Pheochromocytoma in the pediatric age group: current status
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Pediatric pheochromocytoma presents unique challenges, with children experiencing more familial and extraadrenal tumors. Adrenergic blockade significantly reduces complications, leading to better outcomes in young patients.
Area of Science:
- Pediatric Endocrinology
- Oncology
- Surgical Management
Background:
- Pheochromocytoma is rare in children, differing from adult presentations.
- Pediatric cases show higher rates of extraadrenal, bilateral, and multiple tumors.
- Increased incidence of Multiple Endocrine Neoplasia (MEN) and familial disease in children.
Purpose of the Study:
- To evaluate the characteristics and outcomes of pheochromocytoma in pediatric patients.
- To highlight differences between pediatric and adult pheochromocytoma.
- To assess the impact of management strategies, including adrenergic blockade, on patient outcomes.
Main Methods:
- Retrospective review of 16 pediatric patients (≤17 years) with pheochromocytoma.
- Analysis of tumor characteristics, genetic associations (MEN, familial), and surgical outcomes.
- Comparison of outcomes with and without routine alpha- and beta-adrenergic blockade preoperatively.
Main Results:
- Of 14 survivors, 3 had MEN type 2, 2 had familial pheochromocytoma, and 1 had malignant paragangliomas.
- Patients operated on before routine blockade had higher hypertensive complications (4/5), including one fatality.
- No intraoperative complications occurred in 12 patients receiving appropriate blockade.
- 11/14 survivors are normotensive; 2 have controlled hypertension; 1 has severe hypertension.
Conclusions:
- Pediatric pheochromocytoma has distinct features requiring specialized management.
- Adrenergic blockade is crucial for preventing perioperative hypertensive crises and improving outcomes.
- High-resolution computed tomography is the preferred imaging modality for tumor localization.
Abstract:
Pheochromocytoma is an unusual tumor in the pediatric age group. Several aspects serve to differentiate children with pheochromocytoma from their adult counterparts. Children have fewer malignant tumors, more extraadrenal tumors, and greater bilaterality and multiplicity of tumor. Similarly, they have an increased incidence of multiple endocrine neoplasia (MEN) and familial disease. The records of 16 children (age 17 years and younger) with pheochromocytoma were evaluated. One newborn died at 36 hours of age, and a 3-year-old died four days after exploratory laparotomy. Of the 14 survivors (with an average follow-up of 8 years), three had manifestations of multiple endocrine neoplasia type 2 and two had familial pheochromocytoma only. One patient had malignant paragangliomas. Three patients had bilateral tumors; one of these had MEN. Four patients underwent operation before the use of alpha- and beta-adrenergic blockade was routine at our institution. An additional patient received blockade in preparation for the removal of the pheochromocytoma but was not prepared for preoperative angiography. Hypertensive complications were encountered in four of these five children; in one, this complication was fatal. There were no intraoperative complications in the 12 patients who had received appropriate blocking agents. Eleven of the 14 survivors are currently normotensive without the need for antihypertensive medication. Two patients have medically controlled hypertension, and one continues to have severe hypertension. An overview of this series underscores the characteristic features of pheochromocytoma in the pediatric age group. Angiography has been replaced by high-resolution computed tomography for localization.(ABSTRACT TRUNCATED AT 250 WORDS)