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Pheochromocytoma in children: 15 cases
1Hôpital des Enfants Malades, Paris, France.
Journal of Pediatric Surgery
|July 1, 1992
Summary
Pheochromocytoma, a rare childhood tumor, affects 20% of patients, often presenting as hypertension. This review details diagnosis and management in pediatric cases, including those with multiple endocrine neoplasia syndromes.
Area of Science:
- Pediatric Endocrinology
- Oncology
Background:
- Pheochromocytoma is a rare neuroendocrine tumor, with 20% of cases occurring in children.
- Hypertension is the most common symptom, presenting variably from sustained to paroxysmal.
- Pediatric pheochromocytomas can be unilateral, bilateral, extra-adrenal, familial, or recurrent.
Purpose of the Study:
- To review a 28-year experience with pediatric pheochromocytoma.
- To focus on diagnostic and management strategies.
- To investigate the association with multiple endocrine neoplasia (MEN) syndromes.
Main Methods:
- Retrospective review of 15 pediatric patients treated between 1962 and 1990.
- Analysis of clinical presentation, diagnostic methods, and surgical outcomes.
- Evaluation for familial inheritance and co-occurrence of MEN syndromes.
Main Results:
- Pheochromocytoma was diagnosed in 1% of pediatric patients investigated for hypertension.
- The majority of pediatric patients presented with hypertension, often paroxysmal.
- The study included diverse cases, such as unilateral, bilateral, extra-adrenal, familial, and recurrent tumors.
Conclusions:
- Pheochromocytoma requires careful diagnosis and management in children.
- Preoperative and operative strategies are crucial for successful treatment.
- Screening for multiple endocrine neoplasia syndromes is important in pediatric pheochromocytoma cases.