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Pheochromocytoma presenting as stroke in two Taiwanese children
Yang-Hau Van1, Huei-Shyong Wang, Ching-Horng Lai
1Division of Endocrinology, Chang Gung Children 's Hospital, Taoyuan, Taiwan.
Insights
Pheochromocytoma, a rare tumor causing pediatric hypertension, can present as stroke. Surgical removal of these tumors in children cured their hypertension and prevented recurrence.
Area of Science:
- Pediatric Endocrinology
- Pediatric Oncology
- Pediatric Neurology
Background:
- Pheochromocytoma is a rare neuroendocrine tumor, accounting for approximately 1% of pediatric hypertension cases.
- Early diagnosis is crucial as hypertension associated with pheochromocytoma is often curable through tumor eradication.
Observation:
- This report details two Taiwanese children diagnosed with pheochromocytoma, with initial presentations mimicking neurological events.
- Patient 1 experienced a cerebral infarction, while Patient 2 suffered an intracranial hemorrhage.
- Diagnostic imaging revealed an extraadrenal tumor in Patient 1 and an adrenal mass in Patient 2.
Findings:
- Elevated 24-hour urinary norepinephrine excretion confirmed pheochromocytoma in both patients (1480.8 µg/day and 3279 µg/day).
- Preoperative alpha- and beta-adrenergic blockade effectively managed hypertension.
- Successful surgical resection of the tumors normalized blood pressure and urinary catecholamine levels without requiring further medication.
Implications:
- Pheochromocytoma should be considered in the differential diagnosis of pediatric stroke or unexplained severe hypertension.
- Complete tumor resection offers a curative solution for hypertension in pediatric pheochromocytoma.
- Long-term follow-up demonstrated sustained normotension in both patients post-surgery.
Abstract:
Pheochromocytoma is a rare tumor and accounts for about 1% of pediatric hypertension. Its diagnosis is important because the hypertension is usually curable by eradication of the tumor. We report two Taiwanese children with pheochromocytoma presenting as stroke. Patient 1 developed a cerebral infarction and patient 2 had an intracranial hemorrhage. Abdominal MRI of patient 1 showed an extraadrenal tumor 5.1 x 4 x 4.7 cm in size and abdominal CT of patient 2 revealed a left adrenal mass 4 x 2.9 x 4 cm in size. 24-h urinary excretion of norepinephrine for patients 1 and 2 were 1480.8 and 3279 microg/day, respectively. Preoperative alpha- and beta-adrenergic blockade was used for hypertension control. Both patients underwent successful tumor resection, upon which blood pressure and 24-h urinary excretion of catecholamines returned to normal without any further medication. They have been free from hypertension for 4 years (patient 1) and 2 years (patient 2).