Updated: 8/11/2018

Pheochromocytoma

Topic
Review Topic
0
0
Questions
5
0
0
Evidence
4
0
0
Videos
1
Snapshot
  • A 40-year-old gentleman presents with episodic headaches and palpitations. Attributing it to his tendency to worry excessively, he put it off for several months until he began to sweat episodically too. In the clinic, his blood pressure is found to be elevated at 160/120 mmHg.  Plasma metanephrines are elevated and an abdominal CT scan reveals an adrenal mass.
Introduction
  • Nonmalignant tumor of adrenal medulla
    • most common adrenal tumor in adults (vs neuroblastoma, most common in children)
    • secretes catecholamines causing episodic hypertension
  • Pathogenesis
    • from chromaffin cells of neural crest origin
  • Epidemiology
    • 40-50 years old
  • Rule of 10’s
    • 10% malignant
    • 10% bilateral
    • 10% kids
    • 10% extra-adrenal
      • bladder, organ of Zuckerkandl (bifurcation of aorta)
    • 10% calcify
  • Associated conditions
    • von Hippel-Lindau disease
    • MEN 2A
    • MEN 2B
    • neurofibromatosis type 1
Presentation
  • Symptoms are episodic  
    • 5 P’s
      • Pressure (↑ BP)
        Pain (headache)
      • Perspiration
      • Palpitations (tachycardia)
      • Pallor
    • mediated by tumor secretion of epinephrine, norepinephrine, and dopamine
  • Physical exam
    • ↑ BP (diastolic)
Evaluation
  • Serology
    • ↑ free metanephrine level
  • 24-hour urine collection for diagnostic confirmation
    • ↑ vanillyl mandelic acid (breakdown of norepinephrine and epinephrine)
    • ↑ metanephrines (more sensitive than VMA)
  • Histology
    • chromaffin cells with enlarged dysmorphic nuclei
Differential Diagnosis
  • Hyperthyroidism
  • Carcinoid tumors
  • Panic disorder
  • Therapy resistant hypertension from obstructive sleep apnea
Treatment
  • In this sequence of events
    • α-antagonist phenoxybenzamine to prevent unopposed α-action if β receptors are blocked
    • β-blocker
    • tumor resection
Prognosis, Prevention, and Complications
  • Prognosis
    • very good with resection
  • Complications
    • metastases
      • bones, lungs, and liver
 

Please rate topic.

Average 4.4 of 5 Ratings

Thank you for rating! Please vote below and help us build the most advanced adaptive learning platform in medicine

The complexity of this topic is appropriate for?
How important is this topic for board examinations?
How important is this topic for clinical practice?
Questions (5)
Lab Values
Blood, Plasma, Serum Reference Range
ALT 8-20 U/L
Amylase, serum 25-125 U/L
AST 8-20 U/L
Bilirubin, serum (adult) Total // Direct 0.1-1.0 mg/dL // 0.0-0.3 mg/dL
Calcium, serum (Ca2+) 8.4-10.2 mg/dL
Cholesterol, serum Rec: < 200 mg/dL
Cortisol, serum 0800 h: 5-23 μg/dL //1600 h:
3-15 μg/dL
2000 h: ≤ 50% of 0800 h
Creatine kinase, serum Male: 25-90 U/L
Female: 10-70 U/L
Creatinine, serum 0.6-1.2 mg/dL
Electrolytes, serum  
Sodium (Na+) 136-145 mEq/L
Chloride (Cl-) 95-105 mEq/L
Potassium (K+) 3.5-5.0 mEq/L
Bicarbonate (HCO3-) 22-28 mEq/L
Magnesium (Mg2+) 1.5-2.0 mEq/L
Estriol, total, serum (in pregnancy)  
24-28 wks // 32-36 wks 30-170 ng/mL // 60-280 ng/mL
28-32 wk // 36-40 wks 40-220 ng/mL // 80-350 ng/mL
Ferritin, serum Male: 15-200 ng/mL
Female: 12-150 ng/mL
Follicle-stimulating hormone, serum/plasma Male: 4-25 mIU/mL
Female: premenopause: 4-30 mIU/mL
midcycle peak: 10-90 mIU/mL
postmenopause: 40-250
pH 7.35-7.45
PCO2 33-45 mmHg
PO2 75-105 mmHg
Glucose, serum Fasting: 70-110 mg/dL
2-h postprandial:<120 mg/dL
Growth hormone - arginine stimulation Fasting: <5 ng/mL
Provocative stimuli: > 7ng/mL
Immunoglobulins, serum  
IgA 76-390 mg/dL
IgE 0-380 IU/mL
IgG 650-1500 mg/dL
IgM 40-345 mg/dL
Iron 50-170 μg/dL
Lactate dehydrogenase, serum 45-90 U/L
Luteinizing hormone, serum/plasma Male: 6-23 mIU/mL
Female: follicular phase: 5-30 mIU/mL
midcycle: 75-150 mIU/mL
postmenopause 30-200 mIU/mL
Osmolality, serum 275-295 mOsmol/kd H2O
Parathyroid hormone, serume, N-terminal 230-630 pg/mL
Phosphatase (alkaline), serum (p-NPP at 30° C) 20-70 U/L
Phosphorus (inorganic), serum 3.0-4.5 mg/dL
Prolactin, serum (hPRL) < 20 ng/mL
Proteins, serum  
Total (recumbent) 6.0-7.8 g/dL
Albumin 3.5-5.5 g/dL
Globulin 2.3-3.5 g/dL
Thyroid-stimulating hormone, serum or plasma .5-5.0 μU/mL
Thyroidal iodine (123I) uptake 8%-30% of administered dose/24h
Thyroxine (T4), serum 5-12 μg/dL
Triglycerides, serum 35-160 mg/dL
Triiodothyronine (T3), serum (RIA) 115-190 ng/dL
Triiodothyronine (T3) resin uptake 25%-35%
Urea nitrogen, serum 7-18 mg/dL
Uric acid, serum 3.0-8.2 mg/dL
Hematologic Reference Range
Bleeding time 2-7 minutes
Erythrocyte count Male: 4.3-5.9 million/mm3
Female: 3.5-5.5 million mm3
Erythrocyte sedimentation rate (Westergren) Male: 0-15 mm/h
Female: 0-20 mm/h
Hematocrit Male: 41%-53%
Female: 36%-46%
Hemoglobin A1c ≤ 6 %
Hemoglobin, blood Male: 13.5-17.5 g/dL
Female: 12.0-16.0 g/dL
Hemoglobin, plasma 1-4 mg/dL
Leukocyte count and differential  
Leukocyte count 4,500-11,000/mm3
Segmented neutrophils 54%-62%
Bands 3%-5%
Eosinophils 1%-3%
Basophils 0%-0.75%
Lymphocytes 25%-33%
Monocytes 3%-7%
Mean corpuscular hemoglobin 25.4-34.6 pg/cell
Mean corpuscular hemoglobin concentration 31%-36% Hb/cell
Mean corpuscular volume 80-100 μm3
Partial thromboplastin time (activated) 25-40 seconds
Platelet count 150,000-400,000/mm3
Prothrombin time 11-15 seconds
Reticulocyte count 0.5%-1.5% of red cells
Thrombin time < 2 seconds deviation from control
Volume  
Plasma Male: 25-43 mL/kg
Female: 28-45 mL/kg
Red cell Male: 20-36 mL/kg
Female: 19-31 mL/kg
Cerebrospinal Fluid Reference Range
Cell count 0-5/mm3
Chloride 118-132 mEq/L
Gamma globulin 3%-12% total proteins
Glucose 40-70 mg/dL
Pressure 70-180 mm H2O
Proteins, total < 40 mg/dL
Sweat Reference Range
Chloride 0-35 mmol/L
Urine  
Calcium 100-300 mg/24 h
Chloride Varies with intake
Creatinine clearance Male: 97-137 mL/min
Female: 88-128 mL/min
Estriol, total (in pregnancy)  
30 wks 6-18 mg/24 h
35 wks 9-28 mg/24 h
40 wks 13-42 mg/24 h
17-Hydroxycorticosteroids Male: 3.0-10.0 mg/24 h
Female: 2.0-8.0 mg/24 h
17-Ketosteroids, total Male: 8-20 mg/24 h
Female: 6-15 mg/24 h
Osmolality 50-1400 mOsmol/kg H2O
Oxalate 8-40 μg/mL
Potassium Varies with diet
Proteins, total < 150 mg/24 h
Sodium Varies with diet
Uric acid Varies with diet
Body Mass Index (BMI) Adult: 19-25 kg/m2
Calculator

Question locked
Sorry, this question is for
PEAK Premium Subscribers only
Upgrade to PEAK

(M1.EC.4713) A 34-year-old woman comes to the emergency department complaining of severe headache and anxiety, diaphoresis, and palpitations for the last 30 minutes. She has had several similar episodes over the past few weeks. She has no significant past medical history and has a 10 pack-year smoking history. She takes no illicit drugs. Her blood pressure on arrival is 181/80 mmHg and her pulse is 134/min. If this patient was given metoprolol, how would her blood pressure respond? Review Topic

QID: 108485
1

Hypotension due to beta-adrenergic receptor blockade

66%

(144/219)

2

Hypotension due to alpha-adrenergic receptor blockade

9%

(19/219)

3

Hypertension due to alpha-adrenergic receptor blockade

2%

(5/219)

4

Hypertension due to alpha-adrenergic receptor stimulation

17%

(38/219)

5

Hypertension due to alpha- and beta-adrenergic receptor blockade

2%

(4/219)

M1

Select Answer to see Preferred Response

PREFERRED RESPONSE 4

(M1.EC.64) A 34-year-old female presents to the emergency room with headache and palpitations. She is sweating profusely and appears tremulous on exam. Vital signs are as follows: HR 120, BP 190/110, RR 18, O2 99% on room air, and Temp 37C. Urinary metanephrines and catechols are positive. Which of the following medical regimens is contraindicated as a first-line therapy in this patient? Review Topic

QID: 104389
1

Phenoxybenzamine

25%

(28/112)

2

Nitroprusside

22%

(25/112)

3

Propranolol

29%

(33/112)

4

Labetalol

7%

(8/112)

5

Lisinopril

12%

(14/112)

M1

Select Answer to see Preferred Response

PREFERRED RESPONSE 3

(M1.EC.110) A 40-year-old female presents with palpitations, headache, and episodic diaphoresis. On physical exam she has a blood pressure of 170/90 mmHg. Urine analysis shows elevated levels of vanillylmandelic acid. Figure A shows the histology of the adrenal gland. Hyperactivity of cells in which of the follow regions is likely responsible for this patient's symptoms? Review Topic

QID: 100411
FIGURES:
1

Region 1

5%

(1/19)

2

Region 2

11%

(2/19)

3

Region 4

26%

(5/19)

4

Region 5

0%

(0/19)

5

Region 6

58%

(11/19)

M1

Select Answer to see Preferred Response

PREFERRED RESPONSE 5

(M1.EC.116) A 49 year-old-male presents with a primary complaint of several recent episodes of severe headache, sudden anxiety, and a "racing heart". The patient originally attributed these symptoms to stress at work; however, these episodes are becoming more frequent and severe. Laboratory evaluation during such an episode reveals elevated plasma free metanephrines. Which of the following additional findings in this patient is most likely? Review Topic

QID: 100417
1

Decreased 24 hour urine vanillylmandelic acid (VMA) levels

0%

(0/29)

2

Episodic hypertension

76%

(22/29)

3

Anhidrosis

3%

(1/29)

4

Diarrhea

10%

(3/29)

5

Hypoglycemia

10%

(3/29)

M1

Select Answer to see Preferred Response

PREFERRED RESPONSE 2
ARTICLES (4)
VIDEOS (1)
Topic COMMENTS (7)
Private Note