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97 Cards in this Set

  • Front
  • Back
New name for small cellcarcinoma of thyroid
malignant lymphoma
All small cell tumors are either malignant lymphomas, _________, or variations of a malignant neuroendocrine carcinoma
medullary carcinomas
Lymphomas are MALTomas, lymphomas of ___ ___ __ __
mucosa-associated lymphoid tissue
Indolent tumors that remain localized, have “homing properties”, i.e., spread to ______
other mucosa-lined organs
Like all lymphomas of mucosa-associated lymphoid tissue, designated as _______
marginal zone lymphomas
Normal Adrenal Cortex
Three zones:
Three zones:
Zona reticularis: androgens
Zona fasiculata: cortisol
Zona: glomerulosa: aldosterone
What chemical tells the adrenal gland to make cortisol
ACTH
_______ syndrome: excess cortisol
Cushing
_____ disease: excess aldosterone
Conn
______ syndrome: excess androgen
Adrenogenital
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
Cushing disease, in contrast to Cushing syndrome, is hypercorticolism caused by _______
pituitary adenoma
Cushing syndrome, in contrast to Cushing disease, is due to _______
disease intrinsic to adrenal cortex
Hypercorticolism (Cushing Syndrome):
In adults, carcinomas and adenomas, equal in incidence
In children, _____ dominate
carcinomas
Cortical ________, secrete more cortisol than adenomas or hyperplasias; opposite gland, atrophic; elevated cortisol, depressed ACTH
carcinomas
Adrenal Cushing: elevated serum cortisol, low serum _____.
ACTH
Ectopic secretion of ACTH: most common cause is _____
small cell ca of lung


(Other tumors: medullary thyroid ca, carcinoid and islet cell tumors)
Other tumors, secrete _____, stimulate hypothalamus, stimulate pituitary gland, secrete ACTH
CRH (corticotrophin releasing hormone)
(Cushing Syndrome)
Hyperplasia of adrenal gland: diffuse or nodular, 60-70%, most due to ____
ectopic or pit ACTH
Cushing Syndrome Clinical Features
Lipid:truncal obesity, buff hump
Glucose: hyperglycemia, pouly dypsia, glucosuria
Protein:fragile skin and bv, decr muscle
Suppressed immunity
Psychosis
Hirsutism
Menstrual abnormalities
Hypertension
Cushing Syndrome Laboratory Findings
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
Cushing Syndrome & Disease, Laboratory Findings
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
Primary Aldosteronism Characteristics
Excess aldosterone
Sodium retention (hypernatremia)
Potassium excretion (hypokalemia)
Hypertension
Suppressed renin-angiotensin system = decreased renin
Primary Aldosteronism Causes
Adrenal cortical adenoma
Adrenal cortical hyperplasia
80% of cases, adenoma
20% of cases, hyperplasia
Primary aldosteronism caused by adenoma = ____
Conn syndrome
Primary Aldosteronism Clinical Features
Mid-life
Female bias
Hypertension
Hypokalemia, renal wasting of K
Low serum renin
Non-specific symptoms of weakness, parasthesias, visual disturbances
Primary Aldosteronism Diagnosis
Increased serum aldosterone
Depressed serum renin
Hypokalemia
Hypernatremia
Hypertension
Determining The cause of primary aldosteronism, by Adrenal cortical hyperplasia or adrenal cortical adenoma, is very important because ______
the treatment of adenoma is surgical and that for hyperplasia is medical.
Secondary Aldosteronism Characteristics
Activation of renin-angiotensin system
Increased serum renin
Secondary Aldosteronism Causes
Congestive heart failure
Decreased renal perfusion, as seen in:
arteriolonephrosclerosis
renal artery stenosis
hypoalbuminemia
pregnancy (estrogen-induced) increased plasma renin substrate
Small, yellow adrenal cortical adenoma is highly suggestive of ______ syndrome
Conn
Best definition of adenoma
Removal of solitary nodule cures disease.
defines carcinoma
Finding of 3 of 12 histologic changes
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
Congenital Adrenal Hyperplasia: _______ Deficiency
21-Hydroxylase
Disorders of sexual differentiation, such as virilization, caused by adrenocortical neoplasms or congenital adrenocortical ______.
hyperplasia
Virilization caused by neoplasms are more commonly caused by adrenal cortical _______ than adrenocortical adenomas
carcinomas
21-Hydroxylase catalyzes production of 11-deoxycorticosterone from _____
progesterone
Congenital Adrenal Hyperplasia inheritance
Autosomal recessive
Congenital Adrenal Hyperplasia

Spectrum of deficiencies:
1. simple virilizing adrenogenitalism
2. salt-wasting syndrome
3. non-classic adrenogenitalism
Simple Virilizing Adrenogenitalism:
Lack of 21-hydroxylase, causes lack of normal ____
cortisol
Simple Virilizing Adrenogenitalism:

Reduced cortisol, increases ____
ACTH


(Stimulates androgens, virilizes
)
Congenital Adrenal Hyperplasia:

Defect, abnormal production cortisol, secondary to absence of appropriate enzymes for normal production of ____
cortisol
Congenital Adrenal Hyperplasia:

Lack normal cortisol, increases ACTH, stimulates production _______
“cortisol”, androgens


Increased androgens, virilizes
Congenital Adrenal Hyperplasia Treatment
Replace cortisol and restore normal feedback mechanism
Reduce production of androgens
Primary Adrenal Insufficiency

2 types
1. Acute insufficiency
2. Chronic insufficiency (Addison's)
Most common causes of chronic primary adrenal insufficiency
Autoimmune adrenalitis

Tuberculosis
Amyloidosis
Hypothalamic, pituitary origin
Iatrogenic
Hemochromatosis
Sarcoidosis
Primary Chronic Adrenal Insufficiency
most common causes
TB, most common in past
Infections: Histoplasma, most common fungus
Metastatic neoplasms: most common, breast and lung,
Histology of adrenal insuff: autoimmune adrenalitis, cortex destroyed, medulla preserved, prominent infiltrate of _____
lymphocytes (lymphocytic adrenalitis)
Visible abnormality of Addison's pt
increased melanin in skin
Primary Acute Adrenocortical Insufficiency May occur in following clinical settings
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,
Chronic Adrenal Insufficiency Clinical Features
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
Differentiating Primary from Secondary Adrenal Insufficiency
Low ACTH in primary form
Administration of ACTH:
No increase in cortisol in primary form
Prompt increase in ACTH in secondary form
Adrenal Medulla:
Cells are of neuroendocrine origin characterized by following immunohistochemical reactions:
neuron-specific enolase: positive
chromogranin: positive
synaptophysin: positive
Chromaffin (not chromogranin) reaction, positive: K-dichromate _____ color to tumor and cells
brownish
Neuroendocrine cells can secrete other polypeptides, such as ____
histamine, serotonin, ACTH etc.
Adrenal Medulla Cells: secrete ____
epinephrine, norepinephrine (catecholamines); normally more epinephrine than norepinephrine
Metabenzylguadinine, combined with radioactive iodine to yield ________
metaiodobenzylguanidine (MIBG)
MIBG combines with catachol amines to yield image of _____
adrenal medulla




In case of tumors, MIBG combines with catachol amines to produce recognizable image
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
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 Medulla Pheochromocytoma
Pheochromocytoma:
85%, arise in adrenal medulla

15%, extramedullary sites, paragangliomas

Pheochromocytomas vs paragangliomas? (chromaffin)
Pheochromocytomas, chromaffin positive

Paragangliomas, chromaffin negative
Genes Associated with pheochromocytomas
multiple endocrine neoplasia IIa
multiple endocrine neoplasia IIb
what disease?
medullary thyroid carcinoma, unusual facies, mucosal neuromas, colonic ganglioneuromatosis, Marfanoid habitus, corneal nerve hypertrophy, Hirschsprung disease
Multiple endocrine neoplasia IIb
What disease?
pheochromocytomas, neurofibromatosis, café au-lait spots in skin, Schwannomas, meningiomas, gliomas
von Recklinghausen syndrome: pheochromocytomas,
What disease?
medullary thyroid carcinoma
bilateral pheochromocytomas
parathyroid hyperplasia (primary hyperparathyroidism)
Multiple endocrine neoplasia IIa
What disease?
pheochromocytomas, renal, pancreatic, hepatic and epididymal cysts
von Hippel-Lindau syndrome
What disease?
pheochromocytomas, cavernous hemangiomas of 5th cranial nerve
Sturge-Weber syndrome:
Familial pheochromocytomas are usually bilateral or unilateral?
bilateral
Diagnosis of Pheochromocytoma
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
Pheochromocytoma Clinical Features
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
Pheochromocytoma Laboratory Findings
Increased free catecholamines or metabolites (vanillylmandelic acid, (VMA), metanephrines), in urine
Pheochromocytoma Treatment
: Surgical removal of tumor or tumors after careful preparation of patient
Diagnosis of Paragangliomas
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
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
Genetic markers of neuroblastoma
1p deletion
N-myc amplification
DNA hyperploidy
Laboratory findings of neuroblastoma
High urinary catecholamines/metabolites
EM - secretory granules
Histology, stains like neuroendocrine tumor
Where is neuroblastoma?
20-25%, adrenal medulla

2nd, paravertebral, post mediastinum

3rd, multiple sites, neck, pelvis, brain

Gross: minute to large ( >1 kgm)
What does neuroblastoma look like microscopically?
Micro: undiff, small, blue cell tumor with Homer-Wright pseudorosettes to well diff tumor with ganglia, fibrillary glia-like material
Stages of neuroblastoma
Stages I-IVS, S=special, small adrenal tumor , infiltrates in liver, skin, bone marrow, no bone destruction
Neuroblastoma Prognostic Features
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
Parathyroid glands are Derived from ______ pouches, like thymus, therefore, not surprising to find ectopic glands within the thymus
pharyngeal
Parathyroid Dominant cell, _____ cell; other cells: clear cells, oxyphilic cells
chief
Hypocalcemia: low blood calcium, associated with what symptoms?
muscle spasms, tetany & cardiac dysfunction
Three major pools of calcium:
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
Majority of phosphate (~85%) tied up in ____ phase; normal blood concentration of phosphate very similar to Ca
mineral
Frequent adjustments of Ca, described as fluxes between blood & other compartments, controlled by 3 organs:
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
Parathyroid hormone: acts to increase blood Ca by:
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
Vitamin D: increases Ca concentration in blood by:
Facilitating absorption of Ca from small intestine
Acting with parathyroid hormone, enhances fluxes of Ca out of bone
Calcitonin: ?reduces or increases? blood Ca; secreted in response to hypercalcemia
reduces


Suppresses renal resorption of calcium
Inhibits bone resorption which minimizes fluxes of Ca from bone to blood
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
hormone
The Extracellular Calcium-Sensing Receptor is member of ______ family of receptors
G-protein coupled
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 ______&______
PTH & calcitonin
best way to differentiate primary and secondary hyperparathyroidism?
look at phosphate
Primary hyperparathyroidism: intrinsic to parathyroid glands; increased secretion of PTH with resulting _____&______
hypercalcemia & hypophosphatemia
Secondary hyperparathyroidism: PTH resistance in target tissues: resulting in ____&_____
hypocalcemia & hyperphosphatemia(?)
Causes of primary hyperparathyroidism:
1. Adenomas (single) (80%)
2. Parathyroid hyperplasia (5-20%)
3. Parathyroid carcinomas (2-3%)