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12 Cards in this Set
- Front
- Back
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Cushing's Syndrome
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General: due to exogenous glucocorticoids, PRIMARY HYPOTHALAMIC PITUITARY DZ WITH HYPERSECRETION OF ACTH
Causes: primary adenaocortical hyperplasia or neoplasia, ectopic ACTH secretion by endocrine neoplasisa (small cell CA of lung, medullary CA of thyroid, pancreatic tumor) Morphology: exogenous glucocorticoids, inc ACTH by pituitary or ectopic source, primary adrenocortical neoplasms, gland = COOKE'S HYALINE CHANGE (basophilic cells); hypothalamic pituitary origin Adrenal = suppresses low NOT high dexamethasone dose (opposite for adrenal) |
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Primary hyeraldosteronism
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Clinical: Na,K retention, HTN, hypOkalemia, ABSENCE OF INCREASED RENIN LEVELS
Cause: idopathic hyperaldosteronism associated wtih nodular hyperplasia; adrenocrotical neoplasms (Conn's syndrome); glucocorticoid remediable hyperaldosteronism, uncommon familial cause Morphology: ALDOSTERONE SECRETING ADENOMA; solitary, small, bright yellow uniform cells; spironolactone = "SPIRONOLACTONE BODIES" --> laminated cytoplasm inclusions Adenomas DO NOT suppress ACTH secretion B/L idiopathic hyperplastic marked diffuse and focal hyperplasia |
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Secondary hyperaldosteronism
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Cause = RESPONSE TO RENIN ANGIOTENSIN SYSTEM
Morphology: INCREASED PLASMA RENIN LEVELS; dec renal perfusion causes arteriolar nephrosclerosis and renal a stenosis; arterial hypovolemia and edema = HF, cirrhosis, nephritis syndrome; preg causes = estrogen induced increase in plasma renin substrate |
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Adrenogenital syndromes
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Cause: CONGENITAL ADRENAL HYPERPLASIA
General: defects in enzymes involved in cortisol synthesis = 21 HYDROXYLASE DEFICIENCY Morphology: inc secretion of ACTH and dec synthesis of testosterone; ACTH regulates adrenal formation |
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Primary acute adrenocortical insufficiency
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Cause:
rapid withdrawal of steroids failure to inc steroid doses during stress massive adrenal hemorrhage - newborns DIC pts on anticoags bacteremic infection WATERHOUS FRIDERICHSEN SYNDROME |
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WATERHOUSE FRIDERICHSEN SYNDROME
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Overwhelming infl caused by:
N. MENINGIDITIS, pseudomonas, pneumococci, H. flu Rapidly progressive hypOtension and shock; DIC; rapidly develping adrenocortical insufficiency with massive b/l adrenal hemorrhage KIDS adrenals form sacs of blood clinical course = abrupt and devastating |
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Primary Chronic adrenocortical insufficiency "ADDISON'S DISEASE"
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Morphology: AI adrenalitis (irregular shrunken glands); TB/fungi (granulomatous inflam); met neoplasms (adrenal enlargement with infiltrating neoplasms)
Clinical: weakness, fatigue, n/v, diarrhea, wt loss, hyperpigmentated skin (inc ACTH precursor hormone stims melanocytes); hyperkalemia; hypOnatremia, vol depletion; hypOtension |
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Pheocrhomocytoma
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Clinical: age 30-40
Rule of 10: inc malig if SDHB, SDHB, SDHD ==> loss of gene function --> stabilization of HIF1alpha and induced tumorgenesis Gross: well demarcated, well vascularized, hemorrhage, necrosis, cyst formation Histo: no feature predict clinical behavior; bizarre morphology --> nuclear pleo, giant cells, mitotic figures, capsular invasion, vascular invasion aggressive tumors ==> cellular monotony |
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Neuroblastoma
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Clinical: Kids <5, location (adrenal or adjacent retroperitonal area; posterior mediastinum -- 2nd most common)
Gross: solid tumors, lg, soft, necrosis, hemorrhage Micro: sm cells in solid sheets or small nests HOMER WRIGHT PSEUDOROSETTES (cells gather around tangled eosinophilic fibrils), 90% produce catecholamines |
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MEN I (multiple endocrine neoplasm)
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Werner's syndrome
Involves Ab's in Parathyroid: hyperplasia + adenomas Pancreas: gastrinomas, insulinomas, microadenomas Pituitary gland: prolactinomas, somatotroph secreting tumor Cause: germline mutations in MEN1 tumor suppressor gene - encodes for MENIN - gene transcription Clinical: hypoglycemia, peptic ulcers, nephrolithiasis |
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MEN II or IIa (multiple endocrine neoplasia)
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Sipple syndrome
pheochromocytoma medullary carcinoma of thyroid parathyroid hyperplasia linked to the RET PROTO ONCOGENE |
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MEN III or IIb (multiple endocrine neoplasms)
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Medullary carcinoma of the thyroid
more aggressive than MENIIa pheochromocytoma DOES NOT have primary hyperparathyroidism Ganglioneuromas of skin, respiratory tract and GIT MARFANID HABITUS |
