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Department of Internal Diseases, Hypertension and AngiologyThe Medical University of Warsaw
PHAEOCHROMOCYTOMA-Do classical symptoms help in
diagnosis?
Dorota KwapiszDorota Kwapisz, Marta Trafalska, Marta Trafalska
PHAEOCHROMOCYTOMA Phaeochromocytomas are catecholamine-
producing neuroendocrine tumours arising from chromaffin cells of the adrenal medulla or extra-adrenal paraganglia
It`s rare tumour with a highly variable clinical presentation
Pts with paroxysmal signs or symptoms suggestive of a pheo
The classical triad: headache
sweating
palpitations
…hypertension asymptomatic pts with an adrenal
incidentaloma
PHAEOCHROMOCYTOMA
PHAEOCHROMOCYTOMAPHAEOCHROMOCYTOMA
Although the presence of the classical Although the presence of the classical
triad of symptoms of pheo may lead to triad of symptoms of pheo may lead to
correct diagnosis, yet frequently diagnosis correct diagnosis, yet frequently diagnosis
is establised during evaluation of is establised during evaluation of
incidentally discovered mass incidentally discovered mass
(incidentaloma)(incidentaloma)
THE AIM
The aim of the study was:• To asses clinical presentation of pheo in recent
series of cases,
• To compare the pts. with pheo in whom the diagnostics was triggered by symptoms (S –”Symptomatic”) with those in whom diagnostics began with incidental finding of adrenal mass (I – „Incidentaloma”)
DESIGN AND METHODS
n= 43 with pheon= 43 with pheo
mean age 47.4 mean age 47.4 ± ± 13.1 yrs13.1 yrs
M/F 22/21M/F 22/21
n= 29 „symptomatic”, n= 14 „incidentaloma”n= 29 „symptomatic”, n= 14 „incidentaloma”
n= 39 – pheo was located in the adrenals n= 39 – pheo was located in the adrenals
(1 bilateral – 1S)(1 bilateral – 1S)
n= 4 – extraadrenal (1S/3I)n= 4 – extraadrenal (1S/3I)
DESIGN AND METHODS
„ „S” S” SYMPTOMATIC SYMPTOMATIC
„ „I” I” INCIDENTALOMINCIDENTALOMAA
n (pts)n (pts) 2929 1414
Mean Mean ageage
48.45 ± 12.58 48.45 ± 12.58 yrsyrs
45.21 ± 14.30 45.21 ± 14.30 yrsyrs
M/FM/F 15/1415/14 7/77/7
MalignaMalignantnt
1 1 11
MEN IIBMEN IIB 11 11
METHODSMETHODS
Incidence of the most typical symptoms of Incidence of the most typical symptoms of pheopheo
Duration of hypertensionDuration of hypertension
Urine excretion of metanephrines and Urine excretion of metanephrines and catecholaminecatecholamine
METHODS
Automatic Blood Pressure Monitoring(ABP) SpaceLabs 90207 Mean, maximum, SD of 24SBP/24DBP
Echocardiography Hewlett-Packard 770201 LVMI
RESULTSRESULTSSymptomSymptom S+ IS+ I SS II pp
SWEATINGSWEATING 77 %77 % 90 %90 % 50 %50 % <0,022<0,022
HeadacheHeadache 7373 7878 6262
ArrhythmiasArrhythmias 7070 8383 3636
PalpitationsPalpitations 6565 8686 2121
AnxietyAnxiety 5151 5959 3636
PallorPallor 4444 5959 1414
TriadTriad 4040 5252 1414
HypertensionHypertension 2424 2222 2727
Weight lossWeight loss 1919 2121 1414
SYMPTOMSSYMPTOMS
SS
1.1. Sweating - 90%Sweating - 90%
2.2. Palpitations – 86%Palpitations – 86%
3.3. Headaches – 78%Headaches – 78%
The triad – 52%The triad – 52%
II
1.1. Headaches – 62%Headaches – 62%
2.2. Sweating – 50%Sweating – 50%
3.3. Anxiety – 36%Anxiety – 36%
The triad – 14%The triad – 14%
RESULTS – the triadRESULTS – the triad
90%50%
86%21%
78%62%
52%14%
0% 20% 40% 60% 80% 100%
sweating
palpitations
headache
triad
IncidentalomaSymptomatic
ABPABP S + IS + I SS II
24SBP/24DBP24SBP/24DBP 132/80 ± 132/80 ± 16/1016/10
133/79 133/79 ± 17/10± 17/10 131/83 131/83 ± 14/10± 14/10
24HR24HR 79 ± 1379 ± 13 7979 ± 14± 14 7878 ± 12± 12
24SBP/24DBP- 24SBP/24DBP- day/maxday/max
173/107173/107 177/107177/107 166/108166/108
24SBP/24DBP - 24SBP/24DBP - night/maxnight/max
155/ 93155/ 93 156/ 91156/ 91 154/ 96154/ 96
Duration of 5,43 ± 6,26 5,51 5,28 hypertension (yrs)LVMI (g/m2) (g/m2)
URINE TESTS - catecholamines before operation
-1000
100200300400500600700800900
10001100
Patients
Cat
echo
lam
ines
[ug/
24h]
NA Symptomatic A Symptomatic NA Incidentaloma A Incidentaloma
MetanephrinesMetanephrines- mean:- mean:
S-3790,50S-3790,50
I- 3365,30I- 3365,30
p< 0,042p< 0,042
S : S :
270,8 –23666270,8 –23666
I :I :
1375 – 10729,21375 – 10729,2
Correlation between metanephrines and the triad
-1
0
1
1
2
0 5000 10000 15000 20000 25000
Metanephrines [ug/24h]
THE
TRIA
D [0-
no, 1
-yes]
Symptomatic Incidentaloma
RESULTSRESULTS
S+I S IS+I S I
Uricacid (mg/dl) 8,03 Uricacid (mg/dl) 8,03 ±± 15,03 9,20 5,38 15,03 9,20 5,38
p< 0,00(0)p< 0,00(0)
Urea (mg/dl) 40,78 Urea (mg/dl) 40,78 ±± 25,08 41,99 37,90 25,08 41,99 37,90
p< 0,038p< 0,038
Creatinine (mg/dl) 4,49 Creatinine (mg/dl) 4,49 ±± 22,19 22,19 6,17 0,866,17 0,86
p< 0,00(0)p< 0,00(0)
CONCLUSIONS
The incidence of classical symptoms may be
lower than previously reported
Pheochromocytoma may mimic essential hypertension
Lack of sweating may be misleading and lead to wrong diagnosis