Management of pediatric patients with pheochromocytomas associated with genetic syndromes, such as Von Hippel-Lindau (VHL) disease, is complex owing to the need for continuous surveillance, high rates of recurrence, multiple operations during childhood, and the possibility of lifelong exogenous steroid replacement. We report the successful treatment of two siblings with VHL who underwent a total of six cortical-sparing procedures without requiring steroid therapy.
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- von Hippel-Lindau's disease and phaeochromocytoma.Br Med J. 1971; 1: 441
- Mutations in the RET proto-oncogene and the von Hippel-Lindau disease tumour suppressor gene in sporadic and syndromic phaeochromocytomas.J Med Genet. 1995; 32: 934-937
- Recent advances in the diagnosis and treatment of pheochromocytoma in children.Am J Surg. 2007; 194 ([discussion 796-7]): 792-796
- Frequent genetic changes in childhood pheochromocytomas.Ann N Y Acad Sci. 2006; 1073: 166-176
- Cortical-sparing adrenalectomy for patients with bilateral pheochromocytoma.Surgery. 1996; 120 ([discussion 1070-1]): 1064-1070
- Phaeochromocytoma.Lancet. 2005; 366: 665-675
- Phaeochromocytoma in children.Arch Dis Child. 2008; 93: 899-904
- Radiation exposure from CT scans in childhood and subsequent risk of leukaemia and brain tumours: a retrospective cohort study.Lancet. 2012; 380: 499-505
- Surgical management of hereditary pheochromocytoma.J Am Coll Surg. 2004; 198 ([discussion 534-5]): 525-534
- Risk-oriented approach to hereditary adrenal pheochromocytoma.Ann N Y Acad Sci. 2006; 1073: 417-428
- Laparoscopic Partial Adrenalectomy for Bilateral Pheochromocytomas.Ann Surg Oncol. 2008; 15: 2506-2508
- Pheochromocytoma: inherited associations, bilaterality, and cortex preservation.Surgery. 2000; 128 ([discussion 1011-2]): 1007-1011
- Functional and oncologic outcomes of partial adrenalectomy for pheochromocytoma in patients with von Hippel-Lindau syndrome after at least 5 years of followup.J Urol. 2010; 184: 1855-1859
- Laparoscopic partial adrenalectomy for recurrent pheochromocytoma after open partial adrenalectomy in von Hippel-Lindau disease.J Endourol. 2002; 16: 171-174
- Intraoperative haemodynamic stability in patients with phaeochromocytoma-minimally invasive vs conventional open surgery.Clin Endocrinol (Oxf). 2006; 65: 352-358
- Laparoscopic adrenalectomy for pheochromocytoma: a literature review.Biomed Pharmacother. 2002; 56: 132s-138s
- Laparoscopic adrenalectomy in children.Surg Endosc. 2001; 15: 156-160
Accepted: April 3, 2013
Received in revised form: April 2, 2013
Received: December 31, 2012
☆Disclosures: The authors have no disclosures or sources of support to report with respect to the preparation of this manuscript.
© 2013 Elsevier Inc. Published by Elsevier Inc. All rights reserved.