Endocrine 2

  1. What's found in MEN I?
    Neoplasms in: pancreas; parathyroid; pituitary
  2. What's found in MEN IIA?
    • pheocrhomocytoma; Parathyroid adenoma; Medullary carcinoma
    • RET
  3. What's found in MEN IIB?
    • Pheochromocytoma; medullary carcinoma; mucocutaneous GNs; marfainoid
    • RET
  4. What's common in VHL syndrome?
    • Pheochromocytoma; RCC; Hemangioblastoma; Pancreatic Neoplasm
    • VHL
  5. What's common in Neurofibromatosis I?
    • Pheochromocytoma; Neurofibromatosis; Cafe-au-lait spots; Optic nerve glioma
    • NF1
  6. Where's a pheochromocytoma found?
    Adrenal medulla
  7. What's the histological pattern of pheochromocytoma?
    Zellballen Pattern: nesting pattern of cells surrounded be thin septae
  8. What staining is used for Pheochromocytoma?
    Dichromate Solution --> brown color
  9. What's the 10% rule with Pheochromocytoma?
    10% bilateral; extra-adrenal; nonfunctional; malignant25% familial (MEN IIA/IIB)
  10. What's the lab findings from Pheochromocytoma?
    24hr urine collection - free catecholamines and metabolitesVanillylmandelic Acid (VMA); metanephrine (MN); free catecholamines (UFC)
  11. How do you differentiate benign vs. mallignant Pheochromocytoma?
  12. What familial syndromes are associated with pheochromocytoma?
  13. What are clinical symptoms of prolactinoma?
    Dysmenorrhea; galactorrhea; decreased libido in men
  14. What are clinical symptoms of pituitary adenomas?
    pituitary compression/errosion; optic chiasm impingement (Bilateral Hemianopsia); ↑ ICP (HA & n/v)
  15. What are the most common pituitary adenoma types?
    • #1 Prolactinoma
    • #2 GH tumor
  16. What are complications of acromegally?
    Insulin insensitivity --> DM #2
  17. What are functional syndromes associated with pituitary adenomas?
    Cushing's disease (ACTH); hyperthyroidism (TSH)
  18. What are causes of hypopituitaryism?
    Tumor (compression); surgery; Rathkecleft cyst; apoplexy; necrosis; sheehan syndrome
  19. What is a cellular charactersitic of pituitary adenoma?
    Mononuclear neoplasm (usually pleomorphic); lack of reticulin freamework
  20. What are clinical symptoms of Acromegally?
    • Growth of maxilla; mandible; nose; hands; feet
    • Neurological; cardiovascular; cerebrovaccular; respiratory; metabolic issues
  21. What are causes of ambiguous genitalia?
    • XX: increased androgen production
    • XY: Decreased androgen production(androgen insensitivity syndrome)
  22. What are causes of increased androgen production?
    • Decreased cortisol production
    • loss of negative feedback from cortisol
  23. What's the consequence of 21 HO deficiency?
    Aldosterone/cortisol production block; testosterone excess↑ACTH; testosterone; HOTN; shock; death
  24. What are the consequences of 17 HO deficiency?
    • Decreased cortisol; androgen; estrogen
    • Increased aldosterone (HTN)
  25. What's the consequence of 11-HO deficiency?
    • Excess deoxycorticosterone & androgen; lack of cortisol
    • HTN and virulism
  26. What's the cause of ulcers in the jejunum?
    Zollinger Ellison syndrome - gastrin secreting tumor
  27. What is the clinical presentation for Insulinoma?
    Seizures; drowsiness; tremors; palpitations (epi)
  28. What are the clinical symptoms of glucagon secreting tumors?
    DM; erythematous skin rash; thromboemolisms; hypoaminoacidemia
  29. What are the clinical symptoms of a somatostatin secreting tuomr?
    (GH secreting): DM; gallblasdder disease; steatorrhea; hypochorhydria
  30. What's the prognosis for islet tumors?
    • Insulin (B) - benign
    • Glucagon (A) - malignant
  31. What are clinical features of Diabetes Mellitus?
    Polyuria; polydipsia; weight loss (1) gain (2); blurry vision;
  32. which type of DM has amyloid deposits?
    Type 2
  33. Which type of DM is autoimmune?
    Type 1
  34. What are clinical symptoms of VIPoma?
    Wattery diarrhea; hypokalemia
  35. What are charactersitics specific to DM 1?
    Childhood onset; nomal weight; autoimmune; DKA; Inflammatory infiltrate (T/macrophages); b-cell depletion/atrophy
  36. What are charactersitics of DM2?
    adult onset; obese; increased --> decreased insulin
  37. What are Kimmelsteil Wilson Bodies and when are they found?
    Nodular glomerulosclerosis as a complication of DM
  38. What are nephrotic complications of DM?
    Nodular glomerulosclerosis; BM thickening; Kimmelsteil Wilson Bodies
  39. What are the long term complications of Diabetes?
    Arteriosclerosis; Hyalinization; NASH; vessel permeability (leaky)
  40. What is the most common type of pediatric tumor?
    • Total = leukemia
    • Solid tumor = CNS (neuroblastomas)
  41. What are lab findings in Neuroblastomas?
    Urine vanillylmandelic acid & homocanillic acid
  42. What are common histological findings of Neuroblastoma?
    Small blue cells; Pseudorosettes; necrosis; calcification; ganglioneruromas
  43. What are positive prognostic factors for neuroblastoma?
    • Presentation <12 y/oStage 1;2;4S (< 1yr)
    • Non-adrenal; low mitoses; non N-myc
  44. What are signs that neuroblastoma has metastasized to the eyes?
    Panda/Raccoon eyes (black)
Card Set
Endocrine 2
Lab Notes