11 - Endocrine System Flashcards
(191 cards)
1
Q
what are the major endocrine glands
A
- hypothalamus
- pituitary glands
- Thyroid gland
- Parathyroid gland
- Islet cells of the pancreas
- Adrenal glands
- Testes in men, and the ovaries in women
2
Q
Source, major target organ(s) and major physiologic effects:
growth hormone
A
source: anterior pit
target: liver, adipose tissue
effects: promotes growth; control of proteins, lipids and metabolism
3
Q
Source, major target organ(s) and major physiologic effects:
TSH
A
source: anterior pit
target: thyroid gland
effects: stimulates secretion of thyroid hormones
4
Q
Source, major target organ(s) and major physiologic effects:
adrenocorticotropic hormone ACTH
A
source: anterior pit
target: adrenal gland (cortex)
effects: stimulates secretion of glucocorticosteroids
5
Q
Source, major target organ(s) and major physiologic effects:
prolactin
A
source: anterior pit
target: mammary gland
effects: milk production
6
Q
Source, major target organ(s) and major physiologic effects:
LH
A
source: anterior pit
target: ovaries and testis
effects: control of reproducive function
7
Q
Source, major target organ(s) and major physiologic effects:
FSH
A
source: anterior pit
target: ovaries and testis
effects: control of reproductive function
8
Q
Source, major target organ(s) and major physiologic effects:
ADH
A
source: posterior pit
target: kidney
effects: conservation of body water
9
Q
Source, major target organ(s) and major physiologic effects:
oxytocin
A
source: posterior pit
target: ovaries and testis
effects: stimulates milk, ejaculation and uterine contractions
10
Q
both anterior and posterior lobes of pituitary are under control of ___ which is connected by ___
A
hypothalamus; pituitary stalk
11
Q
what is the master gland
A
pituitary gland
12
Q
what are the most common cause of hyperpituitarism
A
pituitary adenomas
13
Q
what are the types of pituitary adenomas
A
functioning (majority) and nonfunctioning
14
Q
what size is microadenoma? macroadenoma?
A
<1 cm = microadenoma (means benign tumor)
>1 cm = macroadenoma
15
Q
The signs and symptoms of pituitary adenoma are related to the ___ and ___
A
endocrine abnormalities and mass effects
16
Q
can the biologic behavior of the adenoma be reliably predicted from its histologic appearance
A
no
17
Q
what is the most common hyperfunctioning pituitary adenoma that is prolactin secreting?
A
lactotrophic adenoma
18
Q
symptoms of lactotrophic adenoma
A
Galactorrhea
Amenorrhea
Infertility
Loss of libido
19
Q
who gets lactotrophic adenoma? how is it treated
A
> women; between 20-40 years of age
In older women and men, the hormonal
manifestations may be subtle
Treated with dopamine receptor agonists
(bromocroptone) if small; surgery if large
20
Q
what is a growth hormone secreting adenoma that is the second most type of functioning pituitary adenomas
A
somatotrophic adenoma
21
Q
what causes giagantism in children and acromegaly in adults?
A
somatotrophic adenoma
22
Q
what are elevated levels of GH before closure of epiphyseal plates (13-15 years for girls and 15-17 years for boys)
A
gigantism
23
Q
does growth occur symmetrically in gigantism
A
yes
24
Q
if increased GH levels persis beyond ephiphyseal closure in gigantism, what happens
A
acromegally is superimposed
25
Elevated levels of GH after closure of epiphyses; Growth noted in soft tissues, skin, viscera, and bones of face, hands & feet
acromegaly
26
oral manifestation of acromegaly
prognathia (protrusion of mandible), teeth separation, and macroglossia
27
treatment of acrimegaly
surgery; dopamine agonits
28
what disease:
Failure to suppress GH production in
response to an oral load of glucose is one of
the most sensitive tests for acromegaly
somatotrophic adenoma
29
how can somatrotrophic adenoma be removed
surgically or treated pharmacologically with GH receptor antagonists
30
what adenoma:
Excess production of the ACTH leads
to adrenal hypersecretion of cortisol
and the development of
hypercortisolism (Cushing disease)
corticotroph adenoma
31
difference between cushing SYNDROME vs DISEASE
syndrome: increase steroid medication, adrenal gland adenoma, increased ACTH production by lung canger, or treat the cause of increased level of cortisol
disease: pituitary gland tumor results in increased ACTH causing hyperplasia of adrenal gland which further results in increased cortisol levels, tx is surgery of pituitary or adrenal gland
32
what is this:
Moon facies
Buffalo hump
Truncal obesity
Violaceous striae
Hirsutism
Hypertension
Glucose intolerance or diabetes mellitus
Visual symptoms if adenoma large
cushing disease (selena gomez)
33
Refers to decreased secretion of pituitary
hormones, which can result from diseases of
the hypothalamus or the pituitary
hypopituitarism
34
T/F: Hypofunction of the anterior pituitary occurs when approx. 75% of the parenchyma is lost
TRUE
35
When accompanied by posterior pituitary dysfunction (diabetes insipidus), hypopituitarism is almost always of ___
hypothalamic origin
36
most cases of hypopituitarism arise from processes involving what? examples?
anterior pituitary
1. Tumors and other mass lesions
2. Traumatic brain injury
3. Pituitary surgery or radiation
4. Pituitary apoplexy (sudden hemorrhage in pituitary gland, often occurring in a pituitary adenoma)
37
what is ischemic necrosis of anterior pituitary also called
Sheehan syndrome aka postpartum pituitary necrosis
38
what is the most common form of ischemic necrosis of anterior pituitary
sheehan syndrome
39
with sheehan syndrome during pregnancy, what happens to anterior pit
enarlges 2x size
40
in sheehan syndrome, is the expansion of gland accompanied by increased in blood supply?
no
41
in sheehan syndrome, since posterior pituitary is supplised directly by arterial branches, is it more or less susceptible to ischemic injury
less susceptible
42
what is the absence of lactation called
agalactorrhea
43
symptoms and tx of sheehan syndrome
symptoms:
- agalactorrhea
- amenorrhea or oligomenorrhea
- hot flashes
- decreased sex drive
tx: lifelong replacement of deficient hormones
44
clinical relevant posterior pituitary syndromes involve what
ADH
45
what are posterior pituitary syndromes
1. diabetes insipidus
2. syndrome of inappropriate secretion of ADH
46
what is caused by ADH deficiency and characterized by excessive urination (polyurea) due to inability of kidneys to resorb water properly from urine
diabeted insipidus
47
DI can occur from what conditions
Head trauma
Tumors
Inflammatory disorders of hypothalamus and pituitary
Surgical complications
Genetic basis (rare)
48
symptoms of DI and tx
Extreme thirst
Dry mouth
Dizziness, fatigue, and nausea
Frequent urination
tx: desmopressin (synthetic aDH)
49
what is this:
ADH excess causes over resorption of free water, resulting in hyponatremia
secretion of inappropriate ADH secretion (SIADH)
50
most frequent causes of SIADH
Secretion of ectopic ADH by malignant neoplasm
(small cell ca of the lungs)
Drugs that increase ADH secretion
A variety of CNS disorders including infections and
trauma
51
in SIADH, if the total body water is increased, does blood volume remain normal with no peripheral edema
YES
52
what this:
Consists of two lateral lobes connected with a thin isthmus
Located below and anterior to the larynx
It develops from an evagination of the
pharyngeal epithelium that descends from the foramen caecum at the base of the tongue to its position in the anterior neck
thyroid gland
53
the thyroid is divided by thin fibrous septae into lobules composed of what?
20-40 follicles
54
what are thyroid follicles lined by
cuboidal or low columnar epi and filled with PAS positive thyroglobulin
55
In response to ___, TSH is
released from the anterior pituitary gland into the circulation
hypothalamic factors
56
where does TSH bind
receptors on thyroid follicular epi cells
57
thyroid follicular epi cells convert thyroglobulin to what?
thyroxine - T4
triiodinethyronine - T3
58
T3 binds to what in target cells
thyroid hormone nuclear receptors
59
affects of T3 and T4 on cellss
1. Stimulation of carbohydrate and lipid catabolism
2. Protein synthesis
60
what is #1 function of thyroid gland
increase in basal metabolic rate
61
what is #2 function of thyroid gland/ohrmone
brain development in fetus and neonates
62
what are chemical agents that inhibit function of thyroid gland
goitrogens
63
why do goitrogens cause hyperplastic enlargement of gland (goiter)
they suppress T3 and T4 synthesis and TSH level increases
64
what is this:
Part of the follicle
Synthesize and secrete the hormone calcitonin
This hormone promotes the absorption of calcium by the skeletal system and inhibits resorption of bones by osteoclasts
parafollicular cells (c cells)
65
what are hyperthyroidism diseaes
1. graves disease (immune mediated)
2. thyrotoxicosis
66
what is the hypermetabolic state caused by elevated circulating levels of free T3 and T4
thyrotoxicosis (hyperthyroidism)
67
most common causes of thyrotoxicosis
1. Diffuse hyperplasia of the thyroid associate with Graves disease (85% cases)
2. Hyperfunction multinodular goiter
3. Hyperfunction thyroid adenoma
68
thyrotoxicosis clinical features
1. increase basal metabolic rate
2. cardiac manifestations
3. overactivity of sympathetic nervous system
4. occular changes
5. skeletal system affected
6. thyroid storm
69
what is the most useful single screening test for hyperthyroidism?
low TSH values!!! measurement of serum TSH concentration
70
In occasional patients, can hyper thyroidism result predominantly from increased circulatory levels of T3 (T3 toxicosis); where in these cases, free t4 levels may be decreased?
YES
71
tx for hyperthyroidism
1. beta blockers
2. thioamide
3. iodine solution
4. radioactive iodine
72
what are hypothyroid diseases
1. cretinism
2. myxedema
73
Condition caused by a structural or functional derangement that interferes with the production of thyroid hormone
Prevalence increases with age
women (10W;1M)
hypothyroidism
74
Refers to hypothyroidism that develops in
infancy and early childhood
cretinism
75
clinical features of cretinism
Severe intellectual disability
Short stature
Coarse facial features
Protruding tongue
Umbilical hernia
76
what is hypothyroidism development in older child or adult
myxedema
77
clinical findings of myxedema
Early symptoms include generalized fatigue, apathy, and mental sluggishness which mimics depression
Speech and intellectual functions are slowed, and patients are listless, cold intolerance, and frequently overweight
Skin is cold
Reduced cardiac output
78
measurement of what is the most sensitive screening test for hypothyroidism
serum TSH levels
79
Diverse group of disorders characterized by
some form of thyroid inflammation
thyroiditis
80
what is clinically significant form of thyroiditis
hashimoto thyroiditis
81
what is an autoimmune disease, results in destruction of thyroid gland and gradual and progressive thyroid failure, between 45 to 65 years of age, more in women
hashimoto thyroiditis
82
Caused by a breakdown in self-tolerance to
thyroid autoantigens
Predisposition has a strong genetic
component
hashitomo thyroiditis
83
Painless enlargement of the thyroid gland
associated with some degree of hypothyroidism
> in middle-aged women
Enlargement symmetric and diffuse
Incidences are at an increased risk for
developing other autoimmune disease
They are also at an increased risk of B-cell
lymphoma
hashimoto thyroiditis
84
lab values of hashimoto thyroidities
Fall in serum levels to T3 and T4
Compensatory increased serum TSH
85
Autoimmune disorder
Peak incidence between 20 and 40 years of age
> women (10:1)
Characterized by the production of autoantibodies against multiple thyroid proteins most importantly the TSH receptors
Most common cause of endogenous hyperthyroidism
graves disease
86
clinical findings of graves disease
1. hyperthyroidism
2. infiltrative opathalmopathy
3. localized, infiltrative dermoopathy, sometimes called pretibial myxedema
87
graves disease lab values
Elevations in serum levels to T3 and T4
Decreased serum TSH
88
graves disease tx
Treated by beta blockers (dampened symptoms related to increased sympathetic nervous system activity)
Radioiodine ablation
Thyroidectomy
89
Enlargement of thyroid caused by impaired
synthesis of thyroid hormone, which is most
often the result of dietary iodine deficiency
goiter
90
is goiter autoimmune deficiency?
no
91
Reduced thyroid hormone production leads to compensatory rise in serum TSH level, which, in turn, causes hypertrophy and hyperplasia of thyroid follicle cells and ultimately enlargement of thyroid gland
goiter
92
This increase in the functional mass of the gland in goiter usually overcomes the hormone deficiency, enduring a ___ in most individuals
euthyroid metabolic state (normal function)!!!!!
93
two types of goiter
1. diffuse nontoxic goiter
2. multinodular goiter
94
Causes enlargement of the entire gland
without producing nodularity
diffuse nontoxic simple goiter
95
clinical features of diffuse nontoxic goiter
Most patients with simple goiter are clinically euthyroid
Therefore, clinical manifestations are
primarily related to mass effect from the
enlarged thyroid gland
96
lab values of diffuse nontoxic goiter
Serum T3 and T4 levels are normal
Serum TSH is usually elevated
97
Produce the most extreme thyroid
enlargement; frequently mistaken for
neoplasms
multinodular goiter
98
Arise because of variations among follicular
cells in their response to external stimuli,
such as trophic hormones
multinodular goiter
99
clinical features of multinodular goiter
Dominant feature is causation of mass effect
Cosmetic effects
May cause airway obstruction
Dysphagia
Most patients are euthyroid (normal funcitoning thyroid gland)
100
diffuse nontoxic goiter lab values
Serum T3 and T4 levels are normal
Serum TSH is usually elevated
101
what are neoplasms of thyroid
thyroid adenoma and thyroid carcinoma
102
do benign thyroid neoplasms or malignant thyroid neoplasms outnumber?
benign
103
A history of radiation therapy to the head and neck region is associated with an [ increased OR decreased] incidence of thyroid malignancy
increased
104
Typically discrete, solitary masses, derived
from follicle epithelium, and hence they are
known as follicular adenomas
Most are nonfunctional nodules
Solitary painless masses that are discovered
on routine physical examinations
Larger masses may produce difficulty in
swallowing
thyroid adenoma
105
thyroid adenoma tx
surgical excision of any suspicion nodules
106
5-year SR > 95%
> women
> after the age of 30
Frequently presents without any symptom
Sign: neck mass
Symptoms: hoarseness or change in voice
thyroid carcinoma
107
thyroid carcinoma subtypes
papillary thyroid carcinoma (follic) (most common)
medullary carcinoma (c cell)
108
lymphoma of thyroid occurs in setting of what? is it curable?
setting of autoimune hashimoto thyroiditis
highly curable
109
what are parathyroid gland diseases
1. hyperparathyroidism
2. hypoparathyroidism
110
how many glands in parathyroid galnd? what cell types?
4 glands, composed of 2 cell types:
Chief cells
Oxyphil cells (have secretory granules containing
parathyroid hormones)
111
function of parathyroid gland
1. To regulate calcium homeostasis
2. The activity of the parathyroid gland is controlled by the levels of free (ionized) calcium in the blood
3. Decreased levels of free calcium stimulates the synthesis and release of the PTHhormone
112
classifications of hyperparathyroidism
1. primary - adenoma or hyperplasia
2. secondary - chronic renal failure
3. tertiary - after renal transplant
113
One of the most common endocrine disorders,
and it is an important cause of hypercalcemia
primary hyperparathyroidism
114
#1 cause of hyperPTH
adenoma
115
Disease of adults
> women
Most common cause is a solitary sporadic
adenoma
May be asymptomatic and identified on
routine blood chemistry profile or associated with the classic clinical manifestations of bones, stones, abdominal groans, and moans
primary hyperPTH
116
lab valuses of primary PTH
high serum calcium
high serum alkaline phosphatase
117
treatment of primary hyperPTH
surgical excision of enlarged gland
118
why is brown tumor brown
bc old blood hemorrhage
119
Cystic bone lesions may appear
in severely affected patients
Hemorrhage and old blood give
the brown appearance
May affect jaw bones
Identical to the much more common
central giant cell granuloma
Excessive osteoclast activity
brown tumor
120
Most severe skeletal manifestation
Develops from central degeneration
and fibrosis of long-standing brown
tumor
Osteitis fibrosa cystica
121
what contains the islents of Langerhans
endocrine pancrease
122
major and minor cells types of endocrine pancreas
The major cell types are :
a, B, d, and PP (pancreatic polypeptide) cells
Minor cell types:
D1 cells
Enterochromaffin cells
123
___ produce ___, which regulates glucose utilization in tissues, and reduces blood glucose levels
beta cells; insulin
124
___ secrete ___, which stimulates glycogenolysis in the liver and thus increases
blood sugar
alpha cells; glucacon
125
___ secrete ___, which suppresses
both insulin and glucagon release
delta cells; somatostatin
126
___ secrete ___,
which exert several GI effects, such as
stimulation of secretion of gastric and intestinal
enzymes and inhibition of intestinal mobility
PP cells; pancreatic polypeptides
127
___ secretes vasoactive intestinal
polypeptides, a hormone that induces
glycogenolysis and hyperglycemia
___ synthesize serotonin
D1 cells; Enterochromaffin cells
128
Group of metabolic disorders sharing the
common feature of hyperglycemia, caused
by defects in insulin secretion, insulin
action, or most commonly, both
diabetes mellitus
129
what should blood sugar normally be
Blood sugar is normally maintained at a very
narrow range of 70-120mg/dL
130
DM fasting plasma glucose
> or = to 126mg/dL
131
DM random plasma glucose
> or = to 200mg/dL
132
DM 2 hour plasma glucose during oral glucose tolerance test (OGTT) w/ 75 g loadnig dose
> or = to 200mg/dL
133
DM glycated hemoglobin HbA1c level
< or = to 6.5%
134
Autoimmune disease characterized by pancreatic ß cells destruction and an absolute deficiency of insulin (5%-10% of cases)
Diagnosed in patients younger than 20
years of age
Type 1 DM
135
Peripheral resistance to insulin action
A secretory response by pancreatic ß cells
that is inadequate to overcome insulin
resistance (90%-95% cases)
Vast majority of patients are overweight
Type 2 DM
136
onset, weight, insulin levels, and ketotic?
type 1 DM
onset: childhood
weight: normal or weight los
insulin: progressive decrease in insulin
keto: diabetic keto acidosis in absence of insulin therapy
137
onset, weight, insulin levels, and ketotic?
type 2 DM
onset: adults
weight: obese
insulin: early increased blood insulin, later has normal or moderate decrease in insulin
keto: nonketotic hyperosmolar coma more common
138
pathogenesis of type 1 dm
dysfunction in T cells selection and regulation leading to breakdown of self toelrance to islet autoantigens
139
pathogenesis of type 2 DM
insulin resistance in peripheral tissues, failure of compensation by beta cells
140
pathology of type.1 dm
insulitis (inflammatory infiltrate of T cells and macrophages), beta cell depletion, islet atrophy
141
pathology of type 2 dm
no insulitis; amyloid deposition in islets, mild beta-cell depletion
142
May arise at any age
Initial 1-2 years after onset, exogenous
requirements may be minimal because of
endogenous insulin secretion
Eventually, however, ß-cell function declines and
insulin requirement increases dramatically
t1 dm
143
Typically seen in obese patients older than 40
Medical attention is sought because of unexpected
fatigue, dizziness, or blurred vision
More frequently, diagnosis is made after routine
blood testing in asymptomatic patients
type 2 dm
144
Onset of ___ is usually marked by the triad of
polyuria, polydipsia, polyphagia, and when
severe, diabetic ketoacidosis, all resulting
from metabolic derangements
type 1 dm
145
Diabetic ketoacidosis is a severe acute
metabolic complication of ___; may occur
less commonly in ___; clinical manifestations of
T1D, T2D
146
clinical manifestations of ketoacidosis
Fatigue, nausea, and vomiting
Severe abdominal pain
A characteristic fruity odor
Deep labored breathing
147
Patients with ___ may develop hyperosmolar
hyperglycemic state due to severe
dehydration resulting from sustained osmotic
diuresis; occurs in older patients who do not
maintain adequate hydration
T2D
148
most common acute metabolic complication ___ is hypoglycemia. what does this cause?
in either type of DM
Causes include missing a meal, excessive
physical exertion, or excessive insulin
administration
Signs and symptoms include dizziness, confusion,
sweating, palpation, and tachycardia
149
morbidity associated with long term T1D and T2D is due to what
damage induced large and medium sized muscular arteries - diabetic macrovascular disease
small vessels - diabetic microvascular disease by chronic hyperglycemia
150
___ causes accelerated atherosclerosis, resulting in increased risk of myocardial infarction, stroke, and lower extremity ischemia
___ are more profound in
the retina, kidneys, and peripheral nerves
Macrovascular disease; Microvascular disease
151
what are consequences of DM
1. hyaline arteriolosclerosis
2. atherosclerosis
3. nephropathy
4. retinopathy
5. neuropathy
152
where is hyaline arteriolosclerosis most evident
skin, muscle, retina, and renal glomeruli
153
what is the most common cause of deaht in diabetics
myocardial infarction
154
what is the most severely damaged organ in diabetics
kidney
155
how an you tell if kidney is severely damaged on histology slide?
Kimmelstiel-Wilson lesion
156
what are oral manifestations of diabetes
1. dental caries
2. periodontitis
157
what are the zones of adrenal cortex
1. Zona glomerulosa (synthesizes mineralocorticoids)
2. Zona reticularis (synthesizes estrogen and androgens)
3. Zona fasciculata (synthesizes glucocorticoids)
158
what is composed of chromaffin cells (synthesize catecholamines, mainly epinephrine)
adrenal medulla
159
what causes hyperfunction of adrenal cortex
1.Hypercortisolism -Cushing
Syndrome-excess cortisol
2. Hyperaldosteronism- excess
aldosterone
3. Virilizing syndromes
160
what causes hypofunction of adrenal cortex
1.Primary adrenocortical
insufficiency-Addison Disease)
161
if pituitary adenoma is responsible for hypercortisolism, it is called what
cushing DISEASE
162
what is a disorder caused by conditions that produced elevated glucocorticoid levels (due to cortico steroid therapy, or production of adrenocorticotropic hormone by adrenal adenoma and carcinoma)
cushing SYNDROME
163
can an ectopic ACTH be secreted by a small-cell lung carcinoma/cancer
yes
164
Unfortunately, therapeutic corticosteroids suppress the production of ACTH by the pituitary gland to the extent that it cannot produce ACTH in response to stress. what is this episode call
An acute episode of hypoadrenocorticosteroid
(Addison crisis) may be precipitated
165
For stressful dental and surgical procedures especially, it is often necessary to increase the corticosteroid dose because of the greater need of cortisol
Consultation with the physician who is managing the corticosteroid therapy is advised
why
potential of addisonian crisis
166
difference between primary, secondary, and tertiary adrenal insuficciency
primary: adrenal problem
secondary: pituitary problem
tertiary: hypothalamus problem
167
Insufficient production of adrenal
corticosteroid hormone caused by the
destruction of adrenal cortex
hypoadrenocorticism/addison disease
168
what causes addison disease? #1 cause?
#1 cause: Autoimmune destruction (most common cause in Western countries)
2. Infections (TB, deep fungal infections, particularly in AIDS patients)
3. Rarely, metastatic tumors, sarcoidosis, amyloidosis, or hemochromatosis
169
when do clinical features appear in addison disease
until 90% of glandular tissue has been destroyed
170
Caused by elevated levels of pro-
opiomelanocortin (POMC), derived from anterior pituitary and is a precursor of both ACTH and melanocyte stimulating hormone (MSH)
hypoadrenocorticism (addison disease)
171
oral manifestations of hypoadrenocorticism (addison disease)
Diffuse or patchy, brown, macular
pigmentation of the oral mucosa
Often the oral mucosal changes are the first manifestation of the disease, with the skin pigmentation occurring afterwards
172
difference between primary and secondary hypoadrenocorticism
In primary hypoadrenocorticism, plasma
levels of ACTH are high (>100 ng/L)
In secondary hypoadrenocorticism the levels
are normal (9 to 52 ng/L) or low (due to
decreased production of ACTH by the
pituitary gland)
173
treatment for addison DISEASE? must you do for dental procedures
hormone replacement therapy
not required for dental procedures using LA and lasting less than 1 hour
174
wat is major source of catecholomines in body
adrenal medulla
175
neoplasms of adrenal medulla
1. Neoplasms of chromaffin cells (pheochromocytomas)
2. Neuronal neoplasms (neuroblastic tumors)
176
adrenal medulla cancers
1. pheochromocytoma
2. neuroblastoma
177
Rare, chromaffin cell tumor of the adrenal medulla; synthesize and release catecholamines and in some cases peptide
hormones
Important to recognize them because they are a rare cause of surgically correctable hypertension
pheochromocytoma
178
pheochromocytoma rule of 10s
10% of pheochromocytomas are extra-adrenal
10% of sporadic adrenal pheochromocytomas are bilateral
10% of adrenal pheochromocytomas
are biologically malignant
10% of adrenal pheochromocytomas are not associated with hypertension
179
Cancer composed of immature neuroblasts
(immature nerve cells)
Most commonly arises in and around the
adrenal glands which have similar origin to nerve cells
Common malignant tumor in childhood
Medianage at presentation 23 months, peak 0-4 years
Slightly more common in boys (1.2:1)
neuroblastoma
180
what are the multiple endocrine neoplasia syndromes
MEN Type 1, 2A, 2B, and 4
THERE IS NO 3
181
is MEN AD or AR? what does this result in
AD
hyperplasia, adenoma, and carincoma
182
AD inheritance
Syndrome characterized by mutations in MEN1 gene
Development of multiple endocrine tumors (and other tumor types)
Classic constellation of pituitary, parathyroid and pancreatic tumors (the 3 P's)
Variety of nonendocrine tumors are also part of this (meningioma, ependymoma,
angiofibroma)
MEN 1 Syndrome (Wermer)
183
is men 2a or 2b more common
2a
184
what cause men 2 syndrome
Both conditions are due to one of the several RET
proto-oncogene mutations
185
how does men 2 affect thyroid and adrenal glands?
thyroid - medullary thyroid cancer when yound adults
adrenal glands - pheochromocytomas
186
what is characterized by
Pheochromocytoma
Medullary carcinoma of the thyroid
Parathyroid hyperplasia
men 2s - sipple syndrome
187
which men syndrome features ORAL FEATURE
men 2b
Ganglioneuromas of mucosa of GI tract, lips & tongue
188
These patients have clinical features that
photocopy MEN-1 patients
In contrast to MEN-1, they harbor germline
CDKN1B mutations leading to reduced levels of
the cell-cycle checkpoints protein p27
men 4 syndrome
189
gene mutation for men 4
CDKN1B mutations
190
gene mutation for men 2
RET proto-oncogene mutations
191
gene mutation for men 1
men1 gene
