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