PHEOCHROMOCYTOMA

DR. BILAL AHMAD

PGT SU-1
 PHEOCHROMOCYTOMA
 0.01-0.1% of population with hypertension
 M=F
 3rd to 5th decades of life
 Rare, investigate only if clinically suspicion:
 Signs or Symptoms
 Severe HTN, HTN crisis
 Refractory HTN (> 3 drugs)
 HTN present at age < 20 or > 50
 ADRENAL LESION FOUND ON IMAGING
( INCIDENTALOMA)
 Pheo: Signs & Symptoms
 The five P’s:
 Pressure (HTN) 90%
 Pain (Headache) 80%
 Perspiration 71%
 Palpitation 64%
 Pallor 42%

 The Classical Triad:

 Pain (Headache), Perspiration, Palpitations
 PHEO: PAROXYSMS
‘SPELLS’
 10-60 min duration
 Frequency: daily to monthly
 Spontaneous
 Precipitated:
 Drugs (opioids, unopposed -blockade,
anesthesia induction, histamine, ACTH
glucagon, metoclopramide)
 Strenuous exercise, movement that increases

intra-abdominal pressure (lifting, straining)

PHEO: SIGNS & SYMPTOMS
 Nausea/Vomiting
 Abdominal pain
 Constipation
 Chest-pains
 Cardiac dysrhythmia & conduction defects
 Orthostatic Hypotension
PHEO: SIGNS (METABOLIC)
 Hypercalcemia
 PTHrP secretion by pheochromocytoma
 Mild glucose intolerance
 Lipolysis
 Weight-loss
 Ketosis > VLDL synthesis (TG)
 PHEO: ‘RULE OF 10’
 10% extra-adrenal (closer to 15%)
 10% occur in children
 10% familial (closer to 20%)
 10% bilateral or multiple (more if familial)
 10% recur (more if extra-adrenal)
 10% malignant
 10% discovered incidentally
 Familial Pheo
 MEN 2a
 50% Pheo (usually bilateral), MTC, HPT

 MEN 2b
 50% Pheo (usually bilatl), MTC, mucosal neuroma,

marfanoid habitus
 Von Hippel-Landau
 50% Pheo (usually bilat), retinoblastoma, cerebellar

hemangioma, nephroma, renal/pancreas cysts

 NF1 (Von Recklinghausen's)
 2% Pheo (50% if NF-1 and HTN)

 Café-au-lait spots, neurofibroma, optic glioma

 Familial paraganglioma
 Familial pheo & islet cell tumor
 24H URINE COLLECTION
 24h urine collection:
 Creatinine, catecholamines, metanephrines,
vanillymandelic acid (VMA).

 Positive results (> 2-3 fold elevation):

 24h Ucatechols > 2-fold elevation
• ULN for total catechols 591-890 nmol/d
 24h Utotal metanephrines > 1.2 ug/d (6.5 umol/d)
 24h U
VMA > 3-fold elevation
 PLASMA
CATECHOLAMINES
 Drawn with patient fasting, supine, with an
indwelling catheter in place > 30 min
 Plasma total catechols > 11.8 nM (2000 pg/mL)
 False positives: same as for 24h urine testing, also
with diuretics, smoking
 CRF & ESRD:
 Oliguric to Anuric  24h Urines inaccurate
 Plasma epinephrine best test for pheo in ESRD
 Plasma norepi and metanephrines falsely elevated in
ESRD
 PLASMA METANEPHRINES
 Not postural dependent: can draw normally
 Secreted continuously by pheo
 False Positive: acetaminophen
 Assay not readily available
 Biochemical Tests: Summary
SEN SPEC
Ucatechols 83% 88%
Utotal metanephrines 76% 94%
Ucatechols+metaneph 90% 98%
UVMA 63% 94%
Plasma catecholamines 85% 80%
Plasma metanephrines 99% 89%
 LOCALIZATION: IMAGING
 CT abdomen
 Adrenal pheo SEN 93-100%
 Extra-adrenal pheo SEN 90%
 MRI
 > SEN than CT for extra-adrenal pheo
 MIBG (meta-iodo-benzylguanidine) Scan
 SEN 77-90% SPEC 95-100%
 MIBG Scan
 I or 131I labelled metaiodobenzylguanidine
123

 MIBG catecholamine precurosr taken up by the

tumor
 Inject MIBG, scan at 24h, 48h, 72h
 Localization: Nuclear medicine

 Indium-pentreotide
111

 Some pheo have somatostatin receptors

 PET
 18 F-fluorodeoxyglucose (FDG)
 6-[18F]-fluorodopamine
 PHEO MANAGEMENT
 Reported mortality for excision of
pheochromoyctoma
 HTN crisis, arrhythmia, MI, stroke
 Hypotensive shock
 Preoperative preperation, -blockade
 Intraoperative monitoring: arterial line, EKG
monitor, CVP line
 Experienced & Coordinated team:
 Endocrinologist, Anesthesiologist and Surgeon
 PREOP WORK UP
 CBC, Electrolytes, creatinine, INR/PTT
 CXR
 ECG
 Echo (r/o dilated CMY secondary to
catechols)
 PREOP PREPERATION
REGIMENS
 Combined  +  blockade
 Phenoxybenzamine

 Selective  -blocker (ex. Prazosin)

1
 Propanolol

 Metyrosine
 Calcium Channel Blocker (CCB)
 Nicardipine
 PREOP:  +  BLOCKADE
 Start at least 10-14d preop
 Allow sufficient time for ECFv re-expansion
 Phenoxybenzamine
 Special pharmacy access only
 Drug of choice
 Covalently binds -receptors (1 > 2)
 Start 10 mg po bid  increase q2d by 10-20 mg/d
 Increase until BP cntrl and no more paroxysms
 Maintenance 40-80 mg/d (some need > 200 mg/d)
 Salt load: NaCl 600 mg od-tid as tolerated
 PREOP:  +  BLOCKADE
 Phenoxybenzamine (cont’d)
 Side-effect: orthostasis with dosage required to normalized
seated BP, reflex tachycardia
 Drawback: periop hypotension/shock unlikely to respond to
pressor agent.
 Selective 1-blockers
 Prazosin, Terazosin, Doxazosin
 Some experience with Prazosin for Pheo preop prep
 Not routinely used as incomplete -blockade
 Less orthostasis & reflex tachycardia then phenoxybenzamine
 Used more for long-term Rx (inoperable or malignant pheo)
 PREOP:  +  BLOCKADE
 -blockade
 Used to control reflex tachycardia and
prophylaxis against arrhythmia during surgery
 Start only after effective -blockade (may ppt

HTN)
 If suspect CHF/dilated CMY  start low dose

 Propanolol most studied in pheo prep

• Start 10 mg po bid  increase to cntrl HR

 PREOP:  +  BLOCKADE
 If BP still not cntrl despite  +  blockade
 Add Prazosin to Phenoxybenzamine

 Add CCB, ACE-I

 Avoid diuretics as already ECFv

contracted
 Metyrosine
 PREOP:  +  BLOCKADE
 Meds given on AM of surgery
 Periop HTN:
 IV phentolamine
– Short acting non-selective -blocker
– Test dose 1 mg, then 2-5 mg IV q1-2h PRN or as
continuous infusion (100 mg in 500cc D5W, titrate to BP)
 IV Nitroprusside (NTP)
 Periop arrhythmia: IV esmolol
 Periop Hypothension: IV crystalloid +/- colloid
 LOCALIZED PHEO
MANAGEMENT
 Surgical resection, i.e., adrenalectomy, is the definitive
treatment for patients with localized pheochromocytoma.
A minimally invasive adrenalectomy is done provided:
 Preoperative imaging reveals an adrenal
pheochromocytoma that is approximately 6 cm or smaller
in diameter.
 No radiographic evidence of invasion into adjacent
structures or evidence of regional or metastatic disease
(i.e., presumably a benign tumor).
 Normal contralateral adrenal gland.
 REGIONAL PHEO
MANAGEMENT
 Surgical resection is the definitive treatment for
pheochromocytoma or extra-adrenal paraganglioma that
is regionally advanced (e.g., from direct tumor extension
into adjacent organs or because of regional lymph node
involvement).
 Surgical management of these patients may require en
bloc resection of all or part of adjacent organs (e.g.,
kidney, liver, inferior vena cava) along with extended
lymph node dissection.
 Postop
 Most cases can stop all BP meds postop
 Postop hypotension: IV crystalloid
 HTN free: 5 years 74% 10 years 45%
 24h urine collection 2 wk postop
 Surveillance:
 24h urine collections q1y for at least 10y
 Lifelong f/up
 Pheo: Unresectable, Malignant
 -blockade
 Selective 1-blockers (Prazosin, Terazosin, Doxazosin)
1st line as less side-effects
 Phenoxybenzamine: more complete -blockade

 -blocker ,CCB, ACE-I, etc.

 Radiation Therapy:
 Hi dose 131I-MIBG or 111indium-octreotide depending
on MIBG scan or octreoscan pick-up
 Sensitize tumor with Carboplatin + 5-FU +

Cyclophosphamide
THANK YOU