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ENDOCRINE

SYSTEM

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PITUITARY GLAND
Pituitary adenomas and hyperpituitarism
Hypopituitarism
Posterior pituitary syndromes
Hypothalamic suprasellar tumors

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PITUITARY ADENOMA
Found in adults(35 to 60
years of age)
Genetic causes:
G-protein mutations
MEN1,CDKN1B,PRKAR1A,
AIP

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PITUITARY ADENOMA
Soft,well-circumscribed
lesion
Microadenoma(<1cm)
Macroadenoma(>1cm)
Composed of uniform,
polygonal cells in sheets
or cords

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PROLACTINOMA
Composed of weakly
acidophilic or
chromophobic cells

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HYPOPITUITARISM
Decreased secretion of pituitary hormones
75% of parenchyma is lost or absent
Causes:
Tumors
Traumatic brain injury
Surgery or radiation
Pituitary apoplexy
Sheehan syndrome

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HYPOPITUITARISM
Rathke cleft cyst
Empty sella syndrome
Genetic defects
Inflammation/infections

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POSTERIOR PITUITARY SYNDROMES


Diabetes insipidus
Syndrome of inappropriate ADH secretion(SIADH)

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HYPOTHALAMIC SUPRASELLAR TUMORS


Gliomas
Craniopharyngiomas
Average 3 to 4 cm
Encapsulated and solid,cystic

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HYPOTHALAMIC SUPRASELLAR TUMORS


Adamantinomatous
craniopharyngioma
Nests or cords of
stratified squamous
epithelium
Compact lamellar
keratin
formation(diagnostic)

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HYPOTHALAMIC SUPRASELLAR TUMORS


Papillary
craniopharyngiomas
Contain solid sheets
and papillae lined by
well-differentiated
squamous epithelium
Lack keratin,
calcification, and cysts

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THYROID GLAND
Hyperthyroidism
Hypothyroidism
Thyroiditis
Graves disease
Diffuse and multinodular goiters
Neoplasms of the thyroid
Congenital anomalies

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HYPOTHYROIDISM
Cretinism
Infancy or early childhood
Cretin-Christian Christ-like
Clinical features:
Severe mental retardation
Short stature
Coarse facial features
Protruding tongue
Umbilical hernia

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HYPOTHYROIDISM
Myxedema
Older child or adult
Clinical features:
Slowing of physical and
mental activity
Generalized fatigue,apathy
Cold intolerant,overweight

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THYROIDITIS
Inflammation of the thyroid gland
Types:
Hashimoto thyroiditis
Subacute(granulomatous) thyroiditis
Subacute lymphocytic thyroiditis

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HASHIMOTO THYROIDITIS
Gradual thyroid failure
due to autoimmune
destruction of thyroid
gland
45 to 65 years of age
More common in
women(10:1 to 20:1)
Breakdown in selftolerance to thyroid
auto-antigens

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HASHIMOTO THYROIDITIS
Diffusely enlarged
Intact capsule
Extensive infiltration of
parenchyma by
mononuclear infiltrates
Hurthle cells-follicular
epithelial cells with
abundant
eosinophilic, granular
cytoplasm

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SUBACUTE (GRANULOMATOUS)
THYROIDITIS
De Quervain thyroiditis
Triggered by viral
infection
Causes thyroid pain
Chronic inflammatory
infiltrate with
multinucleated giant
cells

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SUBACUTE LYMPHOCYTIC
THYROIDITIS
painless thyroiditis
Fibrosis and Hurthle
cells are not
prominent

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GRAVES DISEASE
Triad:
Hyperthyroidism
Ophthalmopathy
Dermopathy

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GRAVES DISEASE
Breakdown in self-tolerance to thyroid auto-antigens resulting
in autoantibodies production:
Thyroid-stimulating immunoglobulin
thyroid growth-stimulating immunoglobulins
TSH-binding inhibitor immunoglobulins

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GRAVES DISEASE
Diffuse hypertrophy
and hyperplasia of
thyroid gland
Follicular epithelial cells
are tall and more
crowded than usual

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DIFFUSE NONTOXIC GOITER


Enlargement of
entire gland
without nodules
Colloid goiter
Follicular epithelium
is flattened and
cuboidal
Abundant colloid
euthyroid

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MULTINODULAR GOITER
Multilobulated
Asymmetrically enlarged
gland
Colloid-rich follicles
Follicular hyperplasia

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ADENOMAS
Solitary,spherical,
encapsulated lesion welldemarcated from
surrounding parenchyma
Uniform-appearing
follicles with colloid

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CARCINOMAS
Papillary
Follicular
Anaplastic
Medullary

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PAPILLARY CARCINOMA
Most common form
of thyroid
cancer(85%)
Solitary or multifocal
lesion
Branching papillae
Orphan Annie eye
nucleus
Psammoma bodies

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FOLLICULAR CARCINOMA
Single nodules,
well-circumscribed
or widely infiltrative
Fairly uniform cells
forming small
follicles containing
colloid

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ANAPLASTIC CARCINOMA
Undifferentiated tumors
Aggressive,65 years of
age
Pleomorphic giant cells
Sarcomatous spindle
cells

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MEDULLARY CARCINOMA
Neuroendocrine
neoplasms from
parafollicular cells
Peak in 40s to 50s
Part of MEN syndrome
Polygonal to spindleshaped cells which
may form nests,
trabeculae, and
follicles

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CONGENITAL ANOMALIES
Thyroglossal duct cyst
Midline neck mass from a
persistent thyroglossal duct
Fibrous cyst lined by
stratified squamous
epithelium or thyroid tissue

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PARATHYROID GLANDS
Hyperparathyroidism
Hypoparathyroidism
Pseudohypoparathyroidism

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PRIMARY HYPERPARATHYROIDISM
Causes:
Adenoma: 85% to 95%
Primary hyperplasia(diffuse or nodular): 5% to 10%
Parathyroid carcinoma: 1%

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FAMILIAL PRIMARY
HYPERPARATHYROIDISM
Multiple endocrine neoplasia-1(MEN-1)
inactivation of MEN-1 gene

MEN-2
mutation of RET gene

Familial hypocalciuric hypercalcemia


decreased sensitivity to extracellular calcium

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PARATHYROID ADENOMA
Solitary,soft nodule,
with a capsule
Composed of fairly
uniform, polygonal
chief cells with small,
centrally placed
nuclei

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SECONDARY
HYPERPARATHYROIDISM
Renal failure
Most common cause of secondary hyperparathyroidism
Hyperplastic parathyroid glands

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HYPOPARATHYROIDISM
Surgically induced
Autoimmune
Mutations in autoimmune regulator(AIRE)gene

Familial
Autosomal dominant-PTH precursor peptide mutation
Autosomal recessive-glial cells missing 2(GCM2)

Congenital absence

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PSEUDOHYPOPARATHYROIDISM
End-organ resistance to PTH
PTH is normal or elevated
Hypocalcemia
hyperphosphatemia

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ENDOCRINE
PANCREAS
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ENDOCRINE PANCREAS
Diabetes mellitus
Group of metabolic disorders sharing the common
underlying features of hyperglycemia
Diagnosis:
RBG >200mg/dL with classic s/s
FBG >126mg/dL on more than one occasion
OGTT >200mg/dL after 2 hrs

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TYPE 1 DIABETES MELLITUS


Autoimmune disease
Islet cell destruction caused by immune effector cells
reacting against B-cell antigens

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TYPE 2 DIABETES MELLITUS


Causes:
Decreased response
of peripheral tissues
to insulin
(insulin resistance)
B-cell dysfunctioninadequate insulin
secretion

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PANCREATIC ENDOCRINE NEOPLASMS


Insulinoma
Gastrinoma
Rare tumors
Glucagonoma
Somatostatinoma
VIPoma
Pancreatic carcinoid tumors

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INSULINOMA
B-cell tumor
Most common
Small,solitary nodule
Monotonous
neoplastic cells
Abundant amyloid
deposition

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GASTRINOMA
Part of Zollinger-Ellison
syndrome(pancreatic
islet cell lesions,
hypersecretion of
gastric acid, severe
peptic ulceration
Histologically bland

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RARE PANCREATIC NEOPLASMS


Glucagonomas
A-cell tumors,increased glucagon
Somatostatinomas
D-cell tumors
VIPoma
Watery diarrhea,hypokalemia,achlorhydria, or WDHA syndrome
Carcinoid tumors
Produces serotonin

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ADRENAL GLANDS
Cushing syndrome
Elevated glucocorticoid levels
Diffuse hyperplasia
Vacuolated lipid-rich cells

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PRIMARY ADRENOCORTICAL
ADENOMAS
May be malignant or benign
Common in women(30s to 50s)
Carcinomas tend to be larger

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ALDOSTERONE-PRODUCING
ADENOMAS
Solitary,small,wellcircumscribed lesions
Bright yellow with lipidladen cortical cells
Spironolactone bodieseosinophilic,laminated
cytoplasmic inclusions

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ADRENOCORTICAL INSUFFICIENCY
Hypofunction
Causes:
Primary adrenal disease(primary hypoadrenalism)
ACTH deficiency(secondary hypoadrenalism)

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WATERHOUSE-FRIDERICHSEN
SYNDROME
Overwhelming bacterial
infection
(Neisseria meningitides)
Rapidly progressive
hypotension leading to
shock
DIC
Massive bilateral adrenal
hemorrhage
Adrenals are converted
to sacs of clotted blood

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ADRENOCORTICAL NEOPLASMS
Adrenocortical adenoma
Well-circumscribed
nodular lesion
Yellow to brown color
Neoplastic cells are
vacuolated due to
intracytoplasmic lipid

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ADRENOCORTICAL CARCINOMAS
Large,invasive lesions
Composed of welldifferentiated cells to
bizarre,monstrous giant
cells

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ADRENAL MEDULLA
Pheochromocytoma
Small,circumscribed
lesions to large
hemorrhagic masses
Polygonal to spindleshaped chromaffin
colored chief cells,
clustered with
sustentacular cells
into small nests or
alveoli(zellballen)

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MULTIPLE ENDOCRINE NEOPLASIA


Type 1
Wermer syndrome
Involves parathyroid,pancreas,pituitary
Primary hyperparathyroidism-most common manifestation
Pancreas: endocrine tumors
Pituitary: prolactinoma

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MULTIPLE ENDOCRINE NEOPLASIA


Type 2
MEN-2A
Sipple syndrome
Pheochromocytoma,medullary carcinoma,parathyroid
hyperplasia
MEN-2B
Pheochromocytoma,medullary carcinoma,neuromas
Familial medullary thyroid cancer
Variant of MEN-2A

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