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adrenal glands 4 tumors (Pheochromocytoma (Diagnosis (Imaging (• CT scans…
adrenal glands 4 tumors
Pheochromocytoma
- rare tumors that produce, store, and secrete catecholamines.
- Clinical syndrome caused by hypersecretion of catecholamines
- Can occur in any sympathetic ganglion in the body
- More than 90% from adrenal medulla (10% extra-adrenal)
- Arise from the chromaf n cells of the adrenal medulla or from the sympathetic ganglia if extra-adrenal.
- „A rule of 10”: 10% extradrenal, bilateral, malignant, part of MEN IIA or IIB, familial syndromes (vHL, NF), 10x more often in children
manifestation
- due to catecholamine excess often intermittent
-Hypertension – paroxysmal or constant
-Triad: headache, palpitations, sweating
-Pallor > flushing
-Anxiety, panic, tremor
-Abdominal and chest pain
-Constipation or diarrhoea
-Orthostatic hypotension
Clinical features
Precipitation of symptoms by:
• emotional stress,
• exercise
• abdominal pressure
• anesthesia
• tyramine-containing foods
Diagnosis
- Plasma metanhephrine and normetanephrine
-metanephrine > 0,61 nmol/l
-normetanephrine >0,31 nmol/l
- Methanephrines in 24-hour urine collection (2-3x)
- Plasma chromogranin A
- Clonidine suppression test
-0,3mg/kg orally, plasma catecholamines measured before and after 3 hours
- Urine/serum epinephrine and norepinephrine levels—if the epinephrine level is elevated, the tumor must be adrenal or near the adrenal gland (organ of Zuckerkandl) because nonadrenal tumors cannot methylate norepinephrine to epinephrine
Imaging
• CT scans of the abdomen - tumor is often large
• MRI with „chemical shift”
• Extra-adrenal tumors
-Scanning with 131I-MIBG – specific uptake in sites of sympathetic activity
-Octreoitide scan
• PET (positron emission tomography)
Laboratory ndings: hyperglycemia, hyperlipidemia, hypokalemia
Treatment
- Surgical - if this is possible
- Medical preoperative and perioperative treatment
-α-blockade: phenoxybenzamine, prazosine (up to 2 weeks before)
-Prior or during surgery: β-blockade
-In case of sudden severe hypertension: sodium nitroprusside infusion
-Plasma volume repletion (blood transfusion, colloids)
- Chemotherapy or radionuclide treatment with 131I-MIBG in malignant pheochromocytoma
- rule of 10s for Pheochro- mocytoma tumors
• 10%arefamilial
• 10% are bilateral (suspect MEN type II)
• 10%aremalignant
• 10%aremultiple
• 10%occurinchildren
• 10%areextra-adrenal (more often malignant)— The most common site is the organ of Zuckerkandl, which is located at the aortic bifurcation.
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ADRENAL MALIGANCIES
adrenocortical carcinoma
- 50-70% of adrenocortical carcinomas secrete steroid hormones
- Functioning secrete: cortisol, androgens or aldosterone alone or in combination
- Nonfunctioning - present clinically as abdominal or flank pain, a palpable tumor or as an adrenal mass discovered by an imaging procedure
- most suggestive clues of malignancy:
-rapidly progressive CS or/and virilisation in woman
-tumor size greater than 6 cm and evidence of locally invasive or metastastic disease (liver, lungs)
-↑↑ urinary 17 KS, ↑↑ DHEA
-malignancy is frequently not suspected until the tumor is examined histological
(non-secreting, „ silent” tumors)
- TREATMENT:
Surgery is the treatment of choice
Mitotane is the drug of choice
-produces partial or complete tumor regression
-reduces production of adrenal hormones
-improves survival
-should be started immediately after surgery
Other chemotherapeutic agents and radiation therapy are not effective
- PROGNOSIS:
The mean survival is about 15 months
The 5-year survival rate is approximately 20%
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