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AdrenocorticalCa.pptx

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AdrenocorticalCa.pptx

  1. 1. Tips on using my ppt. 1. You can freely download, edit, modify and put your name etc. 2. Don’t be concerned about number of slides. Half the slides are blanks except for the title. 3. First show the blank slides (eg. Aetiology ) > Ask students what they already know about ethology of today's topic. > Then show next slide which enumerates aetiologies. 4. At the end rerun the show – show blank> ask questions > show next slide. 5. This will be an ACTIVE LEARNING SESSION x three revisions. 6. Good for self study also. 7. See notes for bibliography.
  2. 2. Learning Objectives
  3. 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. 4. Introduction & History. •
  5. 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. 6. Aetiology
  7. 7. Aetiology • Idiopathic • Congenital/ Genetic • Nutritional Deficiency/excess • Traumatic • Infections /Infestation • Autoimmune • Neoplastic (Benign/Malignant) • Degenerative / lifestyle • Iatrogenic • Psychosomatic • Poisoning/ Toxins/ Drug induced
  8. 8. Aetiology of Aetiology •
  9. 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. 10. Etiology
  11. 11. Etiology • Congenital • Traumatic • Infections /Infestation • Autoimmunity • Neoplastic (Benign/Malignant) • Degenerative
  12. 12. Etiology • mutations of theTP53 and TP57 genes (both antioncogenes) • increased production of insulinlike growth factor 2 (IGF-2).
  13. 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. 14. Pathophysiology
  15. 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. 16. Pathophysiology • Non–glucocorticoid-mediated insulin resistance • Catecholamine excess due to rare instances of coexisting pheochromocytoma • Cachexia (usually preterminal)
  17. 17. Classification1
  18. 18. Classification1 • Functional • Non-functional • Well differentiated • Intermediate • Poorly differentiated to anaplastic
  19. 19. Classification2
  20. 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. 21. Clinical Features •
  22. 22. Clinical Features • Demography • Symptoms • Signs • Prognosis • Complications
  23. 23. Clinical Features: hormonally active AC
  24. 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. 25. Clinical FeaturesSigns
  26. 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. 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. 28. Clinical FeaturesSigns Feminization • Male -gynecomastia and hypertension; • Female – – precocious sexual development – hypertension.
  29. 29. Clinical Features: non-functional AC
  30. 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. 31. Demography
  32. 32. Demography • Incidence & Prevalence • Geographical distribution. • Race • Age • Sex • Socioeconomic status • Temporal behaviour
  33. 33. Demography • Incidence & Prevalence-
  34. 34. Demography • Geographical distribution.
  35. 35. Demography • Race.
  36. 36. Demography • Age
  37. 37. Demography • Sex
  38. 38. Demography • Socioeconomic status
  39. 39. Demography • Temporal behaviour
  40. 40. Signs
  41. 41. Signs • General Examination • Systemic Examination • Local Examination
  42. 42. Signs • General Examination
  43. 43. Signs • Systemic Examination
  44. 44. Signs • Local Examination
  45. 45. Prognosis
  46. 46. Prognosis • Morbidity • Mortality rate • 5 year survival in Malignancy
  47. 47. Investigations
  48. 48. Investigations • Laboratory Studies – Routine – Special • Imaging Studies • Tissue diagnosis – Cytology • FNAC – Histology – Germ line Testing and Molecular Analysis • Diagnostic Laparotomy.
  49. 49. Diagnostic StudiesLaboratory studies
  50. 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. 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. 52. Investigations in Malignancy •
  53. 53. Investigations in Malignancy • For diagnosis • For staging • For Screening • For Monitoring
  54. 54. Diagnostic Studies
  55. 55. Diagnostic Studies Imaging Studies • X-Ray • USG • CT • Angiography • MRI • Endoscopy • Nuclear scan
  56. 56. Management
  57. 57. Management:Surgical  Open or Laparoscopic Resection.  Pulmonary metastasectomy
  58. 58. Management of endocrine syndromes Cushing syndrome • Mitotane • Ketoconazole • Metyrapone • Aminoglutethimide • RU 486 (mifepristone) • etomidate,
  59. 59. Management of endocrine syndromes hyperaldosteronism • Spironolactone • eplerenone, • amiloride, • triamterene, • and various antihypertensives, especially long-acting dihydropyridine calcium channel blockers.
  60. 60. Management of endocrine syndromes Antiestrogens • Clomiphene citrate • Tamoxifen • Toremifene • Danazol
  61. 61. Management of endocrine syndromes antiandrogens • Flutamide • Cyproterone acetate • Bicalutamide (Casodex) • Nilutamide • Megestrol acetate • Ketoconazole, • spironolactone, • and cimetidine
  62. 62. Management of endocrine syndromes Hypertension • similar to pheochromocytoma- long-acting alpha blockers (usually phenoxybenzamine) • beta blockers (eg, propranolol) • metyrosine.
  63. 63. Management of endocrine syndromes • mixed carcinoma associated with pheochromocytoma components- • high-dose, radiolabeled metaiodobenzylguanidine (MIBG.
  64. 64. Management of endocrine syndromes • Patients treated with mitotane may present with features of both glucocorticoid and aldosterone insufficiency requiring replacement therapy..
  65. 65. Operative Therapy
  66. 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. 67. Medical Therapy:Chemo • Mitotane • Mitotane plus etoposide • streptozotocin • Cisplatin • Suramin • Gossypol
  68. 68. Radiation therapy
  69. 69. Radiation therapy • palliation of local disease, such as symptomatic metastases to the bone and local luminal obstructive disease. • radiotherapy to the tumor bed.
  70. 70. Get this ppt in mobile 1. Download Microsoft PowerPoint from play store. 2. Open Google assistant 3. Open Google lens. 4. Scan qr code from next slide.
  71. 71. Get this ppt in mobile
  72. 72. Get my ppt collection • https://www.slideshare.net/drpradeeppande/ edit_my_uploads • https://www.dropbox.com/sh/x600md3cvj8 5woy/AACVMHuQtvHvl_K8ehc3ltkEa?dl =0 • https://www.facebook.com/doctorpradeeppa nde/?ref=pages_you_manage

Editor's Notes

  • drpradeeppande@gmail.com
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