Partial adrenalectomy minimizes the need for long-term hormone replacement in pediatric patients with pheochromocytoma and von Hippel-Lindau syndrome

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• 作者：Dmitry Volkin¹ ; Nitin Yerram¹ ; Faisal Ahmed ; Dawud Lankford ; Angelo Baccala ; Gopal N. Gupta ; Anthony Hoang ; Jeffrey Nix ; Adam R. Metwalli ; David M. Lang ; Gennady Bratslavsky ; W. Marston Linehan ; Peter A. Pinto ; ^{pintop@mail.nih.gov}
• 关键词：Adrenalectomy ; Partial adrenalectomy ; Pediatric VHL ; Pheochromocytoma
• 刊名：Journal of Pediatric Surgery
• 出版年：2012
• 出版时间：November, 2012
• 年：2012
• 卷：47
• 期：11
• 页码：2077-2082
• 全文大小：365 K

文摘

Purpose

Children with von Hippel-Lindau syndrome (VHL) are at an increased risk for developing bilateral pheochromocytomas. In an effort to illustrate the advantage of partial adrenalectomy (PA) over total adrenalectomy in children with VHL, we report the largest single series on PA for pediatric patients with VHL, demonstrating a balance between tumor removal and preservation of adrenocortical function.

Methods

From 1994 to 2011, a prospectively maintained database was reviewed to evaluate 10 pediatric patients with hereditary pheochromocytoma for PA. Surgery was performed if there was clinical evidence of pheochromocytoma and if normal adrenocortical tissue was evident on preoperative imaging and/or intraoperative ultrasonography. Perioperative data were collected, and patients were observed for postoperative steroid use and tumor recurrence.

Results

Ten pediatric patients with a diagnosis of VHL underwent 18 successful partial adrenalectomies (4 open, 14 laparoscopic). The median tumor size removed was 2.6 cm (range, 1.2-6.5 cm). Over a median follow-up of 7.2 years (range, 2.6-15.8 years), additional tumors in the ipsilateral adrenal gland were found in 2 patients. One patient underwent completion adrenalectomy, and 1 underwent a salvage PA with resection of the ipsilateral lesion. One patient required short-term steroid replacement therapy. At last follow-up, 7 patients had no radiographic or laboratory evidence of pheochromocytoma.

Conclusion

At our institution, PA is the preferred form of management for pheochromocytoma in the (VHL) pediatric population. This surgical approach allows for removal of tumor while preserving adrenocortical function and minimizing the adverse effects of long-term steroid replacement on puberty and quality of life.