Lecture notes for medical students

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2. Learning Objectives

3. Learning Objectives
1. Introduction & History
2. Relevant Anatomy, Physiology
3. Aetiology
4. Pathophysiology
5. Pathology
6. Classification
7. Clinical Features
8. Investigations
9. Management
10. Prevention
11. Guidelines
12. Take home messages

4. Introduction & History.
•

5. Introduction .
• Adrenocortical carcinomas (ACs) are
uncommon malignancies that can have
protean clinical manifestations.
• A majority of cases are metastatic at the
time of diagnosis, with the most common
sites of spread being the local periadrenal
tissue, lymph nodes, lungs, liver, and bone.
• AC is relatively rare, however, accounting
for just 0.02-0.2% of all cancer-related
deaths.

6. Aetiology

7. Aetiology
• Idiopathic
• Congenital/ Genetic
• Nutritional Deficiency/excess
• Traumatic
• Infections /Infestation
• Autoimmune
• Neoplastic (Benign/Malignant)
• Degenerative / lifestyle
• Iatrogenic
• Psychosomatic
• Poisoning/ Toxins/ Drug induced

8. Aetiology of Aetiology
•

9. Aetiology of Aetiology
• Idiopathic
• Congenital/ Genetic
• Nutritional Deficiency/excess
• Traumatic
• Infections /Infestation
• Autoimmune
• Neoplastic (Benign/Malignant)
• Degenerative / lifestyle
• Iatrogenic
• Psychosomatic
• Poisoning/ Toxins/ Drug induced

10. Etiology

11. Etiology
• Congenital
• Traumatic
• Infections /Infestation
• Autoimmunity
• Neoplastic (Benign/Malignant)
• Degenerative

12. Etiology
• mutations of theTP53 and TP57 genes (both
antioncogenes)
• increased production of insulinlike growth
factor 2 (IGF-2).

13. Epidemiology
• Incidence 0.6-1.67 cases per million persons per
year.
• In southern Brazil, however, the incidence of
adrenal tumors is 10-15 times that of the general
population, a difference that has been associated
with a mutation in the P53 gene.
• The female-to-male ratio for ACs is approximately
2.5-3:1.
• AC occurs in 2 major peaks: in the first decade of
life and again in the fourth to fifth decades.

14. Pathophysiology

15. Pathophysiology
• 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

16. Pathophysiology
• Non–glucocorticoid-mediated insulin
resistance
• Catecholamine excess due to rare instances
of coexisting pheochromocytoma
• Cachexia (usually preterminal)

17. Classification1

18. Classification1
• Functional
• Non-functional
• Well differentiated
• Intermediate
• Poorly differentiated to anaplastic

19. Classification2

20. Classification2
• Sporadic and syndromic tumors
• The syndromic variants
– Gardner syndrome,
– Beckwith-Wiedemann syndrome
– multiple endocrine neoplasia type 1
– the SBLA syndrome (sarcoma, breast, lung,
and adrenal carcinoma
– Li-Fraumeni syndrome

21. Clinical Features
•

22. Clinical Features
• Demography
• Symptoms
• Signs
• Prognosis
• Complications

23. Clinical Features: hormonally active AC

24. Clinical Features: hormonally active AC
• Physical examination findings in patients
with hormonally active AC include the
following:
• Cushing syndrome
• Virilization -Hirsutism, facial acne,
oligo/amenorrhea, and increased libido
• Feminization (rare)
• profound weakness, hypertension, and/or
ileus from hypokalemia related to
hyperaldosteronism and hypoglycemia.

25. Clinical FeaturesSigns

26. Clinical FeaturesSigns
• palpable mass in the abdomen.
Cushing syndrome
• round face
• double chin
• buffalo-hump fat distribution
• generalized obesity
• failure of growth velocity
• hypertension.

27. Clinical FeaturesSigns
Virilization
• Findings in males --premature puberty with
enlargement of the penis and scrotum,
pubic hair, acne, and deepening voice.
• Findings in females- premature appearance
of pubic and axillary hair, clitoral
hypertrophy, acne, deepening voice,
premature increase in growth velocity, lack
of appropriate breast development, and lack
of menarche.

28. Clinical FeaturesSigns
Feminization
• Male -gynecomastia and hypertension;
• Female –
– precocious sexual development
– hypertension.

29. Clinical Features: non-functional AC

30. Clinical Features: non-functional AC
Any of the following:
• Fever
• Weight loss
• Abdominal pain and tenderness
• Back pain
• Abdominal fullness
• Symptoms related to metastases

31. Demography

32. Demography
• Incidence & Prevalence
• Geographical distribution.
• Race
• Age
• Sex
• Socioeconomic status
• Temporal behaviour

33. Demography
• Incidence & Prevalence-

34. Demography
• Geographical distribution.

35. Demography
• Race.

36. Demography
• Age

37. Demography
• Sex

38. Demography
• Socioeconomic status

39. Demography
• Temporal behaviour

40. Signs

41. Signs
• General Examination
• Systemic Examination
• Local Examination

42. Signs
• General Examination

43. Signs
• Systemic Examination

44. Signs
• Local Examination

45. Prognosis

46. Prognosis
• Morbidity
• Mortality rate
• 5 year survival in Malignancy

47. Investigations

48. Investigations
• Laboratory Studies
– Routine
– Special
• Imaging Studies
• Tissue diagnosis
– Cytology
• FNAC
– Histology
– Germ line Testing and Molecular Analysis
• Diagnostic Laparotomy.

49. Diagnostic StudiesLaboratory studies

50. Diagnostic StudiesLaboratory studies
• Serum glucose
• Serum cortisol
• Serum adrenal androgen
• Urine adrenal hormone
• Urine vanillylmandelic acid (VMA)
• Urine homovanillic acid (HVA) levels
• 1-mg dexamethasone suppression test
• 24-hour urinary cortisol excretion test.

51. Diagnostic Studies Imaging studies
• CT
• MRI
• The typical case is characterized by a large
unilateral adrenal mass with irregular edges.
The presence of contiguous adenopathy
serves as corroborating evidence.
• Histopathology

52. Investigations in Malignancy
•

53. Investigations in Malignancy
• For diagnosis
• For staging
• For Screening
• For Monitoring

54. Diagnostic Studies

55. Diagnostic Studies
Imaging Studies
• X-Ray
• USG
• CT
• Angiography
• MRI
• Endoscopy
• Nuclear scan

56. Management

57. Management:Surgical
 Open or Laparoscopic Resection.
 Pulmonary metastasectomy

58. Management of endocrine syndromes
Cushing syndrome
• Mitotane
• Ketoconazole
• Metyrapone
• Aminoglutethimide
• RU 486 (mifepristone)
• etomidate,

59. Management of endocrine syndromes
hyperaldosteronism
• Spironolactone
• eplerenone,
• amiloride,
• triamterene,
• and various antihypertensives,
especially long-acting dihydropyridine
calcium channel blockers.

60. Management of endocrine syndromes
Antiestrogens
• Clomiphene citrate
• Tamoxifen
• Toremifene
• Danazol

61. Management of endocrine syndromes
antiandrogens
• Flutamide
• Cyproterone acetate
• Bicalutamide (Casodex)
• Nilutamide
• Megestrol acetate
• Ketoconazole,
• spironolactone,
• and cimetidine

62. Management of endocrine syndromes
Hypertension
• similar to pheochromocytoma-
long-acting alpha blockers (usually
phenoxybenzamine)
• beta blockers (eg, propranolol)
• metyrosine.

63. Management of endocrine syndromes
• mixed carcinoma associated with
pheochromocytoma components-
• high-dose, radiolabeled
metaiodobenzylguanidine (MIBG.

64. Management of endocrine syndromes
• Patients treated with mitotane may
present with features of both
glucocorticoid and aldosterone
insufficiency requiring
replacement therapy..

65. Operative Therapy

66. Medical Therapy
• Adjunct to surgery.
• Treatment of endocrine excess syndromes
• Use of mitotane or several multiagent
chemotherapy regimens
• Treatment and prevention of potential
complications
• Strategies for palliative and terminal care
issues, including symptom relief and
management

67. Medical Therapy:Chemo
• Mitotane
• Mitotane plus etoposide
• streptozotocin
• Cisplatin
• Suramin
• Gossypol

68. Radiation therapy

69. Radiation therapy
• palliation of local disease, such as
symptomatic metastases to the bone
and local luminal obstructive disease.
• radiotherapy to the tumor bed.

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