Hypercortisolism secondary to excessive
production of glucocorticoids by adrenal cortex
• Exogenous ACTH dependent and ACTH
independent
• ACTH-independent hypercortisolism, on the
other hand, results from unregulated
overproduction of glucocorticoids by the
adrenal(s) and is relatively rare.
• Up to 50% of
• patients with Cushing syndrome exhibit
urolithiasis;

21. Measurement of ACTH
• Serum levels
• Inferior Petrosal sinus levels after CRH
stimulation
• Low serum levels –ACTH independent Etiology
–Abd imaging

24. • LD DST -+ Only tells there is hypercortisolism
• (Overnight and standard).
• HD DST for cushings disease .- outdated

26. Dexamethasone Suppression Test
• http://emedicine.medscape.com/article/2114
191-overview

27. Bilateral adrenalectomy
• AIMAH
• PPNAD
• Ectopic ACTH syndrome
• Failure of Pit adenoma Rx

28. Primary Aldosteronism

33. PAC/PRA =ARR > 30 – screening
PAC > 20
• Conformatory - Autonomous aldoesterone cannot
be supressed
• Fludorocortisone supression( test upright )PAC>6
• Oral salt loading test (PAC>12)
• IV saline test (PAC>5-10)
• Captopril supression test (PAC>15)

44. Adrenals Tumors

47. • Rare
• Unilateral
• Rarely bilateral
• 5 the decade (60% functional) C+
• Children – low stage functional
(even small size) V+
• local periadrenal tissue, lymph nodes,
lungs, liver, and bone
• Syndromes – BW (IGF) , LF (p53)

48. Renal displacement
Camoflague

49. ACC –C T
• Irregular borders,
• irregular enhancement,
• calcifications, and necrotic areas with cystic
degeneration.
• Mean attenuation on non contrast CT scan in
ACC is significantly higher (39 HU)
• The presence of contiguous adenopathy
serves as corroborating evidence
• Thrombus

51. ACCs
• Larger than benign adrenal tumors,
• Average size of 10 to 12 cm on presentation.
• Indeed over 90% of ACCs are larger than 5 cm
• numerous unnamed collateral vessels
associated with large adrenocortical
carcinomas.
• tumoral capsule,
• a stellate zone of central necrosis

52. MRI
• Venous thrombus
• Iso-T1 (Liver spleen)
• Hyper – T2
• Marked uptake
• Delayed washout

53. • NO Biopsy

54. Symptoms
• Size
• Stage
• Function

56. • Virtually all feminizing adrenal tumors in men
are malignant.

57. Endocrine syndromes associated with
AC include the following:
• Cushing syndrome (30%)
• Virilization and precocious puberty (22%)
• Feminization (10%)
• Primary hyperaldosteronism (2.5%)
• Combined hormone excess (35%)
• Polycythemia (< 1%)
• Hypercalcemia (< 1%)
• Hypoglycemia (< 1%)
• Adrenal insufficiency (particularly from primary adrenal lymphomas)
• Non–glucocorticoid-mediated insulin resistance
• Catecholamine excess due rarely coexisting pheochromocytoma
• Cachexia (usually preterminal

59. Pre op
• Cortisol excess -inhibition with ketoconazole,
mitotane, or metyrapone
• Treatment for appropriate coagulopathies
• Serum potassium levels should be corrected in
hyperaldosteronism
• hypertension control should be initiated.

60. Open Adrenalectomy
• Masses that are greater than 6 cm have a rate
of adrenocortical carcinoma of 25% and
should be managed surgically.
• gold standard for masses suspected of benign
adrenal carcinoma
• Cytoreductive

61. Pathiological evaluation

63. IHC
• Insulinlike growth factor–2 (IGF-2) and Ki-67
overexpression identified ACs with 96% sensitivity and
100% specificity
• adrenocortical cells stain positive for D11, SF-1
• Adrenomedullary tumors positive for neuroendocrine
markers eg, synaptophysin, NSE,Chromogranin A),

64. ACCs
• weight of more than 500 g,
• presence of areas of calcification or necrosis,
• grossly lobulated appearance.
• numerous mitoses,
• scant cytoplasm, and
• none of the rosettes observed in
neuroblastoma.

66. Prognostics
• Disease stage at diagnosis
• Completeness of resection at surgery
• metastasis at the time of diagnosis
• Estrogen receptor (ER)–negative –Poor Prognosis
• overall five-year survival 20-35%

67. Recognition of primary adrenal
lymphoma
• Recognised as distinct from adrenocortical
carcinoma
• associated with a better prognosis than AC,
• Very rare .
• potential roles for standard lymphoma
treatment using multiagent chemotherapy
and radiotherapy.

68. Follow up
• For low-grade tumors, imaging every 3-6 months
and biomarkers (if Active)
• For high-grade tumors, -adjuvant therapy
• Mets- Low grade - adrenalectomy if 90% of tumor
can be removed surgically;
• follow-up should be oriented to the residual
disease (Thoracotomy /Lobectomy in selected pts).
• Survival reported > 10 years

69. Mitotane
• Relatively specific adrenocortical cytotoxin
• It is used as primary therapy, as adjuvant therapy, and
as therapy in recurrent or relapsing disease..
• adrenal inhibition without cellular destruction.
• controls endocrine hypersecretion in 70-75% pts.
• Tumor response correlate with serum levels .
• Months of continuous therapy.
• efficacy at least 15 mcg/mL.
• The potential benefit of postoperative adjuvant therapy
with mitotane is still controversial.

70. (FIRM-ACT)
The First International Randomized Trial in Locally Advanced and Metastatic
Adrenocortical Carcinoma Treatment study
• mitotane and etoposide *
Vs
• mitotane and streptozocin
First line therapy had higher response rates and
longer median progression-free survival than
patients treated with (5 mo vs 2.1 mo, respectively)
*

71. • Sumarin
• Gossypol
• Cisplatin
• Doxorubicin
• Long acting alpla and beta blockers
• Antiandrogens
• Antiestrogens
• New molecules

72. Radiotherapy
• If Local recurrence is expected
• Adjuvant RRx to tumor bed
• 40 gray (Gy), # 1.8-2 Gy
• (including a boost volume to reach from 50-60
Gy in individual patients)

73. Thoracoabdominal Approach

90. Video
• https://www.youtube.com/watch?v=smgB0nO7KTs
(open )
• https://www.youtube.com/watch?v=XJoiYDpcf4c lap
• https://www.youtube.com/watch?v=5s3JHZF2uTY L
Thoraco abdominal nephrectomy
• https://www.youtube.com/watch?v=wZFC8kShzFE
• https://www.youtube.com/watch?v=xC50mVH2RSE

91. https://www.youtube.com/watch?v=U
NGRer0v7o0

94. R0

100. Open Adrenalectomy
• First Resection is the best Resection
• Lnymphadenectomy is absolute
• Nothing Short of R0 Resection for best results

106. References
• Campbell and Walsh 11 e
• SAGES symposium - youtube
• NCCN
• E medicine
• Guidelines for the management of the incidentally discovered
adrenal mass- Anil Kapoor .Canadian Urology association
https://www.cua.org/themes/web/assets/files/guidelines/en/gu
idelines_for_the_management_of_the_incidentally_discovered.
pdf
AACE/AAES Guidelines
https://www.aace.com/files/adrenal-guidelines.pdf