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97 Cards in this Set
- Front
- Back
New name for small cellcarcinoma of thyroid
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malignant lymphoma
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All small cell tumors are either malignant lymphomas, _________, or variations of a malignant neuroendocrine carcinoma
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medullary carcinomas
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Lymphomas are MALTomas, lymphomas of ___ ___ __ __
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mucosa-associated lymphoid tissue
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Indolent tumors that remain localized, have “homing properties”, i.e., spread to ______
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other mucosa-lined organs
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Like all lymphomas of mucosa-associated lymphoid tissue, designated as _______
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marginal zone lymphomas
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Normal Adrenal Cortex
Three zones: |
Three zones:
Zona reticularis: androgens Zona fasiculata: cortisol Zona: glomerulosa: aldosterone |
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What chemical tells the adrenal gland to make cortisol
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ACTH
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_______ syndrome: excess cortisol
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Cushing
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_____ disease: excess aldosterone
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Conn
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______ syndrome: excess androgen
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Adrenogenital
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Causes of
Hypercorticolism (Cushing Syndrome) |
Hypothalamic and/or pituitary causes
Adrenal causes: cortical hyperplasia, adenoma, carcinoma Ectopic ACTH,: non-endocrine neoplasms, like small cell carcinoma of lung |
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Cushing disease, in contrast to Cushing syndrome, is hypercorticolism caused by _______
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pituitary adenoma
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Cushing syndrome, in contrast to Cushing disease, is due to _______
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disease intrinsic to adrenal cortex
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Hypercorticolism (Cushing Syndrome):
In adults, carcinomas and adenomas, equal in incidence In children, _____ dominate |
carcinomas
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Cortical ________, secrete more cortisol than adenomas or hyperplasias; opposite gland, atrophic; elevated cortisol, depressed ACTH
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carcinomas
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Adrenal Cushing: elevated serum cortisol, low serum _____.
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ACTH
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Ectopic secretion of ACTH: most common cause is _____
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small cell ca of lung
(Other tumors: medullary thyroid ca, carcinoid and islet cell tumors) |
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Other tumors, secrete _____, stimulate hypothalamus, stimulate pituitary gland, secrete ACTH
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CRH (corticotrophin releasing hormone)
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(Cushing Syndrome)
Hyperplasia of adrenal gland: diffuse or nodular, 60-70%, most due to ____ |
ectopic or pit ACTH
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Cushing SyndromeClinical Features
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Lipid:truncal obesity, buff hump
Glucose: hyperglycemia, pouly dypsia, glucosuria Protein:fragile skin and bv, decr muscle Suppressed immunity Psychosis Hirsutism Menstrual abnormalities Hypertension |
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Cushing Syndrome Laboratory Findings
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Increased 24 hour urine free cortisol levels
Loss, diurnal pattern, cortical secretion, normally has two peaks, one day, one at night ACTH level, measure urinary cortisol after ACTH and after dexamethasone |
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Cushing Syndrome & Disease, Laboratory Findings
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Pituitary Cushing disease: ACTH elevated, decrease (ACTH) after dexamethasone in high doses
Ectopic ACTH: elevated ACTH, no effect on ACTH by low or high doses of dexamethasone Cushing syndrome, adrenal tumor: low ACTH, suppressed pituitary by high levels of cortisol, no response to low or high doses of dexamethsone |
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Primary Aldosteronism Characteristics
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Excess aldosterone
Sodium retention (hypernatremia) Potassium excretion (hypokalemia) Hypertension Suppressed renin-angiotensin system = decreased renin |
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Primary AldosteronismCauses
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Adrenal cortical adenoma
Adrenal cortical hyperplasia 80% of cases, adenoma 20% of cases, hyperplasia |
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Primary aldosteronism caused by adenoma = ____
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Conn syndrome
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Primary Aldosteronism Clinical Features
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Mid-life
Female bias Hypertension Hypokalemia, renal wasting of K Low serum renin Non-specific symptoms of weakness, parasthesias, visual disturbances |
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Primary Aldosteronism Diagnosis
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Increased serum aldosterone
Depressed serum renin Hypokalemia Hypernatremia Hypertension |
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Determining The cause of primary aldosteronism, by Adrenal cortical hyperplasia or adrenal cortical adenoma, is very important because ______
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the treatment of adenoma is surgical and that for hyperplasia is medical.
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Secondary Aldosteronism Characteristics
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Activation of renin-angiotensin system
Increased serum renin |
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Secondary AldosteronismCauses
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Congestive heart failure
Decreased renal perfusion, as seen in: arteriolonephrosclerosis renal artery stenosis hypoalbuminemia pregnancy (estrogen-induced) increased plasma renin substrate |
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Small, yellow adrenal cortical adenoma is highly suggestive of ______ syndrome
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Conn
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Best definition of adenoma
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Removal of solitary nodule cures disease.
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defines carcinoma
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Finding of 3 of 12 histologic changes
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Adrenal cortical carcinoma
Difficult diagnosis to make Size, color, appearance, not helpful Evidence of invasive growth, metastases, confirms malignancy ___ of 12 histologic changes, including vascular, capsular invasion, when present, makes the diagnosis |
3
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Congenital Adrenal Hyperplasia: _______ Deficiency
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21-Hydroxylase
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Disorders of sexual differentiation, such as virilization, caused by adrenocortical neoplasms or congenital adrenocortical ______.
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hyperplasia
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Virilization caused by neoplasms are more commonly caused by adrenal cortical _______ than adrenocortical adenomas
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carcinomas
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21-Hydroxylase catalyzes production of 11-deoxycorticosterone from _____
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progesterone
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Congenital Adrenal Hyperplasia inheritance
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Autosomal recessive
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Congenital Adrenal Hyperplasia
Spectrum of deficiencies: |
1. simple virilizing adrenogenitalism
2. salt-wasting syndrome 3. non-classic adrenogenitalism |
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Simple Virilizing Adrenogenitalism:
Lack of 21-hydroxylase, causes lack of normal ____ |
cortisol
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Simple Virilizing Adrenogenitalism:
Reduced cortisol, increases ____ |
ACTH
(Stimulates androgens, virilizes ) |
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Congenital Adrenal Hyperplasia:
Defect, abnormal production cortisol, secondary to absence of appropriate enzymes for normal production of ____ |
cortisol
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Congenital Adrenal Hyperplasia:
Lack normal cortisol, increases ACTH, stimulates production _______ |
“cortisol”, androgens
Increased androgens, virilizes |
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Congenital Adrenal HyperplasiaTreatment
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Replace cortisol and restore normal feedback mechanism
Reduce production of androgens |
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Primary Adrenal Insufficiency
2 types |
1. Acute insufficiency
2. Chronic insufficiency (Addison's) |
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Most common causes of chronic primary adrenal insufficiency
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Autoimmune adrenalitis
Tuberculosis Amyloidosis Hypothalamic, pituitary origin Iatrogenic Hemochromatosis Sarcoidosis |
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Primary Chronic Adrenal Insufficiency
most common causes |
TB, most common in past
Infections: Histoplasma, most common fungus Metastatic neoplasms: most common, breast and lung, |
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Histology of adrenal insuff: autoimmune adrenalitis, cortex destroyed, medulla preserved, prominent infiltrate of _____
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lymphocytes (lymphocytic adrenalitis)
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Visible abnormality of Addison's pt
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increased melanin in skin
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Primary Acute Adrenocortical Insufficiency May occur in following clinical settings
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1- Acute crisis in chronic insufficiency
2- Acute withdrawal of steroids from patient 3- Massive bilateral adrenal cortical hemorrhage Anticoagulant therapy DIC in postoperative patients 4- Anticoagulant therapy 5- Bacteremia Acute insufficiency, secondary to bacterial infection, Waterhouse-Friderichsen syndrome. Classic case involves N. meningitidis, |
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Chronic Adrenal InsufficiencyClinical Features
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Non-specific symptoms: fatigue, weakness
GI: anorexia, diarrhea, weight loss Skin: pigmentation, increased ACTH precursors, stimulate melanocytes, seen only with primary adrenal insufficiency Hyponatremia, hypokalemia, due to hypoaldosteronemia |
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Differentiating Primary from Secondary Adrenal Insufficiency
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Low ACTH in primary form
Administration of ACTH: No increase in cortisol in primary form Prompt increase in ACTH in secondary form |
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Adrenal Medulla:
Cells are of neuroendocrine origin characterized by following immunohistochemical reactions: |
neuron-specific enolase: positive
chromogranin: positive synaptophysin: positive |
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Chromaffin (not chromogranin) reaction, positive: K-dichromate _____ color to tumor and cells
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brownish
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Neuroendocrine cells can secrete other polypeptides, such as ____
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histamine, serotonin, ACTH etc.
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Adrenal Medulla Cells: secrete ____
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epinephrine, norepinephrine (catecholamines); normally more epinephrine than norepinephrine
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Metabenzylguadinine, combined with radioactive iodine to yield ________
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metaiodobenzylguanidine (MIBG)
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MIBG combines with catachol amines to yield image of _____
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adrenal medulla
In case of tumors, MIBG combines with catachol amines to produce recognizable image |
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Adrenal Medulla:
Cells, similar to medullary cells, found in extramedullary sites (organ of Zuckerkandl, carotid and aortic bodies, ganglion nodosum of jugular area, urinary bladder) Cells in extramedullary sites are known as _______ |
paraganglion cells
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What problem:
Neoplasm of adrenal medulla Chromaffin cells Secretes catecholamines (norepinephrine, epinephrine) Neoplasm of neuroendocrine cells, stains like NE cells, produces polypeptides in addition to catecholamines (ACTH, serotonin etc) |
Adrenal MedullaPheochromocytoma
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Pheochromocytoma:
85%, arise in adrenal medulla 15%, extramedullary sites, paragangliomas Pheochromocytomas vs paragangliomas? (chromaffin) |
Pheochromocytomas, chromaffin positive
Paragangliomas, chromaffin negative |
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Genes Associated with pheochromocytomas
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multiple endocrine neoplasia IIa
multiple endocrine neoplasia IIb |
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what disease?
medullary thyroid carcinoma, unusual facies, mucosal neuromas, colonic ganglioneuromatosis, Marfanoid habitus, corneal nerve hypertrophy, Hirschsprung disease |
Multiple endocrine neoplasia IIb
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What disease?
pheochromocytomas, neurofibromatosis, café au-lait spots in skin, Schwannomas, meningiomas, gliomas |
von Recklinghausen syndrome: pheochromocytomas,
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What disease?
medullary thyroid carcinoma bilateral pheochromocytomas parathyroid hyperplasia (primary hyperparathyroidism) |
Multiple endocrine neoplasia IIa
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What disease?
pheochromocytomas, renal, pancreatic, hepatic and epididymal cysts |
von Hippel-Lindau syndrome
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What disease?
pheochromocytomas, cavernous hemangiomas of 5th cranial nerve |
Sturge-Weber syndrome:
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Familial pheochromocytomas are usually bilateral or unilateral?
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bilateral
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Diagnosis of Pheochromocytoma
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Bilateral pheos, more common in familial syndromes, 20-40%, are also malignant
Chromaffin reaction: exposure of tumor to k dichromate, brown color is positive reaction macroscopically & microscopically. EM, secretory granules Separation of malignant from benign forms difficult |
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Pheochromocytoma Clinical Features
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Hypertension, most prominent sign
Tachycardia, palpitations, headaches Cold sweats, tremors, anxiety, apprehension Paroxysmal hypertension Sudden release of catecholamines results in hypertension, CHF, pulmonary edema, MI, vent fib |
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Pheochromocytoma Laboratory Findings
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Increased free catecholamines or metabolites (vanillylmandelic acid, (VMA), metanephrines), in urine
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Pheochromocytoma Treatment
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: Surgical removal of tumor or tumors after careful preparation of patient
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Diagnosis of Paragangliomas
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Tumors of extramedullary neuroendocrine cells
Chromaffin reaction - negative Neuron-specific enolase, chromogranin, synaptophysin – positive EM - secretory granules. Many paragangliomas are malignant Patients, teens and twenties 1/10 as common as pheochromocytomas Usually multiple |
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What problem?
Another tumor of adrenal medulla & extradrenal sites Most common extracranial tumor of childhood Most common tumor, children <1 yr 15%, deaths from cancers in children 5 yr survival rate, greatly improved, 60% Most sporadic, some familial |
Neuroblastoma
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Genetic markers of neuroblastoma
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1p deletion
N-myc amplification DNA hyperploidy |
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Laboratory findings of neuroblastoma
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High urinary catecholamines/metabolites
EM - secretory granules Histology, stains like neuroendocrine tumor |
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Where is neuroblastoma?
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20-25%, adrenal medulla
2nd, paravertebral, post mediastinum 3rd, multiple sites, neck, pelvis, brain Gross: minute to large ( >1 kgm) |
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What does neuroblastoma look like microscopically?
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Micro: undiff, small, blue cell tumor with Homer-Wright pseudorosettes to well diff tumor with ganglia, fibrillary glia-like material
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Stages of neuroblastoma
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Stages I-IVS, S=special, small adrenal tumor , infiltrates in liver, skin, bone marrow, no bone destruction
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NeuroblastomaPrognostic Features
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Age, sex: <1 yr = excellent prognosis, regardless of stage, mets in liver, bone marrow.
80% 5 yr surv rate, minimal result More disease, 50% survival rate Stage I or II, <1 yr, 95-98% survival rate >1yr, stages III or IV, 10% survival rate |
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Parathyroid glands are Derived from ______ pouches, like thymus, therefore, not surprising to find ectopic glands within the thymus
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pharyngeal
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Parathyroid Dominant cell, _____ cell; other cells: clear cells, oxyphilic cells
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chief
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Hypocalcemia: low blood calcium, associated with what symptoms?
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muscle spasms, tetany & cardiac dysfunction
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Three major pools of calcium:
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1) Intracellular calcium: fluctuates; fluctuations caused by release of cellular stores or influx from extracellular fluid
Key to role in intracellular signaling, enzyme activation, muscle contractions 2)Blood : ~50% 0f blood calcium bound to proteins 3) Extracellular calcium: Concentration of ionized Ca, normal @ 1mM or 10,000 times concentration of free Ca in cells Concentration of P, same as Ca |
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Majority of phosphate (~85%) tied up in ____ phase; normal blood concentration of phosphate very similar to Ca
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mineral
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Frequent adjustments of Ca, described as fluxes between blood & other compartments, controlled by 3 organs:
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1) Small intestine: Ca absorbed from diet; dependent on calcium-binding protein in epithelial cells
2) Bone: vast reservoir of Ca; resorption of bone mineral releases Ca & phosphate into blood; reverse occurs to allow Ca to be deposited in bone 3) Kidney: normal conditions; ~all of Ca in glomerular filtrate resorbed in tubules; with decreased tubular resorption, Ca lost in urine |
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Parathyroid hormone: acts to increase blood Ca by:
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Increasing production of active form of
vitamin D Mobilizes Ca & phosphate from bone Eliminates phosphate (phosphaturic effect) Maximizes tubular resorption of Ca; minimal loss of Ca in urine |
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Vitamin D: increases Ca concentration in blood by:
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Facilitating absorption of Ca from small intestine
Acting with parathyroid hormone, enhances fluxes of Ca out of bone |
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Calcitonin: ?reduces or increases? blood Ca; secreted in response to hypercalcemia
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reduces
Suppresses renal resorption of calcium Inhibits bone resorption which minimizes fluxes of Ca from bone to blood |
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Considering that Ca is involved in modulating many physiologic processes, Ca can be thought of as a type of _____, and the calcium sensor as its receptor
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hormone
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The Extracellular Calcium-Sensing Receptor is member of ______ family of receptors
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G-protein coupled
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The Extracellular Calcium-Sensing Receptor Sensor is expressed in a broad range of cells, including parathyroid and thyroid C cells, controls synthesis and secretion of ______&______
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PTH & calcitonin
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best way to differentiate primary and secondary hyperparathyroidism?
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look at phosphate
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Primary hyperparathyroidism: intrinsic to parathyroid glands; increased secretion of PTH with resulting _____&______
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hypercalcemia & hypophosphatemia
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Secondary hyperparathyroidism: PTH resistance in target tissues: resulting in ____&_____
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hypocalcemia & hyperphosphatemia(?)
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Causes of primary hyperparathyroidism:
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1. Adenomas (single) (80%)
2. Parathyroid hyperplasia (5-20%) 3. Parathyroid carcinomas (2-3%) |