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Michele Zini 2009
FEOCROMOCITOMA
Michele Zini
Servizio di Endocrinologia
Arcispedale S. Maria Nuova, Reggio Emilia
michele.zini@asmn.re.it
Clinical series:Clinical series: 3% in middle age3% in middle age10% in the elderly 10% in the elderly
Male to female ratio:Male to female ratio: 1.3 : 1.51.3 : 1.5
Autopsy series:Autopsy series: <1% below 30 years of age<1% below 30 years of age 3% at 50 years3% at 50 years>7% around 70 years>7% around 70 years
SideSide right right 53% (50-60)53% (50-60)left left 37% (30-40)37% (30-40)bilateral bilateral 10% (7-15)10% (7-15)
EPIDEMIOLOGYEPIDEMIOLOGY
What is the frequency of incidental adrenal What is the frequency of incidental adrenal masses in the general population?masses in the general population?
Michele Zini 2009
Terzolo M et al., Best Pract Res Clin Endocrinol Metab. 2009 Apr;23(2):233-43
Michele Zini 2009
ADENOMA 52%ADENOMA 52% CARCINOMA 12%CARCINOMA 12%
OTHER 6%OTHER 6%PHEO 11%PHEO 11%
METASTASIS 2%METASTASIS 2%
CYST 5%CYST 5%
MYELOLIPOMA 8%MYELOLIPOMA 8%
GANGLIONEUROMA 4%GANGLIONEUROMA 4%
ADENOMA 78%ADENOMA 78%
Carcinoma 5%
Carcinoma 5%O
ther 2%
Oth
er 2%Pheochrom
ocytoma 6%
Pheochromocytom
a 6%
Metastasis 1%
Metastasis 1%
Cyst 3%
Cyst 3%Myelolipom
a 5%
Myelolipom
a 5%
Incidentaloma surrenalicoIncidentaloma surrenalico
Casistica di Orbassano, 1991-2005Casistica di Orbassano, 1991-2005
181 pts181 pts
• AdenomaAdenoma 63% (33-96)not-secreting 75% (52-97)cortisol-secreting 9.5% (1-29)aldosterone-secreting 2.5% (1.6-2.3)estrogen/androgen-secreting very rarely
• PheochromocytomaPheochromocytoma 7% (1.5-23)• CarcinomaCarcinoma 6.5% (1.2-11)• MyelolipomaMyelolipoma 8% (7-15)• CystCyst 5% (4-22)• GanglioneuromaGanglioneuroma 4% (0-6)• MetastasesMetastases 6.5% (0-21)
EPIDEMIOLOGYEPIDEMIOLOGY
What are the causes of incidental adrenal What are the causes of incidental adrenal masses in the general population?masses in the general population?
Average percentage in the literatureAverage percentage in the literature
EPIDEMIOLOGYEPIDEMIOLOGY
Michele Zini 2009
Kopetschke R et al., European Journal of Endocrinology 161 355–361, 2009
INCIDENTALE RISCONTRO
≠
ASINTOMATICO
Michele Zini 2009
INCIDENTALOMA: CHE FARE ?
AACE/AAES Adrenal Incidentaloma Guidelines, Endocr Pract. 2009;15(Suppl 1)Michele Zini 2009
Why pheochromocytoma has to be Why pheochromocytoma has to be recognized?recognized?
Life-threatening condition, particularly if undiagnosed.
Premature mortality.
High morbidity (arrhythmias, sustained or paroxysmal
hypertension, hyperglycemia).
Hypertensive episodes and arrhythmias can result in
emergencies.
Even clinically silent pheochromocytoma can be lethal.
An increasing number of patients with pheochromocytoma An increasing number of patients with pheochromocytoma
have low-grade hypertension or are normotensive.have low-grade hypertension or are normotensive.
ENDOCRINE ASSESSMENTENDOCRINE ASSESSMENT
Terzolo M et al., Best Pract Res Clin Endocrinol Metab. 2009 Apr;23(2):233-43
Mic
hele Z
ini 2
009
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 comepos. neg. esclusi
Sensibilità 93% 79% 92%
Specificità 75% 97% 95%
Potere predittivo positivo 67% 92% 92%
Potere predittivo negativo 95% 90% 95%
Rapporto di probabilitàdel test positivo 3.7 23 20
Rapporto di probabilitàdel 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
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
INCIDENTALOMAIl laboratorio
• PRA• aldosterone• elettroliti
• metanefrine urinarie
• catecolamine plasmatiche
• catecolamine urinarie
• (metanefrine plasmatiche)
• DHEAS• estradiolo
• testosterone
Michele Zini 2009
• cortisolemia
• cortisoluria 24-ore
• ACTH
• test di soppressione (desametazone 1 mg)
INCIDENTALOMA: PRIORITA’
• Escludere neoplasie maligne (primitive o secondarie)
• Escludere feocromocitoma
• Valutazione funzionale corticale
Michele Zini 2009