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336 Cards in this Set
- Front
- Back
- 3rd side (hint)
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Where is the pituitary gland located
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Sella turcica near optic chiasm and cavernous sinus
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What are the two distinct parts of the pituitary
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Anterior lobe (adenohypophysis
Posterior lobe (neurohypophysis) |
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What is the origin of the anterior lobe
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Rathke pouch- oral cavity
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What is the portal vascular system of the anterior pituitary
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Transports hypothalamic hormones to the anterior pituitary
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What are the major cell types in the anterior pituitary
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Somatotrophs
Lactotrophs Corticotrophs Thyrotrophs Gonadothrophs |
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What hormone do somatotrophs release
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GH
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What hormone do lactotrophs release
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Prolactin
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What hormones do corticotrophs release
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ACTH
MSH |
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what hormone do thryotrophs release
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TSH
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What hormone do Gonadotrophs release
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FSH
LH |
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What is the origin of the posterior pituitary
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Neuroectoderm--outpouching of the third ventricle with modified glial and axonal components from supraoptic and paraventricular nuclei
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What hormones are produced in the hypothalamus and stored in the posterior pituitary
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Oxytocin
Vasopressin (ADH) |
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What are the effects of oxytocin on the human body
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Contracts the pregnant uterus and lactiferous ducts in mammary glands
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When is vasopressin secreted from the posterior pituitary
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Decreased blood volume
Increased osmolarity |
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What role does vasopressin play in the kidney
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Saves water by increasing permeability at collecting duct
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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
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Pituitary adenoma
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What is the abnormal visual field defect in pituitary adenomas
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Bitemporal hemianopsia
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What is the field defect in pituitary adenomas caused by
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Compression of the optic chiasm
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What do pituitary adenomas look like histologically
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Uniform monoclonal polygonal cells in cords or sheets
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A 28 year old African-American female presents with amenorrhea, galactorrhea, nausea, vomiting, and fatigue. What is the next step in diagnosis
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Beta-human chorionic gonadotropin/ urine pregnancy test
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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?
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Prolactinoma
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What is the most common type of hyperfunctioning pituitary adenoma
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Prolactinoma
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What are the histologic findings consistent with prolactinoma
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Lactotroph hyperplasia with secretory granules on immunohistochemical stain
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A 28 year old white male in the county psychiatric hospital presents with galactorrhea. What is the most likely cause of the galactorrhea
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Neuroleptic drugs
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Which drugs are likely to cause galactorrhea
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Haloperidol
Reserpine (antihypertensive) Phenothiazines |
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By what mechanism do most drugs cause galactorrhea
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Blocking dopamine receptors and releasing inhibition of prolactin
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What is the treatment for galactorrhea
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Bromocriptine (dopamine agonist)
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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 protruding jaw, thick tongue, and overly large hands and feet. Diagnosis?
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Growth hormone adenoma with acromegaly
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What study should be ordered first to find growth hormone adenoma of the pituitary
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MRI of the brain
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What syndrome is caused by growth hormone adenoma of the pituitary in a child who is still growing (epiphyses have not closed)
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Gigantism
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What oncogene is associated with growth hormone adenomas
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GSP oncogene
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What is the treatment for growth hormone adenomas
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Surgical removal of tumor or radiation
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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
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Cushing's disease due to corticotroph cell adenoma of pituitary
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What is the syndrome that is associated with pituitary microadenoma, bitemporal hemianopsia, hyperpigmentation, and Cushing's syndrome
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Nelson's syndrome
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What is the cause of Nelson's syndrome
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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
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What are the symptoms of a gonadotroph adenoma of the pituitary gland
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No recognizable syndrome
Decreased libido Amenorrhea |
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What are the symptoms of a thyrotroph adenoma of the pituitary gland
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Tachycardia, palpitations, weight loss, diarrhea
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What are null-cell adenomas of the pituitary
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Nonfunctional adenomas, a cause of hypopituitarism
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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?
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Pituitary apoplexy (sudden hemorrhage)
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What is the treatment for the symptoms of hypopituitarism that occur with pituitary apoplexy
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Glucocorticoids and thyroid hormones
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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?
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Sheehan's syndrome (postpartum necrosis of the anterior pituitary)
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What is the cause for Sheehan's syndrome
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During pregnancy, pituitary size doubles. At delivery, hemorrhage or shock causes anoxia of the anterior pituitary
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What is the treatment for Sheehan's syndrome
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Give glucocorticoids due to decreased ACTH, and thyroid hormones due to decreased TSH
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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?
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Empty sella syndrome
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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?
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Central diabetes insipidus
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What will serum lab tests find in patients with central DI
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Increased serum sodium and osmolarity
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What is the underlying cause for central DI
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Damage to posterior pituitary
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What hormone is lacking in central DI
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ADH
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What are common causes of central DI
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Head trauma
Tumor Sarcoidosis |
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What is the treatment for central DI
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Vasopressin/desmopressin
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43 year old bipolar patient complains of polyuria, polydipsia, and increased thirst. Lab values reveal hypernatremia, serum osmolarity >290, and dilute urine. Diagnosis?
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Nephrogenic DI likely due to chronic lithium exposure
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What is the mechanism of nephrogenic DI
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Renal tubules unresponsive to ADH
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Are ADH levels increased, decreased, or normal in nephrogenic DI
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Normal
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What other drugs can cause nephrogenic DI
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Demeclocycline
Methoxyflurane |
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What is the treatment for nephrogenic DI
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Thiazides
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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?
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Syndrome of inappropriate secretion of ADH
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What is the mechanism of SIADH in a 69 year old smoker
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Ectopic ADH secretion from small cell carcinoma of the lung
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What is the urine like in patients with SIADH
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Inappropriately concentrated urine
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What are other causes of SIADH
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Pain
Narcotics Carbamazepine Oxytocin |
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What are treatments for SIADH
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Fluid restriction
Demeclocycline--inhibits ADH effect on renal tubules |
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What is the dreaded complication that may occur with rapid correction of sodium levels in a patient with SIADH
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Central pontine myelinolysis
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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?
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Craniopharyngioma
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What is the embryologic origin of the thyroid
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Pharyngeal epithelium
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What thyroid hormone does the hypothalamus release
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TRH
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What thyroid hormone does the anterior pituitary release
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TSH
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What hormones do Thryoid follicules release
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T3 and T4
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What do parafollicular cells of the thyroid release
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Calcitonin
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What is the role of thyroid hormones in the body
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Increased basal metabolic rate
Beta-adrenergic effects Bone growth Central nervous system maturity |
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32 year old female presents with palpitations, nervousness, weight loss despite increased appetite, diarrhea, heat intolerance, and fine tremor of the hand. Diagnosis?
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Hyperthyroidism
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What are the common causes of hyperthyroidism
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Graves' disease
Exogenous thyroid hormone Hyperfunctional goiter or adenoma Thyroiditis |
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What are the less common causes of hyperthyroidism
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Struma ovarii
TSH-secreting pituitary adenoma Choriocarcinoma/ hydatidform mole |
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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
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Hypothyroidism
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What is the most common cause of hypothyroidism
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Hashimoto’s thyroiditis
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What drugs can cause hypothyroidism
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Lithium, Amiodarone, Propylthiouracil (PTU)
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What diseases are associated with hypothyroidism
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Sarcoidosis, Amyloidosis, Carpal tunnel syndrome
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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?
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Cretinism
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What is cretinism caused by
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Iodine deficiency in children
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What does iodine deficiency cause in adults
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Myxedema
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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 likely diagnosis
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Hashimoto’s thyroiditis
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What physical examination finding of the thyroid is associated with Hashimoto’s thyroiditis
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Rubbery, nontender thyroid with scalloped borders
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What is the typical presentation of Hashimoto’s thyroiditis
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Transient hyperthyroidism followed by chronic hypothyroidism
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What is the cause of Hashimoto’s thyroiditis
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Autoimmune destruction of the thyroid gland
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What are the histologic features of Hashimoto’s thyroiditis
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Diffusely enlarged thyroid with mononuclear inflammatory cell infiltrate, lymphoid follicles, and Hurthle cells
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What human leukocyte antigen (HLA) type is associated with Hashimoto’s thyroiditis
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HLA-DR3 and HLA-DR5
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What are some of the autoantibodies (AB) associated with Hashimoto’s thyroiditis
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Thyroid peroxidase AB, Antimicrosomal AB, Anti-TSH receptor AB
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What other autoimmune diseases are seen in patients with Hashimoto’s thyroiditis
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Systemic lupus erythematosis (SLE), Rheumatoid arthritis (RA), Sjogren’s syndrome, Pernicious anemia, Autoimmune adrenalitis, Type I diabetes
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None
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For what type of cancers are people with Hashimoto’s thyroiditis at higher risk
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B-cell lymphomas of the thyroid gland
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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?
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deQuervain’s thyroiditis
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What are other names for deQuervain’s thyroiditis
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Subacute granulomatous thyroiditis
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What makes deQuervain’s thyroiditis unique
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Painful thyroid and post-URI
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What viruses have been associated with deQuervain’s thyroiditis
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Mumps, Coxsackie virus, Adenovirus
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What HLA type is associated with deQuervain’s thyroiditis
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HLA-B35
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None
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What does deQuervain’s thyroiditis show microscopically
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Multinucleate giant cells, granulomatous inflammation
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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?
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Subacute Lymphocytic thyroiditis
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What are other names for subacute lymphocytic thyroiditis
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Silent or painless thyroiditis
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None
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What HLA types are associated with subacute Lymphocytic thyroiditis
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HLA-DR3 and HLA-DR5
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What rare cause of thyroiditis is characterized by extensive fibrosis of the thyroid gland
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Reidel’s thyroiditis
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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
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Hyperthyroiditis--Grave's disease
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What is the most common cause of endogenous hyperthyroidism
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Grave's disease
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What is the triad of Grave's disease
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Hyperthyroidism
Ophthalmic pathology (exophthalmos) Pretibial myxedema Grave's makes you HOP |
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What is pretibial myxedema
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Skin that overlies shins is thick and indurated, resembling an orange peel
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What are other abnormal physical examination findings associated with Grave's disease
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Bruit over enlarged thyroid
Lid lag Proptosis Weak extraocular muscles |
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What is the cause of Grave's disease
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Stimulating autoantibody to TSH receptor
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What type of immunoglobin is the autoantibody in Grave's disease
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IgG
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What HLA types are associated with Grave's disease
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HLA-DR3 and HLA-B8
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What other diseases are commonly found in people with Grave's disease
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Systemic lupus erythematosus
Pernicious anemia Diabetes mellitus type I Addison's disease |
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What is the morphology of the thyroid gland in Grave's disease
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Diffusely enlarged gland, with hypertrophy and hyperplasia
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What lab abnormalities are seen in Grave's disease
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Increased T3 and T4
Decreased TSH |
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What is the treatment for Grave's disease
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PTU
Ablation by radiation Surgical removal |
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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
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Simple diffuse (nontoxic) goiter
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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?
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Multinodular goiter
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What syndrome consists of hyperthyroidism with goiter but lacks the ophthalmic and dermatologic characteristics of Grave's disease
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Plummer syndrome
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What is the cause of goiter
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Impaired synthesis of thyroid hormones
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What dietary deficiency causes the impaired synthesis of thyroid hormones in goiter
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Iodine deficiency
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A 36 year old woman presents with a painless lump in her neck. On exam, there is a discrete solitary mass. Diagnosis
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Thyroid adenoma
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True or False? The vast majority (>90%) of discrete solitary masses of the thyroid are benign
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True
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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?
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Thyroid carcinoma
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What are some features that make a lesion of the thyroid suspicious for cancer
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Solitary lesion
Radiation history Younger patient Cold nodule Male |
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When a solitary thyroid lesion is detected, what is the next step in diagnosis
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Fine needle aspiration
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What is the most common type of thyroid cancer
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Papillary carcinoma
Papillary is the most Popular |
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What microscopic findings distinguish papillary carcinoma from other types
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Psammoma bodies
Papillae--branching Orphan Annie nuclei Nuclear grooves |
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What familial syndromes have an increased risk of developing thyroid papillary carcinomas
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Cowden syndrome (familial goiter/ skin hamartomas)
Familial adenomatous polyposis Gardner’s syndrome |
None
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What is the second most common type of thyroid carcinoma (10-20%)
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Follicular carcinoma
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What is seen microscopically in thyroid follicular carcinoma
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Increased follicles with colloid, some Hurthle cells
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What is the third most common type of thyroid carcinoma (5%)
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Medullary carcinoma
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What cell type is associated with thyroid medullary carcinoma
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Parafollicular C cells
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What is seen microscopically in thyroid medullary carcinoma
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Amyloid deposits
Spindle-shaped cells Calcitonin by immunostain |
None
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What substance do the thyroid parafollicular C cells normally secrete
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Calcitonin
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What other substances do thyroid medullary carcinomas secrete besides large amounts of calcitonin
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Carcino-embryonic antigen (CEA)
Somatostatin Serotonin Vasoactive intestinal peptide (VIP) |
None
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What familial syndromes are associated with an increased risk of medullary thyroid carcinoma
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Multiple endocrine neoplasia (MEN) IIA and IIB
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None
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What are the three most important things to remember about medullary thyroid carcinoma
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MEN syndromes
Amyloid C-cells/ Calcitonin. MED student named MAC |
None
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What is the least common type of thyroid carcinoma
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Anaplastic carcinoma
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What is unique about anaplastic thyroid carcinoma
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Very aggressive
Poorly differentiated microscopically Metastasizes to lungs |
None
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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?
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Thyroglossal duct cyst
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A 13 year old female presents with a left-sided anterior neck swelling. Most likely diagnosis?
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Branchial cleft cyst
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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?
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DM type I
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None
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What life threatening condition is associated with DM type I
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Diabetic ketoacidosis
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What is the defect associated with DM type I
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Failure of insulin synthesis by pancreatic beta cells
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What causes the failure of insulin synthesis
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External environmental factors causing insulitis (autoimmune destruction of pancreas), coupled with a genetic predisposition
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What microscopic change is seen in patients with insulinitis
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Lymphocytic infiltration of pancreatic islets
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What HLA types are associated with type I DM
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HLA-DR3 and HLA-DR4
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What are symptoms of DKA
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Fruity odor of breath, hyperglycemia, and Kussmaul’s breathing
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What is the biochemical significance of DKA
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Increased catabolism of fats, producing ketone bodies
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What are the three ketone bodies produced from DKA
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Beta-hydroxybutyric acid
Acetoacetic acid Acetone |
None
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What is the rapid and deep breathing found in DM type I known as
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Kussmaul’s breathing
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What is the first line treatment in DM type I
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Insulin and hydration
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What sinus/ respiratory infections are patients with DKA at risk of contracting
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Mucor and rhizopus infections
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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?
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DM type II
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What is the mechanism of DM type II
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Increased insulin resistance
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What modifiable risk factor is associated with DM type II
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Obesity
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Which type of diabetes is associated more strongly with family history of diabetes
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DM type II
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What is the first-line of treatment for a borderline diabetic
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Diet and exercise
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What is the treatment of choice for a borderline diabetic if diet and exercise do not lower fasting glucose levels
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Oral hypoglycemic agents
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What are the lab values that lead to a diagnosis of DM
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Fasting glucose >126, Random glucose >200, HBA1c > 7
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What is microscopically characteristic of DM type II
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Fibrosis and hyalinization (amyloid) of islets
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What organ systems are especially at risk with DM types I and II
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Cardiovascular, Kidney, Eye, Nervous system
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What are patients with DM at higher risk for which affects the blood vessels and coronary arteries
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Atherosceloris
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What are the complications from atherosclerosis that increase morbidity and mortality in diabetics
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Myocardial infarction and peripheral vascular disease
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What skin finding is associated with atherosclerosis
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Xanthomas
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What nervous system changes are consequences of long-standing DM
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Peripheral neuropathy
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What gastrointestinal consequence of DM results in nausea, vomiting, and early satiety after meals
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Gastroparesis
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What is the earliest sign of DM in the kidney
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Increased thickening of the BM
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What pathology of the kidney is seen later in the course of DM
|
Kimmelstiel-Wilson nodules (glomerulosclerosis)
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What are Armanni-Ebstein lesions of the kidney
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Deposition of glycogen from prolonged hyperglycemia
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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)
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What is the cause of Hemochromatosis symptoms
|
Defect in iron metabolism with increased iron (ferritin) deposition in tissues resulting in multiorgan damage
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What is the hereditary inheritance pattern of hemochromatosis
|
Autosomal recessive
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None
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What is the secondary cause of hemochromatosis
|
Frequent blood transfusions
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What are the elevated lab values in hemochromatosis
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Increased ferritin, increased transferrin, and increased glucose
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None
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What are patients with hemochromatosis at greater risk of developing
|
CHF and hepatocellular carcinoma
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What are the treatments for hemochromatosis
|
Phlebotomy and deforoxamine
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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?
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Pancreatic carcinoma
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What is the name of the sign associated with painless enlarged gallbladder
|
Courvoisier’s sign
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What is the syndrome of migratory thrombophlebitis that often accompanies pancreatic or other adenocarcinoma
|
Trousseau’s syndrome
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What are the two parts of the adrenal glands
|
Adrenal cortex
Adrenal medulla |
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What hormones are produced by the zona reticularis
|
Sex steroids (estrogens/androgens)
|
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What hormones are produced by the zona fasciculata
|
Glucocorticoids (cortisol)
|
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What hormones are produced by the zona glomerulosa
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Mineralocorticoids (aldosterone)
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What hormones are produced by the adrenal medulla
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Catecholamines (epinephrine)
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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 is the most likely diagnosis
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Cushing's syndrome
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What is the hormone abnormality causing Cushing's syndrome
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Excess cortisol production
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What is the technical term for the accumulation of fat on the posterior neck in Cushing's syndrome
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Buffalo hump
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What is the most common cause of Cushing's syndrome
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Exogenous steroid use (Drugs)
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What are the causes of endogenous Cushing's syndrome
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Hypothalamic/ pituitary origin
Adrenal origin Ectopic ACTH from nonendocrine neoplasm |
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What is Cushing's disease
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Cushing's symptoms associated with pituitary adenoma
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What lab values are associated with Cushing's disease
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Increased ACTH (hypersecretion)
Suppresion of ACTH with high levels of dexamethasone |
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What lab values distinguish an adrenal origin of Cushing's syndrome from other causes
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Decreased ACTH (feedback)
Increased cortisol |
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What are some adrenal causes for Cushing's syndrome
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Adrenal adenoma
Adrenal hyperplasia Adrenal carcinoma |
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What lab values suggest that Cushing's syndrome is due to ectopic ACTH from a nonendocrine neoplastic origin
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Increased ACTH
No suppresion of ACTH with any level of dexamethasone |
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What neoplasms frequently secrete ACTH-like substances
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Small cell carcinoma of the lung
Carcinoid tumors Medullary carcinomas of the thyroid |
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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
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Hyperaldosteronism
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What is the most likely cause of primary hyperaldosteronism
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Adrenal adenoma (aldosterone secreting)
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What is the syndrome associated with aldosterone-secreting adrenal adenoma
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Conn's syndrome
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What lab values support a diagnosis of Conn's syndrome
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Increased Na+
Decreased K+ and renin |
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What is the treatment for Conn's syndrome
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Spironolactone
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By what mechanism does spironolactone work
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Inhibits aldosterone at distal tubule, spares K+
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What is the cause of secondary aldosteronism
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Renal failure
Congestive heart failure Cirrhosis |
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What is the underlying cause of secondary aldosteronism
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Activation of the renin-angiotensin system
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What is the distinguishing lab value that separates secondary aldosteronism from primary aldosteronism
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Renin is secreted in secondary aldosteronism
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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
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Congenital adrenal hyperplasia
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What is the most common form of congenital adrenal hyperplasia
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21-hydroxylase deficiency
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Which enzymatic step in the cortisol pathway is 21-hydroxylase involved in
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Progesterone conversion to 11-deoxycorticosterone
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What is the consequence of 21-hydroxylase deficiency
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Decrease in cortisol
Increased ACTH (to raise cortisol levels) Adrenal hyperplasia |
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What is the treatment for congenital adrenal hyperplasia
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Cortisol and mineralocorticoids if needed
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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?
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Addison's disease
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What other lab values would support the diagnosis of Addison's disease
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Decreased serum cortisol and aldosterone
Increased ACTH |
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What are the synonyms for Addison's disease
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Primary chronic adrenocortical deficiency or adrenal atrophy
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What is the most common cause of Addison's disease
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Autoimmune lymphocytic adrenalitis
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What HLA types are associated with the autoimmune form of Addison's disease
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HLA-B8 and HLA-DR3
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What other syndromes are associated with Addison's disease
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Hashimoto's
DM type I Pernicious anemia |
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What are other less common causes of Addison's disease
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Infectious- TB, Histoplasmosis, and Coccidiodes
Metastatic cancer |
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Why is the skin pigmentation increased in Addison's disease
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Increased ACTH stimulates melanocytes
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What is secondary adrenocortical deficiency
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Decreased secretion of stimulatory hormones at the level of hypothalamus or pituitary gland
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In secondary adrenocortical deficiency, what are the lab values
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Decreased ACTH
Decreased cortisol Decreased androgens Normal aldosterone and melanin |
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What is the major difference that sets secondary adrenocortical deficiency apart from primary
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ACTH level decreased
No pigmentation |
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What are reasons for an acute primary adrenal insufficiency
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Stress
Withdrawal of exogenous steroid medicines Adrenal hemorrhage |
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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?
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Waterhouse-Friderichsen syndrome
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What is Waterhouse-Friderichsen syndrome
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Hemorrhagic necrosis of the adrenal cortex often due to meningococcemia or other infection
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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?
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Pheochromocytoma
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What is a pheochromocytoma
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A tumor of chromaffin cells of the adrenal medulla
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What is the 10% rule with pheochromocytomas
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10% of pheochromocytomas are malignant, familial, bilateral, and extraabdominal
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What familial syndromes are associated with pheochromocytomas
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MEN IIa
MEN IIB Neurofibromatosis 1 Von Hippel Lindau Sturge-Webber |
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What lab tests diagnose pheochromocytoma
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Increased urine epinephrine
Increased urine metanephrine Increased urine vanillylmandelic acid (VMA) |
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What is the treatment for pheochromocytoma
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Surgical removal with alpha blockade (lower blood pressure) followed by beta-blocker (to oppose reflex tachycardia)
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A 2 year old child presents with an abdominal mass and elevated blood pressure. Increased urine catecholamines are detected. Diagnosis?
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Neuroblastoma
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What is a neuroblastoma
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Malignant tumor of childhood that originates in adrenal medulla; most common solid tumor in young children
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What oncogene is often amplified in neuroblastoma
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N-myc
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A 65 year old White male presents with diarrhea, cutaneous flushing, asthmatic wheezing, and chest pain. ECG shows right-sided valvular disease. Diagnosis?
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Carcinoid syndrome
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What are the symptoms of carcinoid syndrome
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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
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What causes carcinoid syndrome
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Neuroendocrine tumor, usually in digestive tract, releasing excessive amounts of neuroendocrine hormones into the circulation
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What is the most common site of carcinoid tumor
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Appendix
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What do carcinoid tumors secrete
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Serotonin (5-hydroxytryptamine [HT]), histamine, and prostaglandins
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What do lab values show in patients with carcinoid tumors
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Increased 5-hydroxy indole acetic acid (HIAA)(serotonin metabolite)
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What is the rule of one-third with carcinoid tumors
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One-third of carcinoids are multiple and one-third metastasize
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What is the treatment for carcinoid tumors
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Surgical removal and treatment with octreotide (biological agent)
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What are three categories of multiple endocrine neoplasia syndromes
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MEN I
MEN IIA MEN IIB or MEN III |
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What is another name for MEN I
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Wermer's syndrome
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What chromosome is affected with MEN I
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11q13
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What organs/glands are affected by MEN I
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Pancreas
Parathyroid Pituitary MEN I involves the three Ps |
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What pathology is seen in the parathyroid in those with MEN I
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Hyperparathyroidism (adenoma)
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What pathology is seen in the pancreas with MEN I
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Islet cell tumors- VIPoma, glucagonoma, insulinoma, and gastrinoma (Zollinger-Ellison)
One (MEN I) VIP has a GIG |
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What pathology is seen in the pituitary in MEN I
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Prolactinoma
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What is the eponym for MEN IIA
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Sipple's syndrome
Take 2 sipps |
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What organs/glands are affected by MEN IIA
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Medulla (adrenal)--pheochromocytoma
Medullary carcinoma of the thyroid Parathyroid (hyper) MEN IIA has problems with a pair of Medullas and a parathyroids! |
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What glands/organs ar affected by MEN III (MEN-IIB)
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Medullary carcinoma of thyroid
Adrenal medulla Neuroma 2B a strong MAN is equal to 3 (III) MEN |
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What are the unique things about MEN III
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Neuromas
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What is MEN III lacking that the other MEN syndromes have
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Hyperparathyroidism
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What protooncogene is responsible for the MEN III syndrome
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RET oncogene
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Which MEN syndrome deals with parathyroid, pancreas, and pituitary
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MEN I
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Which MEN syndrome has neuromas
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MEN III (2B)
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Which MEN syndrome has pheochromocytoma, medullary carcinoma of the thyroid, and parathyroid hyperplasia
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MEN IIA
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Which MEN syndrome does not have pheochromocytoma
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MEN I
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Which MEN syndrome does not have parathyroid hyperplasia
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MEN III
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What disease is characterized by renal cell carcinoma, pheochromocytoma, angiomas, cerebellar hemangioblastomas, and cysts of the pancreas and liver
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Von Hippel-Lindau
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What disease is characterized by cafe' au lait spots, schwannomas, meningioma, glioma, and pheochromocytoma
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Von Recklinghausen (neurofibromatosis 1)
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What disease is characterized by cavernous hemangiomas and pheochromocytoma
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Sturge-Weber syndrome
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What syndrome is associated with recurrent peptic ulcer, diarrhea, hypercalcemia, and increased gastrin levels
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Zollinger-Ellison (gastrinoma)
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What tumor is associated with Whipple triad: (1) episodic hyperinsulinemia and hypoglycemia, (2) CNS abnormality--confusion, convulsion, or coma, (3) all problems reversed with glucose administration
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Insulinoma
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In insulinoma, is the C peptide high or low
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High
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What syndrome is associated with hypoglycemia in a health-care worker who also has a low C peptide
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Munchausen syndrome (pyschiatric disorder)--giving self-insulin injections
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What tumor is associated with DM and necrolytic migratory erythema
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Glucagonoma (alpha-cell tumor)
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What tumor is associated with watery diarrhea, hypokalemia, and achlorhydria (WDHA) and is associated with increased levels of VIP
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VIPoma
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What other names are associated with the symptoms of VIPoma
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WDHA syndrome
Verner-Morrison syndrome Pancreatic cholera |
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What are struma ovarii
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Mature teratoma found in the ovary that is only composed of thyroid tissue
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How are thyroid nodules defined
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Warm = functional, avidity for isotope in the lesion same as surrounding glands (normal)
Hot = hyperfunctional, traps more isotope than surrounding glands Cold = hypofunctional, less isotope is trapped than in surrounding glands |
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This picture demonstrates what
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Papillary carcinoma of the thyroid.
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What do these pictures demonstrate
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Papillary carcinoma of the thyroid
Focus of papillary carcinoma (arrow) within normal thyroid parenchyma (left) High power view showing papillary formations of tumor cells. (right) |
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What do these pictures demonstrate
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Papillary carcinoma of the thyroid.
Papillary structures w/ enlarged nuclei Optically clear “Orphan Annie” nuclei (A) Nuclear grooves (B) Even in absence of papillae, these features are classic for papillary carcinoma of the thyroid |
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What does this picture demonstrate
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Papillary carcinoma of the thyroid.
Psammoma bodies (calcified concentrically laminated structures representing “fossilized” tips of papillae) may be present. Psammoma bodies in the thyroid are almost pathognomonic of papillary carcinoma. |
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What diseases have psammoma bodies
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Dystrophic Calcification
Meningioma Papillary Carcinoma of the Ovary Papillary Carcinoma of the Thyroid |
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What does this picture demonstrate
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Fine needle aspiration of papillary carcinoma of the thyroid
“Bubble gum” colloid and a syncytium of malignant cells, some with nuclear grooves. The ground-glass appearance is not always seen in cytologic preparations. |
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What does this picture, taken from a 55 year old woman, demonstrate
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A cold thyroid nodule-- most likely a follicular carcinoma
Encapsulated tumor with a tan-yellow cut surface and small foci of cystic change. As a general rule, the presence of a thick and irregular capsule tends to favor a follicular carcinoma over an adenoma on gross examination. |
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What does this picture demonstrate
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Follicular carcinoma of the thyroid
Focus of follicular carcinoma (arrow) surrounded by an irregular fibrous capsule and residual benign thyroid tissue. |
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What does this picture demonstrate
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Follicular carcinoma of the thyroid.
Small, closely packed neoplastic follicles containing colloid. No well-formed papillae or psammoma bodies are present. The nuclei of tumor cells do not have a ground- glass appearance or grooves as seen in papillary carcinoma. |
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What features distinguish a follicular adenoma from a follicular carcinoma?
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Invasion of the capsule or blood vessels changes the diagnosis from adenoma to carcinoma.
Extension into adjacent thyroid parenchyma and metastases are additional criteria. |
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What is the Tx and prognosis of follicular carcinomas?
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Widely invasive tumors: includes total thyroidectomy and radioactive iodine
Non-metastatic, minimally invasive tumors: may be more conservative treatment. Minimally invasive carcinomas have 70-100% 10-year survival rates, compared with 25-45% 10-year survival rates for widely invasive carcinomas. |
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What do these pictures demonstrate
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Follicular carcinoma of the thyroid.
Extent of capsular invasion is a point of contention: any degree or full thickness? Invaded vessels must lie within or beyond the capsule. |
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What does this picture demonstrate
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Medullary carcinoma of the thyroid
This is an isolated (non-familial) case of medullary carcinoma. The tumor is nodular and well circumscribed. There is no visible fibrous capsule. The cut surface is yellow-brown with fine cystic changes. |
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20% of cases of medullary carcinoma of the thyroid are familial. How do you screen the family
|
Serum calcitonin levels and RET mutations
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What does this picture demonstrate
|
Medullary carcinoma of the thyroid
Low-power: typical lobular architectural pattern of medullary carcinoma. |
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What does this picture demonstrate
|
Medullary carcinoma of the thyroid
Round/polygonal tumor cells with abundant amphophilic granular cytoplasm; some cells have clear cytoplasm. Stromal amyloid deposits, derived from calcitonin or its precursor molecules, is also present. Can be part of MEN II |
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What is the precursor lesion of medullary carcinoma of the thyroid
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C-cell hyperplasia
Amyloid but not AA or AL |
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What does this picture demonstrate
|
Papillary thyroid carcinoma metastatic to neck lymph nodes
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How common are papillary thyroid carcinomas that metastasize to cervical lymph nodes? What is
their significance? |
They occur in 33-61% of papillary carcinomas. They correlate with increased frequency of recurrences and in most series do not correlate with the overall survival.
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Discuss the familial forms of medullary carcinoma.
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Familial medullary carcinomas may occur alone, or in association with pheochromocytoma,
parathyroid hyperplasia/adenoma (multiple endocrine neoplasia [MEN] syndrome type IIA) or with pheochromocytoma, mucocutaneous neuromas and skeletal abnormalities (MEN IIB) Children of parents with MEN II or familial medullary carcinoma are being screened for mutations of the RET proto-oncogene at some institutions |
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What laboratory results would support your diagnosis of medullary carcinoma?
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Medullary carcinoma is derived from C-cells of the thyroid.
A diagnosis can be confirmed by demonstrating elevated calcitonin in the serum. |
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What does this picture demonstrate
|
Hashimoto thyroiditis
Thyroid is usually diffusely enlarged Often firm and rubbery With increasing inflammation the normal red-brown color turns into yellowish brown |
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What does this picture demonstrate
|
Hashimoto thyroiditis
Destruction and atrophy of thyroid follicles Lymphocytic and plasmacytic infiltration of the thyroid stroma with lymphoid follicles; germinal centers can also be seen. |
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What does this picture demonstrate
|
Hashimoto thyroiditis
Lymphocytic and plasmacytic infiltration of thyroid stroma with lymphoid follicles and germinal centers. |
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Discuss the pathogenesis of Hashimoto disease
|
CD4+ T cells targeted at thyroid antigens are activated after an infection or following exposure of sequestered thyroid antigens.
Activated CD4+ T cells stimulate B cells to produce antibodies and CD8+ T cells to destroy the gland parenchyma. |
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Why does the cytoplasm of Hürthle cells looks granular?
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Increased number of mitochondria
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What does this picture demonstrate
|
Hashimoto thyroiditis
Oxyphilic cell metaplasia of follicular epithelium (Hürthle cells), with pink, slightly granular cytoplasm. |
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What autoantibodies are present Hashimoto thyroiditis?
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High titers of thyroid peroxidase, antithyroglobulin antibodies and TSH receptor antibodies
Iodine transporter antibodies may also be seen |
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What is the effect of the anti-TSH receptor antibodies in Hashimoto thyroiditis?
…in Graves disease? |
In Hashimoto thyroiditis they block the action of TSH and contribute to hypothyroidism.
In Graves disease they mimic TSH and result in hyperthyroidism. |
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In what population is Hashimoto thyroiditis most prevalent?
|
Women, between 45 - 65 years old, often presenting with some degree of hypothyroidism.
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Patients with Hashimoto thyroiditis are at increased risk to develop what malignancy?
|
B-cell lymphomas
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What does this picture demonstrate
|
Adrenal cortical adenoma
Note the yellow, well-circumscribed mass with a rim of stretched, uninvolved adrenal tissue around the tumor. |
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A 22-year-old woman who presented with a 6-month history of tiredness, amenorrhea, virilism, decreased libido, and depression. The dexamethasone suppression test failed to suppress her cortisol level. Diagnosis?
|
Adrenal Cortical Adenoma
Well-circumscribed, often encapsulated, round, yellow mass May undergo hemorrhage, necrosis, or cystic degeneration Remainder of the adrenal and the contralateral gland are atrophic. |
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What does this picture demonstrate
|
Adrenal Cortical Adenoma
Clear tumor cells adjacent to a rim of non-tumorous adrenal Regular uniform large cells with ample clear cytoplasm, filled with lipid. Neoplastic cells form sheets, nests or cords. |
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What does this picture demonstrate
|
Adrenal cortical adenoma
Regular uniform large cells with ample clear cytoplasm, filled with lipid. Neoplastic cells form sheets, nests or cords. The histology cannot be used to predict what type of hormone is secreted by the tumor |
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This photograph suggests a cortical adenoma associated with Conn syndrome, not with Cushing syndrome. Why?
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The adjacent adrenal cortex is not atrophic, as would be expected in Cushing syndrome. Adenomas associated with hyperaldosteronism do not usually suppress ACTH secretion.
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What is Conn syndrome?
|
Primary hyperaldosteronism
Typical presentation: hypertension and hypokalemia Aldosterone secreted by an adrenal adenoma or adrenal hyperplasia Leads to enhanced renal tubular sodium reabsorption and, as a result, to volume expansion and hypertension Leads to potassium loss in the distal tubules, responsible for hypokalemia and kaliuresis |
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What features distinguish adrenal adenoma from carcinoma?
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The best indicator of adrenal carcinoma is metastases or spread outside of the adrenal at the time of adrenalectomy.
Histology and size of the adrenal are not reliable features. |
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What are some some causes of hypercalcemia?
|
Parathyroid-related: adenomas, hyperplasias, lithium, MEN
Malignancy-related: SCC, bone metastases, leukemia, Vitamin D-related: vitamin D intoxication High bone turnover: hyperthyroidism, immobilization Renal failure |
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What would you expect the serum levels of PTH to be like if you suspected hypercalcemia from a squamous cell cancer?
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PTH should be very low or undetectable, PTHrP should be high.
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What are the clinical manifestations of primary hyperparathyroidism?
|
Osteitis fibrosa cystica, kidney stones (“stone and bone” disease), mental status and mood changes, hyperactive reflexes, chronic pancreatitis and constipation are among the frequent manifestations.
Hypercalcemia and hypophosphatemia are the characteristic serum abnormalities. |
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What does this picture demonstrate
|
Parathyroid adenoma
Parathyroid gland with adenoma that is significantly enlarged as compared to uninvolved glands. This contrasts with parathyroid hyperplasia in which all parathyroids are usually enlarged. Parathyroid adenoma is typically an encapsulated, ovoid to round, soft tissue mass with homogeneous cut surface. About 10% occur in anomalous locations (mediastinum, pericardium, etc). |
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What is the most common bone disease associated with parathyroid adenoma?
|
Osteopenia
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What does this picture demonstrate
|
Parathyroid adenoma
Well circumscribed and encapsulated tumor. A rim of residual, compressed normal parathyroid tissue containing fat cells can often be identified adjacent to the adenoma. |
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What do these pictures demonstrate
|
Parathyroid adenoma
Chief cells (versus oxyphilic cells) are the dominant cell type in most adenomas and are often arranged in sheets, cords, nests, or glandular formations Stromal fat cells are absent within an adenoma (in normal parathyroid glands, 50% of the gland is constituted by adipocytes) |
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What two types of cells are demonstrated in this picture
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Chief cells (right) and oxyphilic cells (left) in a parathyroid adenoma
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What is demonstrated in these pictures
|
Early bone changes in hyperparathyroidism
Abnormal cavities of osteoclastic resorption in the bone trabeculae accompanied by patchy marrow fibrosis. Burrowing of an overzealous osteoclast into the bone matrix giving the lesion a tunneled (or “moth-eaten”) appearance |
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What do these pictures demonstrate
|
In advanced cases of hyperparathyroidism, multiple osteolytic lesions filled by giant cells, macrophages, plump fibroblasts and hemorrhage (called “brown tumors of hyperparathyroidism” on gross examination) are seen
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|
Discuss the etiology of primary hyperparathyroidism.
|
Primary hyperparathyroidism is a syndrome caused by excessive secretion of PTH, most commonly by a solitary parathyroid adenoma (80% of all cases), or primary hyperplasia of all parathyroid glands (15% of all cases), and rarely, a parathyroid carcinoma.
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What does this picture demonstrate
|
Pheochromocytoma
A tumor mass replaces the adrenal medulla. A thinned adrenal cortex may be seen. There may be cystic areas, hemorrhage and central scarring. |
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Pheochromocytoma: Tumor removed from the abdomen of a 23 year-old caucasian man with hypertension. What operative complications should you anticipate and try to prevent?
|
Hypertensive crisis, arrhythmias and lactic acidosis.
Start alpha-adrenergic blocker 2 weeks prior to surgery (phenoxybenzamine), to be followed by Beta-adrenergic blocker (propanolol) if needed. |
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What does this picture demonstrate
|
Pheochromocytoma
There may be cystic areas, hemorrhage and central scarring. |
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What does this picture demonstrate
|
Pheochromocytoma
The tumor may acquire a brown color when exposed to dichromate fixatives (Zenker's solution) due to oxidation of stored catecholamines. |
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What does this picture demonstrate
|
Pheochromocytoma
Low power view of the tumor showing tumor cells surrounded by fibrous septae Pleomorphism is also appreciated |
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What does this picture demonstrate
|
Pheochromocytoma
Nests of polyhedral tumor cells with granular cytoplasm and “salt and pepper” nuclei, surrounded by thin fibrovascular septa (“zellballen” pattern). There is a thin rim of normal adrenal cortical tissue adjacent to the tumor (left). Some nuclear atypia is usually present. |
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What does this picture demonstrate
|
Pheochromocytoma
This low power image shows replacement of adrenal gland by tumor arising in adrenal medulla. |
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What do these pictures demonstrate
|
Pheochromocytoma
A. Higher power showing nesting pattern of tumor cells in "Zellballen" B. Note pleomorphism of cells in B |
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|
What criterion is used to determine malignancy in pheochromocytoma
|
Metastasis is the only reliable criterion
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What does this picture demonstrate
|
Pheochromocytoma
Periadrenal lymph node replaced by malignant cells arranged in a nest |
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What familial syndromes have been associated with pheochromocytoma
|
MEN type IIA or IIB, von Hippel-Lindau, von Recklinghausen,
Sturge-Weber . |
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What characteristically has retinal angiomas, cerebellar hemangioblastoma, and renal cell cancer
|
von-Hippel Lindau
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What typically has leptomeningeal angiomatosis, seizures, and hemiplegia
|
Sturge-Weber
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What typically has Medullary cancer of the thyroid and pheochromocytoma, adenoma, or hyperplasia of parathyroid
|
MEN types IIA and IIB
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What typically has mucocutaneous neuromas
|
MEN IIB
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What typically has neurofibromas, and "cafe' au lait" spots
|
von Recklinghausen
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What familial syndrome typically has Hyperparathyroidism
|
MEN type IIA
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|
What do these pictures demonstrate
|
A. Normal pituitary: basophils, acidophils and
chromophobes B. GH adenoma: monotonous population of acidophilic cells Tumor cells are uniform, arranged in nests, sheets, or cords resting on a delicate vascular stroma The feature to look for is the presence of only a single cell type (in a normal pituitary, 3 types of cells are seen). |
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Approximately 20% of pituitary adenomas have no clinical or immunohistochemical evidence of hormone production (null-cell adenomas). How might they present clinically?
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As mass lesions compressing the optic chiasm (bitemporal hemianopsia) or the rest of the pituitary causing hypofunction.
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What are prolactinomas?
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The most common type of functional pituitary adenoma
In women of childbearing age, prolactinomas manifest clinically with amenorrhea or galactorrhea. These tumors have been successfully treated with dopamine agonists such as bromocriptine, which inhibit prolactin secretion and produce tumor atrophy. |
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You treated your patient with bromocriptine and the pituitary tumor dramatically reduced in size. You discontinued treatment and the tumor and symptoms have returned. Why?
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The regressive changes induced by bromocriptine persist only as long as the drug is taken on a daily basis.
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What is the clinical presentation of a growth hormone (GH) adenoma
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May produce gigantism in prepubertal children and acromegaly in the adults.
These patients also tend to have visual field defects because GH adenomas tend to be fairly large by the time they are discovered. |
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