|
q
Tumors
of chromaffin tissue which secretes catecholamines in sympathetic NS
(paraganglioma).
q
Very
rare (< 1:1000 cases of hypertension).
q
90%
arise in adrenal, 10% elsewhere in sympathetic chain.
q
10% rule:
|
10%
malignant
|
10% bilateral
|
|
MEN
2A |
|
10%
extra-adrenal
|
10% familial
|
10%
calcify
10% kids
q
Most
tumors release NA & A, but large tumors produce mostly NA.
q
Sipple’s syndrome (MEN 2A) =
Bilateral pheochromocytoma + Medullary thyroid Ca + Parathyroid adenoma.
q
MEN 2B
= MEN 2A + Neurofibromatosis.
Clinical
Features
Clinical features are those of catecholamines
excess.
|
Signs
|
Symptoms
|
|
Pallor
/ Flushing / diaphoresis
|
Stable
/ labile / Paroxysmal Hypertension
|
|
Panic
attack / Anxiety
|
Perspiration
|
|
Tremor
|
Palpitation
|
|
Tachycardia +
arrhythmia
|
Headache
|
|
Raynaud’s
phenomenon
|
|
Diagnosis
1. Clinical.
2. Measurement of
U-metabolites
(metanephrine & VMA) is useful screening test.
3. P- & U-
catecholamines.
4. CT scan:
for tumors.
5. MRI.
6. Scanning with [131I]
MIBG: useful with extra-adrenal tumors.
Treatment
·
Removal
of tumors.
·
a-blockers
(phenoxybenzamine, nitroprusside).
·
b-blockers
(propranolol).
written
by:
Khalid Bin Yaroof. FMHS, UAE University.
| 
