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72 Cards in this Set

  • Front
  • Back
Which is the most common location of a pituitary adenoma - Anterior or Posterior Pituitary? What is the result?
Anterior Pituitary

Excess production of hormones
What are the most commonly produced hormones by pituitary adenomas?
Prolactin

Growth Hormone

ACTH

** "PGA" **
What are the most common functional pituitary adenomas? (1st and 2nd)
1st -Prolactinomas

2nd -Growth hormone Secreting
Abnormal ↑GH before closure of epiphyses

What does this cause?

Age?
Gigantism

Children
Abnormal ↑GH after closure of epiphyses

What does this cause?

Age?
Acromegaly

Adults
Clinical:

• Growth is most conspicuous in skin and soft tissues

• Viscera (thyroid, heart, liver, and adrenals)

• Bones of the face, hands, and feet

- Hands and feet are enlarged with broad, sausage-like fingers
Acromegaly

(↑GH in adults)
Clinical:

• Bone density may be increased (hyperostosis) in both the spine and the hips

• Enlargement of jaw results in protrusion (prognathism), with broadening of the lower face

• Changes develop for decades before being recognized
Acromegaly

(↑GH in adults)
Failure to suppress GH production in response to an oral load of glucose is one of the most sensitive tests for _______
Acromegaly
Dx of Gigantism/Acromegaly:

- Persistently elevated levels of GH stimulate the hepatic secretion of ______, which causes many of the clinical manifestations***
IGF-1 (somatomedin C)
What is the "mass effect" regarding pituitary adenomas?
Larger lesions typically extend superiorly through the diaphragm sella into the suprasellar region, where they often compress optic chiasm and adjacent structures
Lateral expansion of pituitary tumor may compress CN VI (abducens n.) and lead to _______
lateral rectus palsy
Why would visual disturbances be a likely first sign of pituitary adenoma?
Mass effect

Compression of the optic chiasm
What are "invasive pituitary adenomas"?
Infiltrate of neighboring tissues:

-Cavernous and sphenoid sinuses
-dura
-brain itself

(Often have ↑ Intracranial Pressure)
What is the name of a cyst on the anterior pituitary gland?
Craniopharyangioma - Rathke Cleft Cyst
- Mass effect of embryologic remnant, grows and accumulates

**Gooey material, “machine oil” = BUZZWORD**

- Forms an expanding mass arising in the sella turcica -most are suprasellar
Craniopharyangioma - Rathke Cleft Cyst
Name the most common lesions of the posterior pituitary gland (2)
**SIADH**
(Inappropriately high levels of ADH)

**Diabetes insipidus**
(deficiency of ADH)
- Characterized by polyuria due to an inability of the kidney to resorb water properly

- Pts who can drink water can generally compensate for urinary losses

- Pts who are obtunded, bedridden, or otherwise limited in their ability to obtain water may develop life-threatening dehydration
**Diabetes insipidus**
(deficiency of ADH)
Clinical:

- Causes resorption of excessive amounts of free water, resulting in hyponatremia

- Clinical manifestations are dominated by hyponatremia, cerebral edema, and resultant neurologic dysfunction
**SIADH**
(Inappropriately high levels of ADH)
How much ischemia can the anterior pituitary tolerate before hyopituitarism develops?
>75% damage --> hyopituitarism
- Post-partum necrosis of anterior pituitary

- Most common form of clinically significant ischemic necrosis of the anterior pituitary

- Result of obstetric hemorrhage or shock
Sheenan Syndrome
- During pregnancy the anterior pituitary enlarges to almost twice its normal size

- This expansion is not accompanied by an increase in blood supply from the low-pressure venous system, thus-->there is relative anoxia

- Further reduction in blood supply caused by obstetric hemorrhage or shock may precipitate infarction of the anterior lobe
Sheenan Syndrome
You are looking at a slide of anterior pituitary tissue and see an infarcted necrotic area.

You also note ghost architecture

What is going on?
Sheenan Syndrome
When hyperthyroidism is secondary to hormone leakage out of a non-hyperactive gland it is called ______
thyrotoxicosis
What are the most common causes of hyperthyroidism? (4)
• Diffuse hyperplasia associated with Graves disease – 85% of cases

• Administration of exogenous thyroid hormone

• Hyperfunctional multinodular goiter

• Hyperfunctional adenoma of thyroid
What is the earliest and most consistent sign of hyperthyroidism?
**Cardiac Signs**
Tachycardia, palpitations, cardiomegaly, arrythmias (a-fib)
What is the skin like in a patient with hyperthyroidism?
Warm, moist and flushed

(d/t ↑ blood flow and peripheral VD)
A patient presents with tachycardia, a wide, staring gaze, tremors, flushed skin and weight loss with a normal diet. What are you thinking?
Hyperthyroidism
• Associated with:

- Dietary iodine deficiency and endemic goiter

- Biosynthetic defect in hormone synthesis


Clinical features
Impaired development of the skeletal system and CNS:
- Short stature
- Coarse facial features
- Protruding tongue
- Wide set eyes
- Severe MR
- Umbilical hernia
Cretinism

(Hypothyroidism)
What is the most common manifestation of thyroid disease?
Goiter/Enlargement
Diffuse vs. Multinodular Goiters:

- Diffuse involvement of entire gland without nodularity

- Enlarged follicles are filled with colloid–colloid goiter

- Endemic – areas where low levels of iodine—see in mountainous areas – Alps, Andes, Himalayas

- Sporadic – peak incidence in young adult women
Diffuse (Simple) Goiter
Diffuse vs. Multinodular Goiters:

Causes:

• Ingestion of substances that interfere with hormone synthesis (veggies & plants)

• Hereditary enzymatic defects

• Most related to iodine use/dietary cause
Diffuse (Simple) Goiters

(may not be huge or noticeable)
- Most long standing simple goiters are ______
multinodular
Diffuse vs. Multinodular Goiters:

- May be nontoxic or may induce thyrotoxicosis—HIGH TH PRODUCTION*

• Produce most extreme thyroid enlargement

Clinically:
• Signs and symptoms of mass

• Occasionally thyrotoxicosis – rarely hypothyroidism
Multinodular Goiters
Diffuse vs. Multinodular Goiters:

Micro:

- See VERY LARGE follicles
Multinodular Goiters
- Terminal event (death)= infiltration of vessels**

—infiltration of carotids, etc

- Usually not d/t mets, more so mortality is d/t local structure involvement/destruction
Thyroid Carcinoma
____ is mutated in most families with multiple endocrine neoplasia type II (Men II)
RET gene
What is the major risk factor for Thyroid Carcinoma?
Exposure to ionizing radiation
What is the triad seen in multiple endocrine neoplasia type II (Men II)
1) medullary thyroid cancer

2) pheochromocytoma

3) hyperparathyroidism
• May present as asymptomatic thyroid nodule, or as a mass in cervical LN

• Clinical Manifestations:

- Hoarseness, dysphagia, cough, dyspnea—all suggest advanced disease
Papillary Thyroid Carcinoma
What is the most common form of thyroid carcinoma?
Papillary
Which thyroid carcinoma is increased in areas with iodine deficiency?
Follicular
(10-20% thyroid carcinomas)
Which two forms of thryoid carcinoma have the worst prognosis?
Medullary

Anaplastic
This type of thyroid neoplasm has an awful prognosis.

• Neuroendocrine neoplasms:
- Secrete calcitonin and possibly other products

• May present as single or multiple thyroid nodules involving both lobes
Medullary
(5% thyroid carcinomas)
What is the difference between Sporadic and Familial Medullary thyroid Carcinomas?
Sporadic = one lobe

Familial = bilateral and multicentric
Which thyroid carcinoma has an awful prognosis.

• Aggressive, undifferentiated tumors of follicular epithelium

• Most common in elderly; peak age >60

• Lymphoma link

• Poor prognosis with death usually 2° to local aggressive growth

• Women >men
Anaplastic
(<5% thyroid carcinomas)
- Activity of glands is controlled by level of free ionized calcium in blood

↓ levels of ionized calcium stimulate synthesis and secretion of this gland's main hormone
Parathyroid Glands --> secrete PTH
- Derived from pharyngeal pouches

- Usually four glands (upper and lower poles of each thyroid lobe)

- 10% of population only have 2 or 3 glands

- Yellow-brown, ovoid gland

- Two cell types: chief cell (mostly) and oxyphil cell
Parathyroid Glands
What is the most common cause of the loss of the Parathyroid Glands?

****
**Thyroidectomy = difficult because often also take out PT accidentally

Pt. develops HYPOcalcemia ! ! !
What is a congenital loss of the parathyroid glands where there is failure of development of 3rd and 4th pharyngeal pouches before the 8th week of gestation?
Di-George Syndrome
Primary Hyperparathyroidism vs. Hypoparathyroidism vs. Pseudohypoparathyroidism:

Bone disease – pain 2° to fractures due to osteoporosis

Renal disease – stones & obstructive uropathy

GI – constipation, nausea, peptic ulcers, pancreatitis, gallstones

CNS – depression, lethargy, eventually seizures

Neuromuscular – weakness, fatigue

Cardiac – aortic & mitral valve calcifications
Primary Hyperparathyroidism
Primary Hyperparathyroidism vs. Hypoparathyroidism vs. Pseudohypoparathyroidism

Neuromuscular – tetany, muscle cramps, convulsions

Mental status – irritability, psychosis

Intracranial:
Parkinsonian-like movement disorders, ­ intracranial pressure & papilledema

Ocular – calcification of lens, cataract formation

Cardiac – conduction defects which produce prolongation of QT interval
Hypoparathyroidism
Primary Hyperparathyroidism vs. Hypoparathyroidism vs. Pseudohypoparathyroidism

- Abnormalities in the PTH receptor complex and loss of responsiveness to PTH

- Causes hypocalcemia, compensatory parathyroid hyperfunction

- Short stature
- Round face
- Short neck
- Short metacarpals and metatarsals
Pseudohypoparathyroidism
A patient with short stature, a round face and short neck has an x-ray that shows shortness of 4th and 5th metacarpal bones
Pseudohypoparathyroidism
hyperparathyroidism vs. non-parathyroid disease

Labs:
Serum PTH levels are inappropriately ­↑ for level of calcium

****
hyperparathyroidism
hyperparathyroidism vs. non-parathyroid disease

Labs:
Serum PTH levels are ↓ or undetectable during hypercalcemia
non-parathyroid disease
Most common cause of primary hyperparathyroidism

Almost always solitary

Technetium-99m-sestamibi scan–area of increased uptake

Gross:
- Tan fleshy tumor

- Circumscribed, solitary mass

- Measuring 1 -3 cm in diameter

- Hemorrhagic areas are common

- Cystic changes occasionally seen

- Other three glands tend to be atrophic

Micro:
- Rim of normal parathyroid, admixed with adipose cells
Parathyroid Adenoma
You are looking at a slide of a patient with hyperparathyroidism and see:

-Rim of normal parathyroid, admixed with adipose cells
Parathyroid Adenoma
Primary Adrenal Neoplasms:

What shows up more in adults, Adenomas or Carcinomas?
Adenomas and carcinomas are equally common in adults
Primary Adrenal Neoplasms:

What shows up more in KIDS, Adenomas or Carcinomas?
Carcinomas > adenomas in children
Primary Adrenal Neoplasms:

Which has a higher level of CORTISOL, adenomas or carcinomas?
Levels of cortisol > with carcinoma than adenoma
What is the most common cause of pituitary hypersecretion in young adults leading to Cushing Syndrome?
ACTH-producing pituitary adenoma
- About 10% of endogenous Cushing syndrome

- Most common in men in 40-50’s

**Most commonly seen with**
• Small cell carcinoma of lung
• Carcinoid tumors of bronchus or pancreas
• Medullary cancer of thyroid
• Islet cell tumors of the pancreas

- Adrenals are bilaterally hyperplastic
Ectopic ACTH Production
What is the most common overall cause of Hypercortisolism (Cushing Syndrome) ?
Exogenous glucocorticoids (Rx)
Ectopic secretion of ACTH is most commonly seen with what? (4)
• Small cell carcinoma of lung

• Carcinoid tumors of bronchus or pancreas

• Medullary cancer of thyroid

• Islet cell tumors of the pancreas
A middle-aged woman presents with hypertension, hypokalemia:

Routine Panels Show:

high serum aldosterone

low serum renin
Conn Syndrome
(solitary aldosterone-secreting adenoma)
Buzzwords:

-HTN
-Hypokalemia
-Middle-aged woman
-HIGH serum aldosterone
-LOW serum renin
Conn Syndrome
(solitary aldosterone-secreting adenoma)
“Rule of 10’s”

- 10% occur in children
- 10% are bilateral
- 10% occur outside the adrenal gland
- 10% are malignant
- 10% are familial (MEN II & MEN III)
Pheochromocytoma
What is the “Rule of 10’s” regarding pheochromocytoma? (5)
- 10% occur in children

- 10% are bilateral

- 10% occur outside the adrenal gland

- 10% are malignant

- 10% are familial (MEN II & MEN III)
Pheochromocytoma is associated with _____ production and hypertension
catecholamine production and hypertension
You are looking at a slides of adrenal cortical tissue and see:

**LM**
- residual adrenal cortical tissue with darker cells on the left side

**EM**
neoplastic cells contain NEUROSECRETORY GRANULES containing CATECHOLAMINES
Pheochromocytoma
How does a pheochromocytoma patient typically present?
Episodic HTN
What is a classic EM finding for pheochromocytoma?
**neurosecretory granules***

Granules contain catecholamines