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Authors: Anastassios G. Pittas, M.D., Arthur S. Tischler, M.D.

1. Case 1

A 23-year-old woman with past medical history significant for primary hypothyroidism due to Hashimoto's (autoimmune) thyroiditis underwent uneventful same-day sinus surgery. Soon upon arrival at home, she started complaining of malaise, aches, pains, and some abdominal pain. Three days later, she was taken to the emergency room for worsening symptoms of generalized fatigue, malaise and abdominal pain. In the ER, she was found to be confused and her blood pressure was 60/0. With supportive care, her BP was improved to 100/40. Her temperature was 39 C and pulse was 100. Her exam was notable for nose packs (from the sinus surgery) and diffuse abdominal tenderness.

  1. What is the differential diagnosis?
  2. How would you proceed if adrenal insufficiency were suspected?
    1. Administer glucocorticoids.
    2. Administer glucocorticoids and mineralocorticoids.
    3. Perform cortrosyn (synthetic ACTH) stimulation test including measurement of baseline ACTH level and wait for results to return.
    4. Perform cortrosyn (synthetic ACTH) stimulation test including measurement of baseline ACTH level and administer glucocorticoids and mineralocorticoids while waiting for results to return.

      Cortrosyn (synthetic ACTH) stimulation test showed: 0' 7.1 -> 30' 9.2 -> 60' 12.3.
  3. Based on the results of the ACTH stimulation test, we can say that the patient has:
    1. Adrenal insufficiency
    2. Primary adrenal insufficiency, due to autoimmune adrenalitis given her history of autoimmune thyroid disease
    3. Secondary adrenal insufficiency
    4. Normal adrenal function

      Her serum Na was low at 131. Her potassium was elevated at 5.4. A baseline (time 0') ACTH level was 268 (normal 4-12).
  4. The most likely diagnosis is
    1. Primary adrenal insufficiency
    2. Secondary Adrenal insufficiency

      The patient was given high dose steroids and did well. She was discharged on prednisone and fludocortisone.
  5. Does she need glucocorticoid supplementation when she is well? When she is ill?
  6. PATH - From the following pairs of choices pick those that best apply to the changes that would be seen in the patient’s adrenal glands:
    A. unilateral B. bilateral
    C. sparing of medulla D. equal involvement of cortex and medulla
    E. atrophy F. hyperplasia

  7. PATH - Inflammatory changes seen in the adrenal gland would most likely be described as:
    1. Granulomatous inflammation with caseous necrosis
    2. Little or no evidence of inflammation
    3. Acute inflammation
    4. Infiltration by lymphocytes and plasma cells, with occasional germinal centers
  8. PATH – From the following list, the additional change LEAST likely to be present is:
    1. Replacement of cortical cells by fibrous tissue
    2. Hypertrophied islands of residual cortical cells
    3. Extensive hemorrhagic necrosis
  9. PATH - True or False: Foci of ectopic adrenal tissue in this patient would be likely to show histological changes similar to those in the adrenals
    1. True
    2. False
  10. PATH - True or False: Almost 40% of patients with Hashimoto’s thyroiditis are at risk to develop adrenal insufficiency
    1. True
    2. False

2. Case 2

A 38-year-old woman has had hypertension for five years. Her symptoms include severe frontal headache, muscle weakness, and polyuria. Her blood pressure was consistently high at around 170/100 mmHg. Serum potassium levels have been noted to be low in the past. When she was given a thiazide diuretic for her hypertension, she became very weak and unable to stand. Her potassium fell to 1.5 mEq/L while on a thiazide.

  1. Based on this presentation, how would you proceed in the evaluation?
    1. No additional work-up is needed, switch therapy to a calcium channel blocker and add other antihypertensive agents as needed to control blood pressure
    2. Perform CT of the abdomen and chest
    3. Proceed with further work-up to better delineate the cause of her hypertension

      On physical examination, she was mildly obese with grade 1 hypertensive retinopathy. Her blood pressure was 182/110 mm Hg. There were no other pertinent physical findings.

      Laboratory evaluation revealed: Serum sodium 147 mEq/L, potassium 3.1 mEq/L, bicarbonate 34 mEq/L, and chloride 93 mEq/L, creatinine 0.9 mg/dl
  2. How would you proceed in the evaluation?
    1. Start a beta blocker
    2. Start an ACE inhibitor
    3. Perform biochemical testing for pheochromocytoma
    4. Perform biochemical testing for hyperaldosteronism
    5. Perform imaging for renal artery stenosis

      The patient was placed on a fixed sodium intake of 5 grams per day for 3 days. Plasma aldosterone was 42 ng/dl and the plasma renin activity was <0.2 ng/ml/hr. She excreted more than 30 mEq of potassium in her urine while her aldosterone level in the urine was 22 (normal less than 14 micrograms) over a 24-hour period.
  3. How would you proceed in the evaluation?
    1. Treat with aldosterone inhibitor
    2. Perform imaging of adrenal gland
    3. Refer to surgeon for bilateral adrenalectomy
    4. Refer to an endocrinologist

      A CT scan of the abdomen was performed and revealed normal adrenal anatomy.
  4. How would you proceed next?
    1. Refer to surgeon for bilateral adrenalectomy
    2. Refer to an endocrinologist
    3. Perform functional imaging

      The patient had adrenal venous sampling which demonstrated a 4:1 gradient for aldosterone (corrected for cortisol) in the right adrenal vein compared to the left adrenal vein.
  5. The correct treatment for her condition is:
    1. Right adrenalectomy
    2. Left adrenalectomy
    3. Bilateral adrenalectomy
    4. Medical treatment with aldosterone antagonist.
  6. PATH - The adrenal removed from this patient is LEAST likely to show:
    1. Cortical Adenoma
    2. Cortical Carcinoma
    3. Cortical Hyperplasia
  7. PATH - The opposite adrenal is MOST likely to appear:
    1. Atrophic
    2. Hyperplastic
    3. Normal
  8. PATH - What additional change, if present, might indicate that the patient had been treated with an aldosterone inhibitor for several weeks before surgery:
    1. Intracytoplasmic inclusions in cortical cells
    2. Intranuclear inclusions in cortical cells
    3. Intranuclear inclusions in medullary cells
    4. Atrophy of the adrenal medulla
  9. PATH - True or False: Adrenal cortical tumors that produce aldosterone are readily distinguished histologically from those that produce glucocorticoids
    1. True
    2. False

3. Case 3

A 6 month old male was admitted to the hospital for evaluation of an abdominal mass found on routine exam by his pediatrician. The developmental parameters were within normal limits. A CT scan revealed a partially calcified mass in the region just above the left kidney. At surgery, a 50 gram encapsulated mass was found in this region, and representative sections were prepared for microscopic examination.

  1. PATH - What is the differential diagnosis of renal and peri renal masses in this age group?
  2. PATH - On the basis of the histological examination, would you expect this child to have evidence of endocrine hyperfunction?
  3. PATH - How would you classify this lesion?
  4. PATHTrue or False: This patient would be expected to have a very poor prognosis because the tumor cells are histologically immature
    1. True
    2. False

4. Case 4

A 32-year-old woman has had periodic severe headaches for several months. Episodes are abrupt in onset and last about 30 minutes. She notes that she is very anxious with a sensation of impending doom and being "shaky" when she has the headaches. During the episodes, she also experiences sweating and palpitations. Her physician found nothing abnormal except mild systolic hypertension with a blood pressure of 150/90 mm Hg and mild obesity. Skin exam was normal.

Despite the unremarkable examination, based on the history, pheochromocytoma is suspected.

  1. How common is pheochromocytoma as a cause of hypertension?
    1. 0.1 % of all cases of hypertension
    2. 2 % of all cases of hypertension
    3. 10 % of all cases of hypertension
    4. 25 % of all cases of hypertension
  2. Which of the following is usually NOT a symptom pheochromocytoma:
    1. Pounding headaches
    2. Palpitations
    3. Pallor
    4. Perspiration
    5. Alopecia
  3. The best next diagnostic test is
    1. Imaging of the adrenal glands
    2. Measurement of catecholamine products and/or metabolites in the urine over 24 hours.
    3. Measurement of serum epinephrine in the morning

      Laboratory evaluation revealed: total urinary catecholamine of 280 mcg/24 hours (normal 14-110 mcg/24 hr.).
  4. At this point, how would you treat this patient and why?
    1. Adrenalectomy
    2. Alpha blocker
    3. Beta blocker
    4. Alpha blocker followed by beta blocker
    5. Beta blocker followed by alpha blocker
  5. Obtaining a careful medical history and family history is crucial to rule out
    1. Malignancy
    2. Multiple Endocrine Neoplasia
    3. Factitious hyperadrenalism
  6. Once pheo has been confirmed biochemically, what would you do next?
    1. Exploratory abdominal surgery to look for the tumor
    2. MRI of the abdomen followed by adrenalectomy if an adrenal tumor is found on MRI
    3. MRI of the abdomen, Control of hypertension, adrenalectomy if an adrenal tumor is found on MRI
  7. PATH - What are the differences (clinical and histological) between this lesion and the lesion seen in the previous case?
  8. PATH - What criteria are useful in the distinction of adrenal cortical and medullary tumors?
  9. PATH - What is the natural history of this lesion?
  10. PATH - We learn belatedly that the patient was adopted as an infant and her family history is therefore irrelevant. Are there any pathology findings that would suggest a need for genetic studies to rule out familial disease in the patient or her children?