Pheochromocytoma of the Urinary Bladder

Journal review: “Pheochromocytoma of the Urinary Bladder” by Fu-Chi Yang & Yu-Chuan Hsu & Chun-Wen Chen et all from European Journal of Pediatric (2010) 169:127–129

Author reported a case of 16 years old girl presented to ED with the chief complaint of severe headache which is

1. Two days duration

2. Since past three years and treated as migraine

3. Holocranial in location

4. Pulsating in nature

5. Lasted about 30-60 minutes

6. Increasing in severity and frequency

7. Provoked by micturation and strenuous physical exercise

Patient develops hypertensive crisis and extremely intense headache after admission which is reduce by infusion of sodium nitroprusside and Labetolol.

Investigation done

1) Brain CT (normal)

2) Echocardiography (no cardiac abnormality)

3) Abdominal US (No adrenal lesion)

4) CT scan of abdomen and pelvis (lobulated hypervascular mass in anteroinferior wall of urinary bladder)

Therefore, 24 hr urinary excretion was done and found to be elevated which is (893.9 μg/day; normal range, 15–80 μg/day)

Her Bp was then controlled with phenoxybenzamine, propranolol, and captopril 2 weeks prior to laporatomy with complete excision including wide tumor free margin and partial cystectomy. All hypertensive drugs were discontinued after surgery and her headache disappear.

Lesson to be learned

1) Evaluate between primary benign headache (including migraine and tension) and secondary headache

2) Most of headache in pediatric are benign but may be secondary to more serious pathology.

3) look at associating symptom that might point up to diagnosis. Like in this patient, the relationship is between headache and micturation, sympathetic symptoms and hypertensive crisis.

Discussion

Pheochromocytoma is a catecholamine secreting tumor causing essential hypertension that arises from chromaffin cells of sympathetic nervous system.

It is usually found in adrenal gland. If not, then it is called as extra adrenal Pheochromocytoma or paragangliomas.

Incidence of bladder pheochromocytoma is rare which 0.06% of all bladder tumors. It occurs between ages 11 to 78 years with peak ages is third to fifth decade of life.

Compared to adrenal pheochromocytoma, paragangliomas are easily to recur and metastasize therefore regular blood pressure monitoring, annual determination of catecholamines, periodic endocrine and radiological evaluation is needed following surgery.