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FEOCROMOCITOMA
Michele Zini
Servizio di Endocrinologia
Arcispedale S. Maria Nuova, Reggio Emilia
michele.zini@asmn.re.it

Michele Zini 2009
EPIDEMIOLOGY

What is the frequency of incidental adrenal
masses in the general population?
 Clinical series:
 Male to female ratio:

3% in middle age
10% in the elderly
1.3 : 1.5

 Autopsy series:

<1% below 30 years of age
3% at 50 years
>7% around 70 years

 Side

right
left
bilateral

53% (50-60)
37% (30-40)
10% (7-15)
Michele Zini 2009
Terzolo M et al., Best Pract Res Clin Endocrinol Metab. 2009 Apr;23(2):233-43
Michele Zini 2009
ADENOMA 52%

CARCINOMA 12%
CYST 5%
MYELOLIPOMA 8%

OTHER 6%

METASTASIS 2%

PHEO 11%

GANGLIONEUROMA 4%
Incidentaloma surrenalico
Casistica di Orbassano, 1991-2005
ADENOMA 78%

181 pts

%
1%
6%
6%
s1
s
a
a
si
si
ta
ta
om
om
yt
yt
as
as
et
oc
et
oc
M
m
M
m
ro
ro
ch
ch
eo
eo
Ph
Ph
%
%
r2
r2
he
he
Ot
Ot

Ca
rci
no
ma
Cy
5%
st
M
3%
ye
lo
lip
om
a
5%
EPIDEMIOLOGY

What are the causes of incidental adrenal
masses in the general population?
Average percentage in the literature

• Adenoma

(33-96)

not-secreting
cortisol-secreting
aldosterone-secreting
estrogen/androgen-secreting

•
•
•
•
•

63%
75%
9.5%
2.5%
very rarely

(52-97)
(1-29)
(1.6-2.3)

Pheochromocytoma
Carcinoma
Myelolipoma
Cyst
Ganglioneuroma

7%
6.5%
8%
5%
4%

(1.5-23)
(1.2-11)
(7-15)
(4-22)
(0-6)
EPIDEMIOLOGY

Kopetschke R et al., European Journal of Endocrinology 161 355–361, 2009
Michele Zini 2009
INCIDENTALE RISCONTRO
≠
ASINTOMATICO

Michele Zini 2009
INCIDENTALOMA: CHE FARE ?

AACE/AAES Adrenal Incidentaloma Guidelines, Endocr Pract. 2009;15(Suppl 1)
Michele Zini 2009
ENDOCRINE ASSESSMENT

Why pheochromocytoma has to be
recognized?
 Life-threatening condition, particularly if undiagnosed.
 Premature mortality.
 High

morbidity (arrhythmias,
hypertension, hyperglycemia).

 Hypertensive

episodes

and

sustained
arrhythmias

or
can

paroxysmal
result

in

emergencies.
 Even clinically silent pheochromocytoma can be lethal.
 An increasing number of patients with pheochromocytoma

have low-grade hypertension or are normotensive.
Michele Zini 2009

Terzolo M et al., Best Pract Res Clin Endocrinol Metab. 2009 Apr;23(2):233-43
AACE/AAES Adrenal Incidentaloma Guidelines, Endocr Pract. 2009;15(Suppl 1)
Michele Zini 2009
Lee JA et al., Arch Surg. 2007;142(9):870-874
Michele Zini 2009
Lee JA et al., Arch Surg. 2007;142(9):870-874
(Calcolato 1 FN e 1 FP)

Valori borderline considerati come
pos.
neg.
esclusi
Sensibilità

93%

79%

92%

Specificità

75%

97%

95%

Potere predittivo positivo

67%

92%

92%

Potere predittivo negativo

95%

90%

95%

Rapporto di probabilità
del test positivo

3.7

23

20

Rapporto di probabilità
del test negativo

0.1

0.2

0.1
Michele Zini 2009
Sawka AM et al. A systematic review of the literature examining the diagnostic efficacy
of measurement of fractionated plasma free metanephrines in the biochemical
diagnosis of pheochromocytoma. BMC Endocrine Disorders.2004
Michele Zini 2009
Sawka AM et al. A systematic review of the literature examining the diagnostic efficacy
of measurement of fractionated plasma free metanephrines in the biochemical
diagnosis of pheochromocytoma. BMC Endocrine Disorders.2004
Michele Zini 2009
Authors' conclusions
A negative fractionated plasma metanephrine
measurement is highly effective in ruling out disease.
However, a positive test result only moderately
increases suspicion of disease

Sawka AM et al. A systematic review of the literature examining the diagnostic efficacy
of measurement of fractionated plasma free metanephrines in the biochemical
diagnosis of pheochromocytoma. BMC Endocrine Disorders.2004
Michele Zini 2009
INCIDENTALOMA
Il laboratorio
•
•
•
•

metanefrine urinarie
catecolamine plasmatiche
catecolamine urinarie
(metanefrine plasmatiche)

• PRA
• aldosterone
• elettroliti

•
•
•
•

cortisolemia
cortisoluria 24-ore
ACTH
test di soppressione
(desametazone 1 mg)

• DHEAS
• estradiolo
• testosterone

Michele Zini 2009
INCIDENTALOMA: PRIORITA’
• Escludere neoplasie maligne
(primitive o secondarie)
• Escludere feocromocitoma
• Valutazione funzionale corticale

Michele Zini 2009

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Incidentaloma surrenalico e feocromocitoma

  • 1. FEOCROMOCITOMA Michele Zini Servizio di Endocrinologia Arcispedale S. Maria Nuova, Reggio Emilia michele.zini@asmn.re.it Michele Zini 2009
  • 2. EPIDEMIOLOGY What is the frequency of incidental adrenal masses in the general population?  Clinical series:  Male to female ratio: 3% in middle age 10% in the elderly 1.3 : 1.5  Autopsy series: <1% below 30 years of age 3% at 50 years >7% around 70 years  Side right left bilateral 53% (50-60) 37% (30-40) 10% (7-15) Michele Zini 2009
  • 3. Terzolo M et al., Best Pract Res Clin Endocrinol Metab. 2009 Apr;23(2):233-43 Michele Zini 2009
  • 4. ADENOMA 52% CARCINOMA 12% CYST 5% MYELOLIPOMA 8% OTHER 6% METASTASIS 2% PHEO 11% GANGLIONEUROMA 4%
  • 5. Incidentaloma surrenalico Casistica di Orbassano, 1991-2005 ADENOMA 78% 181 pts % 1% 6% 6% s1 s a a si si ta ta om om yt yt as as et oc et oc M m M m ro ro ch ch eo eo Ph Ph % % r2 r2 he he Ot Ot Ca rci no ma Cy 5% st M 3% ye lo lip om a 5%
  • 6. EPIDEMIOLOGY What are the causes of incidental adrenal masses in the general population? Average percentage in the literature • Adenoma (33-96) not-secreting cortisol-secreting aldosterone-secreting estrogen/androgen-secreting • • • • • 63% 75% 9.5% 2.5% very rarely (52-97) (1-29) (1.6-2.3) Pheochromocytoma Carcinoma Myelolipoma Cyst Ganglioneuroma 7% 6.5% 8% 5% 4% (1.5-23) (1.2-11) (7-15) (4-22) (0-6)
  • 7. EPIDEMIOLOGY Kopetschke R et al., European Journal of Endocrinology 161 355–361, 2009 Michele Zini 2009
  • 9. INCIDENTALOMA: CHE FARE ? AACE/AAES Adrenal Incidentaloma Guidelines, Endocr Pract. 2009;15(Suppl 1) Michele Zini 2009
  • 10. ENDOCRINE ASSESSMENT Why pheochromocytoma has to be recognized?  Life-threatening condition, particularly if undiagnosed.  Premature mortality.  High morbidity (arrhythmias, hypertension, hyperglycemia).  Hypertensive episodes and sustained arrhythmias or can paroxysmal result in emergencies.  Even clinically silent pheochromocytoma can be lethal.  An increasing number of patients with pheochromocytoma have low-grade hypertension or are normotensive.
  • 11. Michele Zini 2009 Terzolo M et al., Best Pract Res Clin Endocrinol Metab. 2009 Apr;23(2):233-43
  • 12. AACE/AAES Adrenal Incidentaloma Guidelines, Endocr Pract. 2009;15(Suppl 1) Michele Zini 2009
  • 13. Lee JA et al., Arch Surg. 2007;142(9):870-874 Michele Zini 2009
  • 14. Lee JA et al., Arch Surg. 2007;142(9):870-874 (Calcolato 1 FN e 1 FP) Valori borderline considerati come pos. neg. esclusi Sensibilità 93% 79% 92% Specificità 75% 97% 95% Potere predittivo positivo 67% 92% 92% Potere predittivo negativo 95% 90% 95% Rapporto di probabilità del test positivo 3.7 23 20 Rapporto di probabilità del test negativo 0.1 0.2 0.1 Michele Zini 2009
  • 15. Sawka AM et al. A systematic review of the literature examining the diagnostic efficacy of measurement of fractionated plasma free metanephrines in the biochemical diagnosis of pheochromocytoma. BMC Endocrine Disorders.2004 Michele Zini 2009
  • 16. Sawka AM et al. A systematic review of the literature examining the diagnostic efficacy of measurement of fractionated plasma free metanephrines in the biochemical diagnosis of pheochromocytoma. BMC Endocrine Disorders.2004 Michele Zini 2009
  • 17. Authors' conclusions A negative fractionated plasma metanephrine measurement is highly effective in ruling out disease. However, a positive test result only moderately increases suspicion of disease Sawka AM et al. A systematic review of the literature examining the diagnostic efficacy of measurement of fractionated plasma free metanephrines in the biochemical diagnosis of pheochromocytoma. BMC Endocrine Disorders.2004 Michele Zini 2009
  • 18. INCIDENTALOMA Il laboratorio • • • • metanefrine urinarie catecolamine plasmatiche catecolamine urinarie (metanefrine plasmatiche) • PRA • aldosterone • elettroliti • • • • cortisolemia cortisoluria 24-ore ACTH test di soppressione (desametazone 1 mg) • DHEAS • estradiolo • testosterone Michele Zini 2009
  • 19. INCIDENTALOMA: PRIORITA’ • Escludere neoplasie maligne (primitive o secondarie) • Escludere feocromocitoma • Valutazione funzionale corticale Michele Zini 2009