Endocrine System Flashcards

1
Q

Rapid enlargement of pituitary adenoma with hemorrhage, depression of consciousness

A

Pituitary apoplexy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Most common cause of hyperpituitarism

A

Anterior lobe adenoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Pituitary adenoma classifications

Size

Hormone production

A

Macro >1cm
Micro <1cm

Functioning (GH+Prolactin) mc
Non functioning

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Best characterized molecular abnormalities in pituitary adenomas

A

G protein mutation
in the alpha subunit interfering with its intrinsic GTPase activity resulting in activation of Gsalpha generation of cAMP and unchecked cellular proliferation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

G protein mutation is a mutation the gene

found in 40% of

A

GNASI

GH secreting somatotroph

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

5% of adenomas are inherited with identified mutations in the genes

A

MEN I (familial pituitary a)
CDKN I B
PRKARI A
AIP

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Gene affected in pituitary adenomas appearing before 35 years secreting GH

A

aryl hydrocarbon receptor interacting protein

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Mutation of the gene is assoc with aggressive pituitary adenoma behavior and recurrence
Mutation demonstrates brisk mitotic activity and designated atypical adenoma

A

TP53

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Well circumscribed soft lesion with uniform polygonal cells arrayed in sheets, cords or papillae
Reticulin is sparse
Cytoplasm is acidophilic, basophilic or chromophobic depending on secretory product
Cellular Monorphism and absence of significant reticulin network distinguish it from non neoplastic anterior pituitary parenchyma

A

Pituitary adenoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Non functioning hormone negave p adenomas tend to be

and cause

A

macrocytic

hypopituitarism and destroy adjacent anterior pituitary adenoma (mass effect and visual disturbances)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Most common type of hyperfunctioning adenoma

A

Prolactinoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Hyperprolactinemia cause

and may manifest earlier in premenop than men and post menop reaching considerable size

A

Amenorrhea
Galactorrhea
Loss of libido
Infertility

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Other causes of hyperprolactinemia

A
Pregnancy
High estrogen t
Renal failure
Hypothyroidism
Hypothalamic lesion
Dopamine inhibiting drugs (reserpine)
Mass on suprasellar compartment disturbing normal inhibitory hypothalamic influence (stalk effect)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Second most common type of functional pituitary adenoma

A

GH secreting

Somatotroph cell adenoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Persistent hypersecretion of GH stimulates the hepatic secretion of

causing clinical manifestation

A

insulin like growth factor I

somatomedin C

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Gen increase in body size with disproportionately long arms and legs
GH adenoma occuring before closure of epiphyses prepubertal

A

Gigantism

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Growth most conspicuous with soft tissue, skin and viscera and bones of face, hands and feet
Enlargement of jaw called
Broadening of lower face and separation of teeth
Hands and feet enlarged with broad sausage like
GH adenoma developing after epiphyses closure
Impaired glucose tolerance and DM

A

Acromegaly

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Stain + with periodic acid schiff due to inc ACTH
clinically silent or cause hypercortisolism manifested as
ACTH on adrenal cortex

A

Cushing syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Hypercortisolism caused by excess production of ACTH by PITUITARY

A

Cushing disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Corticotroph adenoma after removal of adrenal glands for tx of Cushing syndrome
Loss of inh effect of corticosteroids on microadenoma
No hypercortisolism but pituitary tumor mass effect and hyperpigmentation

A

Nelson syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Become detected only when they already produce mass effects
Demonstrate immunoreactivity for a subunit specific B FSH and BLH
FSH predominant secreted hormone

A

Gonadotroph adenoma

LH and FSH adenoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Account for 1% of all pituitary adenomas

Rare cause of hyperthy

A

Thyrotroph

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Exceedingly rare commonly metastasizes distantly

A

Pituitary carcinoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Hypopituitarism occurs when there is loss of function of as much as of anterior pituitary

A

75%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Hypopituitarism with posterior pituitary disfunction in the form of diabetes insipidus is almost always

A

hypothalamic in origin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Post partum ischemic necrosis of anterior pituitary in setting of hemorrhage
Inc bec of prolactin cell but without concomittant inc in bs
Post pituitary less susceptible bec of arterial bs
Also in DIC, sickle cell, inc ICP, trauma and shock

A

Sheehan syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

ADH deficiency causes

Excessive urination due to improper water reabsorption

A

Central Diabetes Insipidus

Nephrogenic if unresponsiveness of tubular cell to ADH

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Resorption of excessive amounts of free water with resultant hyponatremia
Ectopic ADH by small cell lung ca
Hypothalamic injury
Hyponatremia, cerebral edema, neurologic dysfunction
Inc TBW but N bv no periph edema

A

SIADH

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

Hypermetabolic state due to elevated circulating levels of T3 and T4

Hyperthyroidism is only one category of this

A

Thyrotoxicosis

Hypermetabolic state by excess thyroid hormone and overactivity of SNS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

Thyrotoxicos in elderly whom typical features of thyroid hormone excess are blunted
Unexplained weight loss and worsening CV disease

A

Apathetic hyperthyroidism

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

Most useful single screening test for hyperthyroidism

A

TSH

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

Worldwide mc cause of hypothyroidism

A

Iodine deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

Hypothyroidism in infancy or early childhood
Himalayas and Andes where iodine is endemically deficient
Impaired skeletal and CNS dev’t
Severe mental retardation, short stature, coarse facial, protruding tongue, umbilical hernia
MR is severe if maternal thyroid deficiency

A

Cretinism

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

Hypothyroidism in older children and adults
Cretinoid state in adult
Generalized apathy, mental sluggishness, depression like
Listless, cold intolerant, obese
Edema in skin, enlargement of tongue, deepening of voice
Dec bowel motility, pericardial effusion, heart enlargement and heart failure

A

Hypothyroidism

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

Most common cause of hypothyroidism in areas where iodine is sufficient
Major cause of nonendemic goiter in children

A

Hashimoto thyroiditis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

Breakdown in self tolerance to autoantigen
CD8 cytotoxic cell mediated cell death thyrocyte destructi on
Cytokine mediated interferon y resulting in mac activation and follicle damage
Binding of antithyroid ab followed by ab dependent cell mediated toxicity
Concordance in 40% of twins

Most significant link is cytotoxic T lymph associated

A

Hashimoto’s

Antigen 4 gene CTLA4 coding for negative regulator of T cell function

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

Diffuse sym enlarged with mononuclear inflammatory infiltrate
Well developed germinal center
Atrophic follicle with abundant eosinophilic granular cytoplasm called

A

Hashimoto’s thyroiditis

Hurthle cell Oxyphil cell

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

Metaplastic response of normally low cuboidal epithelium to ongoing injury
Numerous prominent mitochondria
Inc interstitial connective tissue
Small atrophic thyroid fibrosing variant but does not extend beyond capsule

A

Hurthle cell

Oxyphil cell of Hashimoto

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

Painless enlargement of thyroid assoc with degree of hypothyroidism preceded by transient thyrotoxicosis from 2 release of TH (hasitoxicosis)
Often have autoimmune disease
Inc risk for

A

Hashimoto’s

B cell non Hodgkin lymphoma and predisposition to papillary thyroid

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

Caused by viral infection or inflammatory process
Hx of URTI prior
Immune response is not self perpetuating
Gland is firm with intact capsule
Disruption of thyroid follicle
extravasation of colloid leading to PMN infiltrate
Exuberant granulomatous rx with giant cells with colloid
Healing by inflamm resol and fibrosis
PAIN in neck with swallowing, fever, malaise enlargement of thyroid
Transient hyperthy from follicle disruption
Inc leukocyte and ESR
Self limited, Eu in 6-8 w

A

Subacute Granulomatous de Quervain Thyroiditis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q
Silent or painless thyroiditis
Following pregnancy
Autoimmune bec circulating ab 
Thyrotoxicosis then euthyroid state
Lymphocytic infiltration and hyperplastic germinal center
A

Subacute Lymphocytic Thyroiditis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

Rare disorder unknown etiology
Extensive FIBROSIS of thyroid and neck
Hard fixed thyroid mass simulating neoplasm
Idiopathic fibrosis also in retroperitoneum
Autoimmune due to circ autoantibody

A

Riedel thyroiditis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

Most common cause of endogenous hypothyroidism

Diffuse hypertrophy and hyperplasia
Colloid is pale with scalloped margins

A

Graves

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

Triad of Graves

W 7x

A

Thyrotoxicosis
Ophthalmopathy
Infiltrative dermopathy (pretibial myxedema)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

Graves is assoc with genetic susceptibility from

A

HLA DR3
CTLA-4
PTPN22

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

Breakdown in self tolerance to thyroid autoantigen with TSH receptor
Thyroid stimulating IgG binding to TSH receptor mimicing TSH (specific)
Also proliferates thyroid follicular epithelium
TSH binding inh globulin prevent TSH fr binding to receptor on epithelial cell inh thyroid cell function
Coexistence of stimulating and inh IgG hence spontaneous hypothy

T cell mediated autoimmune phenomenon in infiltrative ophthalmopathy (inc volume of retroorbital tissue)

A

Grave’s disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

Most common manifestation of thyroid disease

A

goiter

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
48
Q

Degree of thyroid enlargement is proportional to the

A

Level and duration of thyroid hormone deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
49
Q

Endemic of

A

goiter is present in >10% of population

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
50
Q

Diffuse symmetric enlargement of gland (diffuse goiter) forming enlarged, colloid rich colloig goiter then eventually producing irregular enlargement called multinodular goiter.

Minority manifest with thyrotoxicosis producing autonomous nodules independent of TSH stimulation

A

Diffuse goiter
Multinodular goiter

Plummer syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
51
Q

Solitary thyroid nodules are more likely to be

A

neoplastic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
52
Q

Nodules in young patients tend to be

A

neoplastic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
53
Q

Nodules in males tend to be

A

neoplastic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
54
Q

Hx of irradiation at head and neck is assoc with

A

inc risk of malig

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
55
Q

Hot nodules or nodules that take up radioactive iodine are more likely

A

benign

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
56
Q

Follicular adenomas are assoc with mutations in

A

TSH receptor signalling pathway
Activating mutation of TSHR and alpha subunit of Gs GNAS allow follicular cells to secrete thyroid hormone (autonomy) hence hot nodule
Minority: less than 20 RAS, PIK3CA or PAX8/PPARG

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
57
Q

Solitary, well defined intact capsule with multiple nodules on cut surface no compression of adjacent thyroid parenchyma with Hurthle
Cell occasionally

A

Follicular adenoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
58
Q

Careful evaluation of this in follicular adenoma is distinguishing from a carcinoma

A

intact integrity of capsule

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
59
Q

Two pathways in thyroid carcinoma generation

A

MAP Kinase

PI-3K/AKT

60
Q

Genetic anomaly in most papillary thyroid carcinomas

A

MAP kinase by
rearrangement of RET or NTRKI
activating point mutation in BRAF

61
Q

Follicular thyroid carcinomas are assoc with genetic mutations of

A

PI3K/AKT pathway

PAX8/PPARG in less than 10%

62
Q

Highly aggressice arising from dedifferentiation from papillary or follicular
Sometimes by inactivation of TP53

A

Anaplastic carcinoma

63
Q

Arise from parafollicular C cells
Occur in MEN 2
Assoc with this mutation

A

Medullary thyroid

RET protooncogene

64
Q

Major rf predisposing to thyroid cancer is

A

ionizing radiation in first two decades of life

65
Q

Chernobyl nuclear disaster

A

Papillary carcinom

66
Q

Deficiency of iodine

A

Follicular carcinoma

67
Q

Diagnosis is based on nuclear features in absence of papillary architecture
Contain chromatin, optically clear ground glass or Orphan annie eye nuclei
Pseudoinclusion
Papillary architecture
Psammoma bodies
Most common variant: follicular

A

Papillary carcinoma

68
Q

Most common metastases of papillary

A

Cervical node

Lung (hematogenous)

69
Q

Widely invasive or minimal
Requires extensive histologic sampling of tumor-capsule thyroid interface to exclude:
Most frequent solitary COLD nodule

A

Follicular carcinoma

Capsular or vascular invasion

70
Q

Follicular adenomas metastasize through

A

hematogenous (lungs, bone, and liver)

with uncommon regional mets

71
Q

Aggressive mortality of
100%
Bulky masses growing beyond thyroid capsule into neck structures
Highly anaplastic: pleomorphic giant, spindle with sarcomatous, mixed spindle and giant

A

Anaplastic carcinoma

72
Q

Neuroendocrine neoplasm from parafollicular cells
Secrete:
Sporadic in 70, MEN2A or 2B in 30
Demonstrate RET mutation
Multicentricity C cell hyperplasia
Polygonal spindle shaped cells forming nests with Amyloid deposit

A

Medullary carcinoma

73
Q

95% of cases of primary hyperparathy is caused by

A

Adenoma
Primary hyperplasia 5-10
Parathyroid 1%

74
Q

Genetic defects in familial primary hyperparathyroidism

A

MEN1 and MEN2A
CyclinD1 gene inversion
chromosomal inversion
MEN 1 mutation

75
Q

Rare cause of hyperparathy due to inactivating mutation of calcium sensing receptor on parathyroid cell

A

Familial hypocalciuric hypercalcemia

76
Q

Invariable confined to single glands composed predominantly of chief cells
Endocrine atypia

A

Parathyroid adenoma

77
Q

Multiglandular process
Chief cell hyperplasia
Water clear cell hyperplasia from accumulation of glycogen

A

Parathyroid hyperplasia

78
Q

Circumscribed diff to distinguish from adenoma
Gray white irregular masses
Diagnosis based on cytologic detail is unreliable
Only definitive dx is invasion of surrounding tissue and mets
Skeletal and kidney changes

A

Parathyroid carcinoma

79
Q

Bone resorption with inc osteoblastic activity and new bone trabeculae
Grossly thinned cortex with marrow of inc fibrous tissue accompanied by foci of hemorrhage and cyst

A

osteitis fibrosa cystica

80
Q

Osteoclast, reactive giant cells and hemorrhagic debris

A

Brown tumor

81
Q

Most common manifestation of primary hyperparathyroidism is

A

inc serum ionized calcium

82
Q

Causes of hyperkalemia with raised PTH

A

Hyperparathyroidism
Primary adenoma>hyperplasia
Tertiary
Familal hypocalciuric hypercalcemia

83
Q

Hypercalcemia

Decreased PTH

A
Hypercalcemia of malignancy
Osteolytic metastases
PTH-rP-mediated
Vit D toxicity
Immobilization
Drugs thiazide
Granulomatous disease sarcoidosis
84
Q

Painful bones
Renal stones
Abdominal groans
Psychic moans

A

Primary hyperparathyroidism

85
Q

Chronic depression of serum calcium

Most common cause:

A

Secondary hyperparathyroidism

Renal failure causing hyperphosphatemia elevating PTH inc serum Ca and reduces alpha hydroxylase which converts Vit D leading to dec Ca reabsorption in intestine

86
Q

Hyperplastic parathyroid glands
Inc chief cell water-clear cell
Bone changes
Metastatic calcification

A

Secondary hyperparathyroidism

87
Q

Metastatic calcification of blood vessels secondary to hypophosphatemia resulting with significant ischemic skin damage

A

calciphylaxis

88
Q

Most important cause of symptomatic hypercalcemia resulting from osteolytic metastases or release of PTH related protein from nonparathyroid tumor

A

Malignancy

89
Q

Most common cause of hypoparathy

A

surgical

other causes
Congenital absence also with thymic displasia DiGeorge and cardiac defect
Autoimmune hypoparathyroidism

90
Q

Hereditary polyglandular deficiency arising from antibodies to MEN
Chronic fungal infection of skin and mucous
Causes by mutations in the gene:
Make autoantibodies against own IL17 hence inc susceptibility to Candida
Inc neuromuscular irritability tingling, spasm, facial grimace carpopedal spasm or tetany

A

Hypoparathyroidism

91
Q

Hypercortisolism typically manifests as
caused by condition that produces elevation in glucocorticoid levels
Mostly iatrogenic with the other 3 as primary hypo-pituitary, secretion of ACTH by non, primary adrenocortical neoplasm

A

Cushing’s syndrome

92
Q

Accounts for 70% of spontaneous Cushing syndrome
Primary hypothalamic pituitary disease assoc with hypersecretion of ACTH
Pituitary containing ACTH microadenoma without mass effect or corticotroph hyperplasia
Adrenal glands are charac by degree of bilateral nodular cortical hyperplasia secondary to inc ACTH

A

Cushing disease

93
Q

Often times, secretion of ectopic ACTH is caused by

A

Small cell carcinoma of the lung

carcinoid, medullary carcinoma or PanNET

94
Q

Primary adrenal adenoma or carcinomax

Primary cortical hyperplasia is a form of Cushing’s designated as

A

ACTH independent Cushing Syndrome

95
Q

Biochemical hallmark of adrenal cushing

A

elevated cortisol with low serum level of ACTH

96
Q

Macronodules of primary cortical hyperplasia of the cortex

A

> 3cm

97
Q

Micronodules of primary cortical hyperplasia

A

1-3mm diameter

98
Q

Most common pituitary alteration in Cushing syndrome resulting from high levels of endogenous or exogenous glucocorticoid is
cortical atrophy, diffuse hyperplasia, macronodular or micronodular hyperplasia or adenoma

A

Crooke hyaline change

normal granular basophilic cytoplasm replaced by homogenous lightly basophilic material due to accumulation of intermediate keratin

99
Q

Supression of endogenous ACTH from exogenous glucocorticoids in Cushings result in

A

bilat cortical atrophy
due to lack of stimulation
with normal zona glomerulosa thickness

100
Q

In ACTH dependent Cushing, glands are yellowish enlarged with

A

lipid laden, diffuse hyperplasia appearing vacuolated

101
Q

In primary cortical hyperplasia the cortex is replaced by with 1-3mm darkly pigmented nodules called

A

Macro or micro

Lipofuscin wear and tear

102
Q

Yellow tumors with thin well developed capsules less than 30g in zona fasciculata

A

Adrenal adenoma

103
Q

Larger than adenoma

200-300g all of anaplastic characteristics

A

Adrenal carcinoma

104
Q

Hypercortisolism causes selective atrophy of

A

Type II fast twitch myofiber and dec muscle mass of proximal limb

105
Q

Extraadrenal cause
Inc aldosterone bec of RAAS activation
Inc levels of plasma renin with dec re al perfusion, arterial hypovolemia and edema, pregnancy

A

Secondary hyperaldosteronism

106
Q

Primary autonomous production of aldosterone
With supression of RAAS
DEC plasma renin activity
due to bilateral nodular hyperplasia pf adrenals 60%, adrenocortical neoplasm, or familial hyperald from genetic defect leading to overactivity of:

A

Primary Hyperaldosteronism

Aldosterone synthase
CYP11B2

107
Q

Bilateral nodular hyperplasia

Most common UNDERLYING cause of primary hyperald 60% of cases

A

Bilateral idiopathic hyperaldosteronism

108
Q

Solitary aldosterone secreting adenoma is called

A

Conn’s syndrome

109
Q

Hallmarked by eosinophilic laminated cytoplasmic inclusions called
Found after tx of spironolactone which is DOC for primary hyperald

A

Aldosterone adenoma

110
Q

Diffuse or focal hyperplasia of cells resembling those of normal zona glomerulosa

A

Bilateral idiopathic hyperplasia

111
Q

Clinical hallmark of hyperaldosteronism

A

hypertension

112
Q

Patterns of adrenocortical insufficiency

A

1 primary acute adrenocortical insufficiency
2 primary chronic adrenocortical insuff/Addison
3 secondary adrenocortical insuff

113
Q

Classically associated with N meningitidis septicemia, pseudomonas, h influenzae
As acute crisis after stress that results in inability of the atrophic adrenal glands to produce glucocorticoid
Massive adrenal hemorrhage

A

Waterhouse Friderichsen Syndrome

114
Q

AR hereditary disease in enzyme involved in adrenal steroid biosynthesis ie cortisol
Dec cortisol leads to inc ACTH due to absence of feedback
Adrenal hyperplasia causes precursor steroid channeled into synthesis of androgen with virilizing activity

A

Congenital adrenal hyperplasia

115
Q

Most common enzyme defect causing CAH is

A

21 hydroxylase deficiency 90% assoc with CYP21A2

116
Q

CAH with bilateral hyperplasia is assoc with:
esp in those with
salt losing 21 hydroxylase def
Charac by incomplete migration or chromaffin cell to center of gland
pronounced intermingling of nests of chromaffin and cortical cell

A

Adrenomedullary dysplasia

117
Q

Masculinization in females, clitoral hypertrophy, pseudohermaphroditism oligomen, hirsutism, acne
In males enlargement of external genitalia precoc pub and oligospermia

A

21 hydroxylase deficiency

118
Q

Form of CAH with mineralocorticoid activity
Sodium retention
Hypertension

A

11B hydroxylase

119
Q

The adrenal cortex can secrete excess androgen in either two of settings

A
Adrenocortical neoplasm (virilizing)
Congenital adrenal hyperplasia
120
Q

Dec in cortisol production results in inc androgen production due to

A

compensatory inc in ACTH secretion

121
Q

Waterhouse-Frederichsen
Sudden longterm corticosteroid therapy withdrawal
Stress in chronic adrenal insuff

A

Acute adrenal insuff

122
Q
Autoimmune adrenalitis APS1 and APS2
TB
AIDS
Mets
Amyloidosis
Fungal infec
Hemochromatosis
Sarcoidosis
A

Chronic adrenal insuff

123
Q

Progressive destruction of adrenal cortex

90% attributable to 4 disorders: AIDS, TB, Autoimmune adrenalitis, mets

A

Chronic adrenal insuff

Addison Disease

124
Q

60-70% of cases most common cause of primary adrenal insuff

Autoimmune destruction of steroid producing cells and autoantibodies to steroidogenic enzymes

A

Autoimmune adrenalitis

125
Q

Autoimm polyendocrine syndrome APS1 is caused by mutations in
Chronic mucocutaneous candidiasis, skin ab, dental enamel, nails with auto adrenalitis, hypoparathy, hypogonadism and pernicious anemia

A

Autoimmune regulator AIRE on ch21

126
Q

Protein involved in expession of tissue antigen in thymus and T cell elimination

A

AIRE

127
Q

Early adulthood combination of adrenal insuff and autoimmune thyroiditis or type1 DM

A

APS2

128
Q

TB adrenalitis is assoc with infections of

A

lung

genitourinary tract

129
Q

Any disorder of hypothalamus pituitary mets, infection infarction irradiation resulting to dec ACTH but without hyperpigmentation

A

Secondary adrenocortical insufficiency

130
Q

Clinica manif of adrenocortical insuff only manifest when at least

such as weakness, easy fatigability, GI disturbances inc ACTH and hyperpigmentation

A

90%

Adrenocortical insufficiency

131
Q

Functional adenomas are more likely

While virilizing neoplasms are

A

Assoc with hyperaldosteronism
Cushing’s

Carcinoma

132
Q

Rare neoplasms occuring at any age
Invasive, variegated, poorly demarcated with areas of necrosis and hemorrhage
Strong tendency to invade the adrenal vein and lymphatics

A

Adrenocortical carcinoma

133
Q

Neoplasms composed of chromaffin cells which synthesize and release catecholamine giving rise to surgically correctable hypertension

A

Pheochromocytoma

134
Q

Pheochromocytoma rule of 10:

Location
Laterality
Type of tumor

A

Extraadrenal: organ of Zuckerkandl and carotid body aka paraganglioma

Bilateral inc to 50%

Malignant

135
Q

Familial pheochromocytoma cases are related to mutations in

A

25% RET type2 MEN
NF1
VHL
SDHB, SDHC, SDHD mitochondrial ox phos

136
Q

Polygonal spindle shaped cells with finely granular appearance called

Capsular and vasc invasion even in benign lesion
Mere presence of mitotic lesion does not imply malig

A

Zellballen

Pheochromocytoma

137
Q

Definitive diagnosis of pheochromocytoma is clinched by

A

presence of metastases involving regional LN, Liver lung and bone

138
Q

Most common extracranial solid tumor
Infants - 5 yrs
Anywhere in SNS within brain and abdomen
Majority in retroperitoneal or adrenal medulla

A

Neuroblastoma

139
Q

Group of inherited diseases resulting in proliferative lesions of multiple endocrine organs
Younger age
Synchronous or metachronously
Multifocal
Preceeded by asymptomatic stage of endocrine hyperplasia
More aggressive and recur

A

Multiple Endocrine Neoplasia

140
Q

AD
Gene at 11q13 tsg
Parathyroid, pancreas, pituitary

A

MEN 1

141
Q

Most common manifestation of MEN 1 (80-95%)

Initial

A

Primary hyperparathyroidism
Hyperplasia
Adenoma

142
Q

Leading cause of death in MEN1
Aggressive metastatic microadenomas functional like Zollinger Ellison
Gastrinomas more likely duodenum

A

Endocrine tumor of pancreas

143
Q

Most frequent pituitary tumor in MEN 1 is a

A

Prolactin secreting microadenoma

sometimes acromegaly

144
Q

Two distinct group unified by activating mutations of RET

A

MEN 2

145
Q

Medullary carcinoma
Adrenal pheochromocytoma
Parathyroid gland hyperplasia with Primary Hyperparathyroidism

A

MEN 2A

146
Q

Distinct germline RET with single AA change
Usually involves thyroid and adrenal medulla but WITHOUT
Primary Hyperparathyroidism
More EXTRAENDOCRINE manifestation like ganglioneuroma of mucosa (GI, lips, tongue) and Marfanoid habitus overly long bones of axial skeleton

A

MEN 2B

147
Q

All persons carrying germline mutations of RET are advised to have prophylactic
to prevent inevitable development of medullary carcinoma

A

thyroidectomy