1) Target Audience a) Medical Students, nurses, residents (all years)
i) Identify pheochromocytoma and discuss the typical symptoms and
ii) Discuss the management of hypertension related to pheochromocytoma
i) Discuss prognosis of pheochromocytoma with patient
a) Order dissection protocol CT b) Treat hypertension when patient returns from CT symptomatic and with increased
c) Disposition patient to floor or ICU depending on medications used for treatment
Please indicate below the monitoring required (if different from standard EM monitoring) as well as any other equipment/props that will be required. If U/S images or mannequin moulage is required, please describe below
Ultrasound images if required: Mannequin Moulage if required: Scheduling standardized patient (SP) actors can often add a lot to the scenarios if the case benefits from a “difficult” patient or health care provider. This does, however, require planning and scheduling for the same. Please indicate if an SP is required for the scenario and describe briefly below:
CXRs and EKGs are often ordered in these scenarios whether they are relevant to the case or not. Please indicate below the findings on these and find an appropriate ECG and CXR that would fit with the scenario to include in the materials. Supporting Files
1. EKG: Sinus tachycardia with nonspecific changes 2. CXR: Normal 3. Other: Dissection protocol CT showing paraganglioma adjacent to right adrenal
a) Simulation setup: i) ED bay with 2 beds (1) Crash cart (2) Airway cart (3) Monitor (BP, Rhythm strip, SaO2, HR) (4) IVs and IV fluid setup
(1) Phentolamine (2) Labetalol (3) Nipride (4) Esmolol (5) Nitroglycerine (6) Metoprolol
i) Physician Learner(s) ii) Nurse learner(s) iii) SP or Simulator
(1) I was standing at the bus stop and I suddenly developed pain in my chest.
It went to my back between my shoulder blades. I was sweaty and nauseous with it.
(2) I’ve had similar symptoms before including when they told me I had a
heart attack 2 years ago. At that time, they did an angiogram and found I didn’t have any “blockages”
iv) Family members NONE v) Nurse Actor NONE
6) Case Narrative (describes flow of case)
i) Community ED ii) Consultative services available but may not be in-house
(a) I suddenly developed chest pain that went into my back and left
shoulder blade with associated nausea and sweatiness. It lasted 10 minutes. It felt like electric shocks going down my back. People said I was white as a ghost. After the 10 minutes, I had a dull ache in my left chest. Since then, I’ve been having trouble breathing only when I walk.
i) CT for PE or dissection ii) Recognize paraganglioma as being similar to pheochromocytoma iii) Treat hypertension with appropriate medications (phentolamine, labetalol, etc)
i) After CT, patient is more hypertensive and symptomatic (due to iodine
exacerbating pheochromocytoma secretion)
(1) Catecholamine-secreting neuroendocrine tumor arising from adrenal
(2) Hereditary pheochromocytomas seen in multiple endocine neoplasia type
2, von Hippel-Lindau syndrome, neurofibromatosis type 1 and familial paragangliomas
(3) Sporadic forms diagnosed usually at age 40-50
ii) What is the classic presentation of a pheochromocytoma?
(a) Episodic headache (b) Sweating (c) Tachycardia
(2) Half have episodic HTN, remainder have essential HTN (3) 5-15% have normal blood pressure between paroxysms of hypertension (4) HA occurs in 90% of symptomatic patients (5) Generalized sweating in 60-70% of patients (6) Other symptoms:
(a) Palpitations, dyspnea, generalized weakness, panic-attack symptoms
(esp with epinephrine-secreating tumors)
iii) What is the “rule of 10’s” for pheochromocytoma
(a) Higher in familial forms (up to 20%)
(a) 33% for extra-adrenal pheochromocytomas
iv) What is the best diagnostic workup ?
(1) Plasma (sensitivity 99%, specificity 89%) or urinary (Sens 97%, spec
69%) fractionated metanephrines are most sensitive
(2) For tumor localization, CT or MRI with and without contrast but iodine
(3) MIBG (I-metaiodobenzylguanidine) scanning has greater specificity
v) Treatment for pheochromocytoma-associated hypertensive crisis?
(a) Rapid onset and short duration (b) 0.5-5.0 mcg/kg
(a) Short-acting nonselective alpha blocker (b) Initial test dose of 1 mg followed by repeat 5 mg boluses or continuous
(c) Maximal response 2-3 minutes after bolus (d) Duration of action 10-15 minutes
(a) Calcium channel blocker (dihydropyridine) (b) Infusion of 5 mg/hour titrated to BP
(i) Increase infusion by 2.5 mg/hr every 15 minutes to maximum of
(i) Irreversible, long-acting, nonspecific alpha-adrenergic blocker
(b) Doxazosin, prazosin, terazosin for long-term management but
blockade incomplete so suboptimal acutely
(a) To be started ONLY AFTER adequate alpha blockade achieved (due
to potential for unopposed alpha stimulation if peripheral vasodilatory beta receptors are blocked
vi) What is the definitive treatment and outcome?
(1) 10% are malignant and can’t be differentiated from benign ones.
(a) Can cause local r distant metastases which may not be evident for up
(2) Surgical removal improves symptoms and survival (3) 5 year survival <50%
i) Lenders JWM, et al. Lancet 2005: 366: 665-75
Appendix A—Patient Summary Information Chest pain Arrived by (car/EMS) Vital Signs: PMH: Prior MI 2005. Subsequent cath clean. Medications: ASA, Toprol XL (50 qD), Avandamet (rosiglitazone/metformin) (2/500 qD), Prilosec, Crestor Allergies: Codeine, ACE inhibitors Data Summary: (if normal, please indicate)
CBC: Hb 14.1 Hct 41.7 WBC 13.1 Plts 342 Electrolytes: 136/4.2 101/26 13/0.7 Glucose: 180 Troponin: 0.06 CK-MB 3.8 (<6.2 nG/mL) Other relevant labs: EKG: Normal CXR: Normal Urinalysis: Normal CT Dissection protocol: CT of the chest and abdomen with IV contrast material
is negative for pulmonary embolism or aortic dissection. There is an approximately 4.7 x 6.6 cm mass which appears to be inferior to the right lobe of the liver, superior to the upper pole of the right kidney, and abuts the right adrenal gland. The exact organ of origin is difficult to determine from this study but the appearance is most suggestive of a right adrenal mass. The high-density or enhancement within it raises the possibility of pheochromocytoma or paraganglioma. Less likely would be an exophytic hemorrhagic cyst. Correlation with urinary metanephrines is recommended. There is a cyst in the lower pole of the left kidney. Prrevious cholecystectomy.
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