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Pathology Endocrine


copy deck
Where is the pituitary gland located
Sella turcica near optic chiasm and cavernous sinus
What are the two distinct parts of the pituitary
Anterior lobe (adenohypophysis
Posterior lobe (neurohypophysis)
What is the origin of the anterior lobe
Rathke pouch- oral cavity
What is the portal vascular system of the anterior pituitary
Transports hypothalamic hormones to the anterior pituitary
What are the major cell types in the anterior pituitary
What hormone do somatotrophs release
What hormone do lactotrophs release
What hormones do corticotrophs release
what hormone do thryotrophs release
What hormone do Gonadotrophs release
What is the origin of the posterior pituitary
Neuroectoderm--outpouching of the third ventricle with modified glial and axonal components from supraoptic and paraventricular nuclei
What hormones are produced in the hypothalamus and stored in the posterior pituitary
Vasopressin (ADH)
What are the effects of oxytocin on the human body
Contracts the pregnant uterus and lactiferous ducts in mammary glands
When is vasopressin secreted from the posterior pituitary
Decreased blood volume
Increased osmolarity
What role does vasopressin play in the kidney
Saves water by increasing permeability at collecting duct
A 42 year old white female presents with increasing nausea, vomiting, and headache for the past month. On physical exam, she has abnormal vision in the temporal fields. What is the most likely diagnosis
Pituitary adenoma
What is the abnormal visual field defect in pituitary adenomas
Bitemporal hemianopsia
What is the field defect in pituitary adenomas caused by
Compression of the optic chiasm
What do pituitary adenomas look like histologically
Uniform monoclonal polygonal cells in cords or sheets
A 28 year old African-American female presents with amenorrhea, galactorrhea, nausea, vomiting, and fatigue. What is the next step in diagnosis
Beta-human chorionic gonadotropin/ urine pregnancy test
A 28 year old African-American female presents with amenorrhea, galactorrhea, and fatigue. Beta-HCG is negative. Physical exam reveals a visual field defect. MRI shows a small lesion in the pituitary gland. Diagnosis?
What is the most common type of hyperfunctioning pituitary adenoma
What are the histologic findings consistent with prolactinoma
Lactotroph hyperplasia with secretory granules on immunohistochemical stain
A 28 year old white male in the county psychiatric hospital presents with galactorrhea. What is the most likely cause of the galactorrhea
Neuroleptic drugs
Which drugs are likely to cause galactorrhea
Reserpine (antihypertensive)
By what mechanism do most drugs cause galactorrhea
Blocking dopamine receptors and releasing inhibition of prolactin
What is the treatment for galactorrhea
Bromocriptine (dopamine agonist)
35 year old female presents with headaches and generalized aches and pains. She comments that she does not look the same as she did when she was younger, and that her voice has changed and is now deeper. On physical exam, she has a large head with protru
Growth hormone adenoma with acromegaly
What study should be ordered first to find growth hormone adenoma of the pituitary
MRI of the brain
What syndrome is caused by growth hormone adenoma of the pituitary in a child who is still growing (epiphyses have not closed)
What oncogene is associated with growth hormone adenomas
GSP oncogene
What is the treatment for growth hormone adenomas
Surgical removal of tumor or radiation
45 year old female presents with weight gain, headaches, hypertension, and menstrual abnormalities. On exam, she has a buffalo hump, truncal obesity, and abdominal striae. ACTH and cortisol are markedly elevated. Diagnosis
Cushing's disease due to corticotroph cell adenoma of pituitary
What is the syndrome that is associated with pituitary microadenoma, bitemporal hemianopsia, hyperpigmentation, and Cushing's syndrome
Nelson's syndrome
What is the cause of Nelson's syndrome
Loss of inhibitory effect of corticosteroids on a corticotroph adenoma of the pituitary. Adenoma increases in size after removal of adrenal glands for treatment of Cushing's disease
What are the symptoms of a gonadotroph adenoma of the pituitary gland
No recognizable syndrome
Decreased libido
What are the symptoms of a thyrotroph adenoma of the pituitary gland
Tachycardia, palpitations, weight loss, diarrhea
What are null-cell adenomas of the pituitary
Nonfunctional adenomas, a cause of hypopituitarism
a 38 year old woman with a history of a benign pituitary adenoma presents to the ER with a sudden excruciating headache, double vision, and weakness. Her blood pressure is 89/58. Diagnosis?
Pituitary apoplexy (sudden hemorrhage)
What is the treatment for the symptoms of hypopituitarism that occur with pituitary apoplexy
Glucocorticoids and thyroid hormones
A 28 year old female G1P1 presents to the ER with a headache, dizziness, fatigue, and low blood pressure. She had a difficult delivery 1 week ago that required transfusion. On physical exam, she appears diaphoretic and pale. Diagnosis?
Sheehan's syndrome (postpartum necrosis of the anterior pituitary)
What is the cause for Sheehan's syndrome
During pregnancy, pituitary size doubles. At delivery, hemorrhage or shock causes anoxia of the anterior pituitary
What is the treatment for Sheehan's syndrome
Give glucocorticoids due to decreased ACTH, and thyroid hormones due to decreased TSH
A 45 year old G5P5 complains of headaches, fatigue, and high blood pressure. She is obese and has a visual field defect. MRI shows cerebrospinal fluid where the pituitary gland should be. Diagnosis?
Empty sella syndrome
26 year old male with a recent history of head trauma presents with increased volume and frequency of urination, thirst, and polydipsia. UA is negative for glucose and shows low osmolarity. Diagnosis?
Central diabetes insipidus
What will serum lab tests find in patients with central DI
Increased serum sodium and osmolarity
What is the underlying cause for central DI
Damage to posterior pituitary
What hormone is lacking in central DI
What are common causes of central DI
Head trauma
What is the treatment for central DI
43 year old bipolar patient complains of polyuria, polydipsia, and increased thirst. Lab values reveal hypernatremia, serum osmolarity >290, and dilute urine. Diagnosis?
Nephrogenic DI likely due to chronic lithium exposure
What is the mechanism of nephrogenic DI
Renal tubules unresponsive to ADH
Are ADH levels increased, decreased, or normal in nephrogenic DI
What other drugs can cause nephrogenic DI
What is the treatment for nephrogenic DI
69 year old smoker is found restless and confused. Labs show hyponatremia, low serum osmolarity, and elevated ADH levels. There is cerebral edema evident on CT. Diagnosis?
Syndrome of inappropriate secretion of ADH
What is the mechanism of SIADH in a 69 year old smoker
Ectopic ADH secretion from small cell carcinoma of the lung
What is the urine like in patients with SIADH
Inappropriately concentrated urine
What are other causes of SIADH
What are treatments for SIADH
Fluid restriction
Demeclocycline--inhibits ADH effect on renal tubules
What is the dreaded complication that may occur with rapid correction of sodium levels in a patient with SIADH
Central pontine myelinolysis
4 year old child presents with headaches and bitemporal hemianopsia. On CT, the area of the sella turcica is calcified. When surgery is performed, the surgeon reports that there is fluid in the sella turcica that resembles motor oil. Diagnosis?
What is the embryologic origin of the thyroid
Pharyngeal epithelium
What thyroid hormone does the hypothalamus release
What thyroid hormone does the anterior pituitary release
What hormones do Thryoid follicules release
T3 and T4
What do parafollicular cells of the thyroid release
What is the role of thyroid hormones in the body
Increased basal metabolic rate
Beta-adrenergic effects
Bone growth
Central nervous system maturity
32 year old female presents with palpitations, nervousness, weight loss despite increased appetite, diarrhea, heat intolerance, and fine tremor of the hand. Diagnosis?
What are the common causes of hyperthyroidism
Graves' disease
Exogenous thyroid hormone
Hyperfunctional goiter or adenoma
What are the less common causes of hyperthyroidism
Struma ovarii
TSH-secreting pituitary adenoma
Choriocarcinoma/ hydatidform mole
45 year old female presents with fatigue, depression, constipation, cold intolerance, and weight gain. On physical exam, she is found to have decreased reflexes, cool skin, and brittle hair. Diagnosis
What is the most common cause of hypothyroidism
Hashimoto’s thyroiditis
What drugs can cause hypothyroidism
Lithium, Amiodarone, Propylthiouracil (PTU)
What diseases are associated with hypothyroidism
Sarcoidosis, Amyloidosis, Carpal tunnel syndrome
A 5 year old Chinese child presents to clinic with mental retardation, short stature, and umbilical hernia. On exam, the child has course facial features and protruding tongue. Diagnosis?
What is cretinism caused by
Iodine deficiency in children
What does iodine deficiency cause in adults
45 year old white female presents with fatigue, weight gain, and depression. She recalls that a few weeks ago, she felt very nervous, jittery, and had palpitations. Lab values show that her TSH is elevated and T3 and T4 are decreased. What is the most li
Hashimoto’s thyroiditis
What physical examination finding of the thyroid is associated with Hashimoto’s thyroiditis
Rubbery, nontender thyroid with scalloped borders
What is the typical presentation of Hashimoto’s thyroiditis
Transient hyperthyroidism followed by chronic hypothyroidism
What is the cause of Hashimoto’s thyroiditis
Autoimmune destruction of the thyroid gland
What are the histologic features of Hashimoto’s thyroiditis
Diffusely enlarged thyroid with mononuclear inflammatory cell infiltrate, lymphoid follicles, and Hurthle cells
What human leukocyte antigen (HLA) type is associated with Hashimoto’s thyroiditis
What are some of the autoantibodies (AB) associated with Hashimoto’s thyroiditis
Thyroid peroxidase AB, Antimicrosomal AB, Anti-TSH receptor AB
What other autoimmune diseases are seen in patients with Hashimoto’s thyroiditis
Systemic lupus erythematosis (SLE), Rheumatoid arthritis (RA), Sjogren’s syndrome, Pernicious anemia, Autoimmune adrenalitis, Type I diabetes
For what type of cancers are people with Hashimoto’s thyroiditis at higher risk
B-cell lymphomas of the thyroid gland
A 35 year old female presents with fatigue and pain in the neck, jaw, and throat. She has symptoms of hypothyroidism and had symptoms of hyperthyroidism 1 week ago. She reports that she just recovered from an URI. Diagnosis?
deQuervain’s thyroiditis
What are other names for deQuervain’s thyroiditis
Subacute granulomatous thyroiditis
What makes deQuervain’s thyroiditis unique
Painful thyroid and post-URI
What viruses have been associated with deQuervain’s thyroiditis
Mumps, Coxsackie virus, Adenovirus
What HLA type is associated with deQuervain’s thyroiditis
What does deQuervain’s thyroiditis show microscopically
Multinucleate giant cells, granulomatous inflammation
35 year old postpartum female presents with palpitations, tachycardia, fatigue, and tremor. Labs show increased T3 and T4 with decreased TSH. The thyroid is slightly enlarged and a biopsy shows many small lymphocytes. Diagnosis?
Subacute Lymphocytic thyroiditis
What are other names for subacute lymphocytic thyroiditis
Silent or painless thyroiditis
What HLA types are associated with subacute Lymphocytic thyroiditis
What rare cause of thyroiditis is characterized by extensive fibrosis of the thyroid gland
Reidel’s thyroiditis
26 year old female presents with weight loss, palpitations, anxiety, and thinning of hair. On physical exam, she has tachycardia, exophthalmos, increased reflexes, and moist skin. Diagnosis
Hyperthyroiditis--Grave's disease
What is the most common cause of endogenous hyperthyroidism
Grave's disease
What is the triad of Grave's disease
Ophthalmic pathology (exophthalmos)
Pretibial myxedema

Grave's makes you HOP
What is pretibial myxedema
Skin that overlies shins is thick and indurated, resembling an orange peel
What are other abnormal physical examination findings associated with Grave's disease
Bruit over enlarged thyroid
Lid lag
Weak extraocular muscles
What is the cause of Grave's disease
Stimulating autoantibody to TSH receptor
What type of immunoglobin is the autoantibody in Grave's disease
What HLA types are associated with Grave's disease
HLA-DR3 and HLA-B8
What other diseases are commonly found in people with Grave's disease
Systemic lupus erythematosus
Pernicious anemia
Diabetes mellitus type I
Addison's disease
What is the morphology of the thyroid gland in Grave's disease
Diffusely enlarged gland, with hypertrophy and hyperplasia
What lab abnormalities are seen in Grave's disease
Increased T3 and T4
Decreased TSH
What is the treatment for Grave's disease
Ablation by radiation
Surgical removal
20 year old female presents with a painless lump in her neck. She denies any symptoms of palpitations, racing heart, or nervousness. On physical exam, the thyroid is diffusely enlarged without nodularity. Diagnosis
Simple diffuse (nontoxic) goiter
20 year old female presents with a painless lump in her neck. She denies any symptoms of palpitations, racing heart, or nervousness. On physical exam, the thyroid is irregularly asymmetrically enlarged with palpable nodularity. Diagnosis?
Multinodular goiter
What syndrome consists of hyperthyroidism with goiter but lacks the ophthalmic and dermatologic characteristics of Grave's disease
Plummer syndrome
What is the cause of goiter
Impaired synthesis of thyroid hormones
What dietary deficiency causes the impaired synthesis of thyroid hormones in goiter
Iodine deficiency
A 36 year old woman presents with a painless lump in her neck. On exam, there is a discrete solitary mass. Diagnosis
Thyroid adenoma
True or False? The vast majority (>90%) of discrete solitary masses of the thyroid are benign
68 year old man presents with hoarse voice, dysphagia, and cough. He mentions he was exposed to radiation from the Chernobyl plant explosion several decades ago. On physical exam, a small solitary mass is detected in the thyroid. Diagnosis?
Thyroid carcinoma
What are some features that make a lesion of the thyroid suspicious for cancer
Solitary lesion
Radiation history
Younger patient
Cold nodule
When a solitary thyroid lesion is detected, what is the next step in diagnosis
Fine needle aspiration
What is the most common type of thyroid cancer
Papillary carcinoma

Papillary is the most Popular
What microscopic findings distinguish papillary carcinoma from other types
Psammoma bodies
Orphan Annie nuclei
Nuclear grooves
What familial syndromes have an increased risk of developing thyroid papillary carcinomas
Cowden syndrome (familial goiter/ skin hamartomas)
Familial adenomatous polyposis
Gardner’s syndrome
What is the second most common type of thyroid carcinoma (10-20%)
Follicular carcinoma
What is seen microscopically in thyroid follicular carcinoma
Increased follicles with colloid, some Hurthle cells
What is the third most common type of thyroid carcinoma (5%)
Medullary carcinoma
What cell type is associated with thyroid medullary carcinoma
Parafollicular C cells
What is seen microscopically in thyroid medullary carcinoma
Amyloid deposits
Spindle-shaped cells
Calcitonin by immunostain
What substance do the thyroid parafollicular C cells normally secrete
What other substances do thyroid medullary carcinomas secrete besides large amounts of calcitonin
Carcino-embryonic antigen (CEA)
Vasoactive intestinal peptide (VIP)
What familial syndromes are associated with an increased risk of medullary thyroid carcinoma
Multiple endocrine neoplasia (MEN) IIA and IIB
What are the three most important things to remember about medullary thyroid carcinoma
MEN syndromes
C-cells/ Calcitonin.

MED student named MAC
What is the least common type of thyroid carcinoma
Anaplastic carcinoma
What is unique about anaplastic thyroid carcinoma
Very aggressive
Poorly differentiated microscopically
Metastasizes to lungs
A 14 year old female presents with intermittent draining from a midline swelling in her anterior neck. She reports that it often moves with her tongue. Diagnosis?
Thyroglossal duct cyst
A 13 year old female presents with a left-sided anterior neck swelling. Most likely diagnosis?
Branchial cleft cyst
A 14 year old White female presents with weight loss of 10lb in the last few weeks, fatigue, polydipsia, polyphagia, and polyuria. On exam, the patient appears dehydrated and is breathing rapidly and deeply. She has fruity odor on her breath. Diagnosis?
DM type I
What life threatening condition is associated with DM type I
Diabetic ketoacidosis
What is the defect associated with DM type I
Failure of insulin synthesis by pancreatic beta cells
What causes the failure of insulin synthesis
External environmental factors causing insulitis (autoimmune destruction of pancreas), coupled with a genetic predisposition
What microscopic change is seen in patients with insulinitis
Lymphocytic infiltration of pancreatic islets
What HLA types are associated with type I DM
What are symptoms of DKA
Fruity odor of breath, hyperglycemia, and Kussmaul’s breathing
What is the biochemical significance of DKA
Increased catabolism of fats, producing ketone bodies
What are the three ketone bodies produced from DKA
Beta-hydroxybutyric acid
Acetoacetic acid
What is the rapid and deep breathing found in DM type I known as
Kussmaul’s breathing
What is the first line treatment in DM type I
Insulin and hydration
What sinus/ respiratory infections are patients with DKA at risk of contracting
Mucor and rhizopus infections
A 56 year old black female presents with polyuria. She is moderately obese and has acanthosis nigricans on her posterior neck. Lab values show fasting hyperglycemia and HBA1c of 8.1. Diagnosis?
DM type II
What is the mechanism of DM type II
Increased insulin resistance
What modifiable risk factor is associated with DM type II
Which type of diabetes is associated more strongly with family history of diabetes
DM type II
What is the first-line of treatment for a borderline diabetic
Diet and exercise
What is the treatment of choice for a borderline diabetic if diet and exercise do not lower fasting glucose levels
Oral hypoglycemic agents
What are the lab values that lead to a diagnosis of DM
Fasting glucose >126, Random glucose >200, HBA1c > 7
What is microscopically characteristic of DM type II
Fibrosis and hyalinization (amyloid) of islets
What organ systems are especially at risk with DM types I and II
Cardiovascular, Kidney, Eye, Nervous system
What are patients with DM at higher risk for which affects the blood vessels and coronary arteries
What are the complications from atherosclerosis that increase morbidity and mortality in diabetics
Myocardial infarction and peripheral vascular disease
What skin finding is associated with atherosclerosis
What nervous system changes are consequences of long-standing DM
Peripheral neuropathy
What gastrointestinal consequence of DM results in nausea, vomiting, and early satiety after meals
What is the earliest sign of DM in the kidney
Increased thickening of the BM
What pathology of the kidney is seen later in the course of DM
Kimmelstiel-Wilson nodules (glomerulosclerosis)
What are Armanni-Ebstein lesions of the kidney
Deposition of glycogen from prolonged hyperglycemia
45 year old white male presents with new-onset diabetes and abdominal pain. His skin is tan and he has hepatosplenomegaly. He also complains of impotence and setting off metal detectors. Diagnosis?
Hemochromatosis (bronze diabetes)
What is the cause of Hemochromatosis symptoms
Defect in iron metabolism with increased iron (ferritin) deposition in tissues resulting in multiorgan damage
What is the hereditary inheritance pattern of hemochromatosis
Autosomal recessive
What is the secondary cause of hemochromatosis
Frequent blood transfusions
What are the elevated lab values in hemochromatosis
Increased ferritin, increased transferrin, and increased glucose
What are patients with hemochromatosis at greater risk of developing
CHF and hepatocellular carcinoma
What are the treatments for hemochromatosis
Phlebotomy and deforoxamine
69 year old patient presents with new-onset diabetes, weight loss, stomach and back ache, anorexia, and fatigue. His skin appears slightly yellow on exam and he has a painlessly enlarged gallbladder. Diagnosis?
Pancreatic carcinoma
What is the name of the sign associated with painless enlarged gallbladder
Courvoisier’s sign
What is the syndrome of migratory thrombophlebitis that often accompanies pancreatic or other adenocarcinoma
Trousseau’s syndrome
What are the two parts of the adrenal glands
Adrenal cortex
Adrenal medulla
What hormones are produced by the zona reticularis
Sex steroids (estrogens/androgens)
What hormones are produced by the zona fasciculata
Glucocorticoids (cortisol)
What hormones are produced by the zona glomerulosa
Mineralocorticoids (aldosterone)
What hormones are produced by the adrenal medulla
Catecholamines (epinephrine)
A 42 year old female presents with hypertension, weight gain, new-onset diabetes, easy bruising, and menstrual abnormalities. On physical exam, she has truncal obesity, moon facies, an accumulation of fat on the posterior neck, and abdominal striae. What
Cushing's syndrome
What is the hormone abnormality causing Cushing's syndrome
Excess cortisol production
What is the technical term for the accumulation of fat on the posterior neck in Cushing's syndrome
Buffalo hump
What is the most common cause of Cushing's syndrome
Exogenous steroid use (Drugs)
What are the causes of endogenous Cushing's syndrome
Hypothalamic/ pituitary origin
Adrenal origin
Ectopic ACTH from nonendocrine neoplasm
What is Cushing's disease
Cushing's symptoms associated with pituitary adenoma
What lab values are associated with Cushing's disease
Increased ACTH (hypersecretion)
Suppresion of ACTH with high levels of dexamethasone
What lab values distinguish an adrenal origin of Cushing's syndrome from other causes
Decreased ACTH (feedback)
Increased cortisol
What are some adrenal causes for Cushing's syndrome
Adrenal adenoma
Adrenal hyperplasia
Adrenal carcinoma
What lab values suggest that Cushing's syndrome is due to ectopic ACTH from a nonendocrine neoplastic origin
Increased ACTH
No suppresion of ACTH with any level of dexamethasone
What neoplasms frequently secrete ACTH-like substances
Small cell carcinoma of the lung
Carcinoid tumors
Medullary carcinomas of the thyroid
A 45 year old white male presents with hypertension, water retention, muscle wasting, and paresthesias. Lab values show hypokalemia, hypernatremia, and a metabolic alkalosis. What is the most likely diagnosis
What is the most likely cause of primary hyperaldosteronism
Adrenal adenoma (aldosterone secreting)
What is the syndrome associated with aldosterone-secreting adrenal adenoma
Conn's syndrome
What lab values support a diagnosis of Conn's syndrome
Increased Na+
Decreased K+ and renin
What is the treatment for Conn's syndrome
By what mechanism does spironolactone work
Inhibits aldosterone at distal tubule, spares K+
What is the cause of secondary aldosteronism
Renal failure
Congestive heart failure
What is the underlying cause of secondary aldosteronism
Activation of the renin-angiotensin system
What is the distinguishing lab value that separates secondary aldosteronism from primary aldosteronism
Renin is secreted in secondary aldosteronism
A term female infant is born with ambiguous genitalia and low blood pressure. Labs show increased serum potassium and hyponatremia. What is the most likely diagnosis
Congenital adrenal hyperplasia
What is the most common form of congenital adrenal hyperplasia
21-hydroxylase deficiency
Which enzymatic step in the cortisol pathway is 21-hydroxylase involved in
Progesterone conversion to 11-deoxycorticosterone
What is the consequence of 21-hydroxylase deficiency
Decrease in cortisol
Increased ACTH (to raise cortisol levels)
Adrenal hyperplasia
What is the treatment for congenital adrenal hyperplasia
Cortisol and mineralocorticoids if needed
A 62 year old female presents with hypotension, nausea, vomiting, and increased pigmentation of the skin. Labs show decreased serum sodium, chloride, glucose, and bicarbonate, but increased potassium. Diagnosis?
Addison's disease
What other lab values would support the diagnosis of Addison's disease
Decreased serum cortisol and aldosterone
Increased ACTH
What are the synonyms for Addison's disease
Primary chronic adrenocortical deficiency or adrenal atrophy
What is the most common cause of Addison's disease
Autoimmune lymphocytic adrenalitis
What HLA types are associated with the autoimmune form of Addison's disease
HLA-B8 and HLA-DR3
What other syndromes are associated with Addison's disease
DM type I
Pernicious anemia
What are other less common causes of Addison's disease
Infectious- TB, Histoplasmosis, and Coccidiodes
Metastatic cancer
Why is the skin pigmentation increased in Addison's disease
Increased ACTH stimulates melanocytes
What is secondary adrenocortical deficiency
Decreased secretion of stimulatory hormones at the level of hypothalamus or pituitary gland
In secondary adrenocortical deficiency, what are the lab values
Decreased ACTH
Decreased cortisol
Decreased androgens
Normal aldosterone and melanin
What is the major difference that sets secondary adrenocortical deficiency apart from primary
ACTH level decreased
No pigmentation
What are reasons for an acute primary adrenal insufficiency
Withdrawal of exogenous steroid medicines
Adrenal hemorrhage
A 17 year old white male presents with signs of meningitis. His lumbar puncture shows meningococcemia, and he is immediately started on several IV antibiotics. Hours after his admission, his BP drops and adrenal insufficiency is diagnosed. Diagnosis?
Waterhouse-Friderichsen syndrome
What is Waterhouse-Friderichsen syndrome
Hemorrhagic necrosis of the adrenal cortex often due to meningococcemia or other infection
A 67 year old white male presents with severe hypertension, headaches, chest pain, sweating, and tremor. Work up for MI is negative, but lab values show increased urinary excretion of catecholamines and their metabolites. Diagnosis?
What is a pheochromocytoma
A tumor of chromaffin cells of the adrenal medulla
What is the 10% rule with pheochromocytomas
10% of pheochromocytomas are malignant, familial, bilateral, and extraabdominal
What familial syndromes are associated with pheochromocytomas
Neurofibromatosis 1
Von Hippel Lindau
What lab tests diagnose pheochromocytoma
Increased urine epinephrine
Increased urine metanephrine
Increased urine vanillylmandelic acid (VMA)
What is the treatment for pheochromocytoma
Surgical removal with alpha blockade (lower blood pressure) followed by beta-blocker (to oppose reflex tachycardia)
A 2 year old child presents with an abdominal mass and elevated blood pressure. Increased urine catecholamines are detected. Diagnosis?
What is a neuroblastoma
Malignant tumor of childhood that originates in adrenal medulla; most common solid tumor in young children
What oncogene is often amplified in neuroblastoma
A 65 year old White male presents with diarrhea, cutaneous flushing, asthmatic wheezing, and chest pain. ECG shows right-sided valvular disease. Diagnosis?
Carcinoid syndrome
What are the symptoms of carcinoid syndrome
About 70% of carcinoid syndrome patients experience flushings of the face and neck. Other symptoms may include abdominal pain, cyanosis, diarrhea, erectile dysfunction, fever, heart damage, skin lesions, and wheezing
What causes carcinoid syndrome
Neuroendocrine tumor, usually in digestive tract, releasing excessive amounts of neuroendocrine hormones into the circulation
What is the most common site of carcinoid tumor
What do carcinoid tumors secrete
Serotonin (5-hydroxytryptamine [HT]), histamine, and prostaglandins
What do lab values show in patients with carcinoid tumors
Increased 5-hydroxy indole acetic acid (HIAA)(serotonin metabolite)
What is the rule of one-third with carcinoid tumors
One-third of carcinoids are multiple and one-third metastasize
What is the treatment for carcinoid tumors
Surgical removal and treatment with octreotide (biological agent)
What are three categories of multiple endocrine neoplasia syndromes
What is another name for MEN I
Wermer's syndrome
What chromosome is affected with MEN I
What organs/glands are affected by MEN I

MEN I involves the three Ps
What pathology is seen in the parathyroid in those with MEN I
Hyperparathyroidism (adenoma)
What pathology is seen in the pancreas with MEN I
Islet cell tumors- VIPoma, glucagonoma, insulinoma, and gastrinoma (Zollinger-Ellison)

One (MEN I) VIP has a GIG
What pathology is seen in the pituitary in MEN I
What is the eponym for MEN IIA
Sipple's syndrome

Take 2 sipps
What organs/glands are affected by MEN IIA
Medulla (adrenal)--pheochromocytoma
Medullary carcinoma of the thyroid
Parathyroid (hyper)

MEN IIA has problems with a pair of Medullas and a parathyroids!
What glands/organs ar affected by MEN III (MEN-IIB)
Medullary carcinoma of thyroid
Adrenal medulla

2B a strong MAN is equal to 3 (III) MEN
What are the unique things about MEN III
What is MEN III lacking that the other MEN syndromes have
What protooncogene is responsible for the MEN III syndrome
RET oncogene
Which MEN syndrome deals with parathyroid, pancreas, and pituitary
Which MEN syndrome has neuromas

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