Published in:
01-09-2016 | Scientific Letter
Recurrent Pheochromocytomas in a Child with Familial von Hippel-Lindau Syndrome
Authors:
Djordje Savic, Maja Milickovic, Sladjana Todorovic, Miroslav Vukadin, Aleksandar Vlahovic, Blagoje Grujic, Mila Stajevic
Published in:
Indian Journal of Pediatrics
|
Issue 9/2016
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Excerpt
To the Editor: von Hippel-Lindau (VHL) syndrome is a rare hereditary disease characterized by the presence of benign or malignant tumors, such are CNS hemangioblastoma, pheochromocytoma, retinal angioma, “clear cell” renal carcinoma, renal and pancreatic cysts [
1]. VHL syndrome is inherited in autosomal dominant pattern with high penetrance, but 20 % of cases are new mutations. Pheochromocytomas have been described in 10–50 % of patients with VHL syndrome [
2]. We present a case of 12-y-old boy with type 2 VHL syndrome who had successive development of bilateral adrenal pheochromocytomas, as well as extraadrenal pheochromocytomas. …