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Experience with surgical excision in childhood pheochromocytoma
Hyun-Young Kim1, Hye Seung Lee, Seung-Eun Jung
1Department of Surgery, Seoul National University College of Medicine, Seoul, Korea. khy@medimail.co.kr
Insights
Pediatric pheochromocytoma, a dangerous cause of high blood pressure, requires early diagnosis and surgical removal. Long-term follow-up is crucial for managing this condition in children.
Area of Science:
- Pediatric Endocrinology
- Pediatric Oncology
- Surgical Oncology
Background:
- Pheochromocytoma is a rare but potentially fatal tumor causing hypertension in children.
- Surgical management and long-term outcomes in pediatric cases are not well-established.
Purpose of the Study:
- To analyze the clinical characteristics and long-term surgical outcomes of pheochromocytoma in pediatric patients.
- To identify key factors for successful treatment and management.
Main Methods:
- Retrospective review of 15 children treated for pheochromocytoma between 1984 and 2002.
- Analysis of tumor location, disease extent, surgical procedures, recurrence rates, and follow-up duration.
Main Results:
- 15 children (average age 11.7 years) underwent surgery for pheochromocytoma.
- 11 tumors were adrenal, 4 extra-adrenal; 14 localized, 1 regional disease.
- 26.7% recurrence rate observed, with one patient experiencing three recurrences.
Conclusions:
- Early diagnosis and surgical excision are vital for treating pediatric pheochromocytoma.
- Long-term follow-up is essential to monitor for recurrence and ensure optimal patient outcomes.
Abstract:
Pheochromocytoma is one of the potentially fatal causes of childhood hypertension. The study aims to analyze the results of our experiences in pheochromocytomas and the long-term results of its surgical treatment in children. The records of 15 children (11 boys, 4 girls) treated for pheochromocytoma in our unit during the period of 1984 and 2002 were reviewed retrospectively. The average age at surgery was 11.7 yr (range 6 yr 9 months-15 yr 7 months). Localized disease is defined as the cases without the invasion of surrounding tissue, regional disease as the invasion of surrounding tissue and metastatic disease as distant metastases. The mean follow-up after pheochromocytoma excision was 95 months (range 5 to 221 months). Tumors were located in the adrenal gland in 11 (bilaterally in 4) and extra-adrenally in 4. Localized disease occurred in 14 patients and regional disease in one. Only one patient was associated with von Hippel Lindau syndrome. Adrenalectomy or total excision of extra adrenal tumor was performed. Four patients (26.7%) recurred after the first operation (at 2 yr 9 months to 14 yr) and regional disease recurred in one patient three times. Early diagnosis, surgical excision, and long-term follow up are most important for the appropriate treatment of childhood pheochromocytoma.