Snapshot A 45-year-old woman presents to the emergency room from her primary care physician’s office for high blood pressure unresponsive to therapy. She has a history of neurofibromatosis type 1, though without any neurological deficits. She has multiple café-au-lait spots on her body. She is found to be hypertensive to 154/121 mmHg. Her 24-hour urine metanephrines and VMA come back elevated. Her abdominal CT comes back with an adrenal mass. Introduction Nonmalignant tumor of adrenal medulla most common adrenal tumor in adults vs neuroblastoma, which is most common in children secretes catecholamines causing episodic hypertension Pathogenesis chromaffin cells from neural crest origin Epidemiology 40-50 year old Rule of 10’s 10% malignant 10% bilateral 10% extra-adrenal bladder and organ of Zuckerkandl (bifurcation of aorta) 10% calcify 10% kids 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 therapy-resistant hypertension Physical exam ↑ BP (diastolic) skin hyperpigmentation (café-au-lait spots) may suggest NF1 Evaluation Best initial test ↑ free metanephrine level in plasma Confirmation with 24-hour urine collection ↑ vanillyl mandelic acid (breakdown of norepinephrine and epinephrine) ↑ metanephrines (more sensitive than VMA) Histology chromaffin cells with enlarged dysmorphic nuclei Imaging adrenal mass seen on MRI or CT MIBG scanning nuclear isotope scan to detect extra-adrenal involvement Differential Diagnosis Functional adrenal tumors various different hormonal syndromes can result, such as Cushing syndrome secondary to unregulated cortisol secretion Conn syndrome secondary to unregulated aldosterone secretion male feminization secondary to unregulated estrogen secretion female virilization secondary to unregulated androgen secretion e.g., androstenedione, DHEA, and testosterone levels 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 transformation to malignant tumor (10%) hypertensive crisis
QUESTIONS 1 of 4 1 2 3 4 Previous Next Lab Values Blood Hematologic Cerebrospinal Sweat, Urine, and BMI 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/LFemale: 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/mLFemale: 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/dL2-h postprandial:<120 mg/dL Growth hormone - arginine stimulation Fasting: <5 ng/mLProvocative 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/mm3Female: 3.5-5.5 million mm3 Erythrocyte sedimentation rate (Westergren) Male: 0-15 mm/hFemale: 0-20 mm/h Hematocrit Male: 41%-53%Female: 36%-46% Hemoglobin A1c ≤ 6 % Hemoglobin, blood Male: 13.5-17.5 g/dLFemale: 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/kgFemale: 28-45 mL/kg Red cell Male: 20-36 mL/kgFemale: 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/minFemale: 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 hFemale: 2.0-8.0 mg/24 h 17-Ketosteroids, total Male: 8-20 mg/24 hFemale: 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 ( ) xy AC 7 8 9 ÷ 4 5 6 × 1 2 3 - 0 . = + Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK You have 100% on this question. Just skip this one for now. Take This Question Anyway (M2.ON.45) A 43-year-old male with a history of thyroid cancer status post total thyroidectomy presents to his primary care physician after repeated bouts of headaches. His headaches are preceded by periods of anxiety, palpitations, and sweating. The patient says he is unable to pinpoint any precipitating factors and instead says the events occur without warning. Of note, the patient's father and uncle also have a history of thyroid cancer. On exam his vitals are: T 36.8 HR 87, BP 135/93, RR 14, and O2 Sat 100% on room air. The patient's TSH is within normal limits, and he reports taking his levothyroxine as prescribed. What is the next best step in diagnosing this patient's chief complaint? Review Topic QID: 104370 Type & Select Correct Answer 1 Abdominal CT scan with and without IV contrast 24% (4/17) 2 24-hour urine free cortisol 0% (0/17) 3 High dose dexamethasone suppression test 0% (0/17) 4 Plasma fractionated metanephrines 65% (11/17) 5 Plasma aldosterone/renin ratio 0% (0/17) M2 Select Answer to see Preferred Response SUBMIT RESPONSE 4 You have 100% on this question. Just skip this one for now. Take This Question Anyway (M2.ON.4694) A 39-year-old woman, with a history of thyroidectomy and primary hyperparathyroidism presents for surgical evaluation for a right adrenal mass. Preoperatively, which of the following medications should she receive to prevent a hypertensive emergency intraoperatively? Review Topic QID: 107708 Type & Select Correct Answer 1 Atenolol 50% (2/4) 2 Labetolol 25% (1/4) 3 Lisinopril 0% (0/4) 4 Nifedipine 0% (0/4) 5 Phenoxybenzamine 25% (1/4) M2 Select Answer to see Preferred Response SUBMIT RESPONSE 5