Department of Internal Diseases, Hypertension and Angiology The Medical University of Warsaw PHAEOCHROMOCYTOMA- Do classical symptoms help in diagnosis?

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