Neuroendocrine tumor of the pancreas and bilateral adrenal pheochromocytomas. A rare manifestation of von Hippel–Lindau disease in childhood B Jan M. Langrehr a, * , Marcus Bahra a , Glen Kristiansen b , Hartmut P.H. Neumann f , Luitgard M. Neumann c , Ursula Plo ¨ ckinger d , Enrique Lopez-Ha ¨nninen e a Department for General, Visceral and Transplantation Surgery, Charite ´, Campus Virchow Clinic, 13353 Berlin, Germany b Institute of Pathology, Charite ´, 10117 Berlin, Germany c Institute of Human Genetics, Charite ´, 13353 Berlin, Germany d Department for Gastroenterology and Hepatology, Charite ´, 13353 Berlin, Germany e Institute for Radiology, Charite ´, 13353 Berlin, Germany f Department of Nephrology and General Medicine, Albert-Ludwigs University, 79095 Freiburg, Germany Abstract Background: A 12-year-old girl presenting with intermittent epigastric pains and diarrhea was referred to our clinic. Diagnostic workup revealed nonfunctional bilateral adrenal pheochromocytomas as well as a neuroendocrine tumor of the pancreatic head. This is the first report on the combination of a neuroendocrine pancreatic tumor with adrenal pheochromocytoma in a pediatric patient with von Hippel–Lindau (VHL) disease. Methods: von Hippel–Lindau disease was confirmed by molecular genetic analysis of peripheral blood lymphocytes, which revealed the mutation VHL c. 695 G N A. The family history showed also VHL disease in the mother who carried the same mutation. Results and Conclusion: Open laparotomy, organ-sparing enucleation of pheochromocytoma, and pylorus-preserving resection of the pancreatic head tumor were successfully performed. After an uneventful postoperative course, the child fully recovered. She was free of further manifestations of VHL disease 30 months after surgery. D 2007 Elsevier Inc. All rights reserved. In 1936, Davison et al [1] referred to the development of hemangioblastomas in the central nervous system and in the retina as the von Hippel–Lindau syndrome, with or without the simultaneous occurrence of clear-cell renal carcinomas, pheochromocytomas, and/or tumors of the pancreas. In 1894, Collins [2] had already reported bilateral occurrence of retinal hemangioblastomas in twins, which were then referred to as naevi . The German ophthalmologist von Hippel, however, referred to this entity as angiomatosis retinae [3]. Several years later, in 1926, the Swedish pathologist Lindau reported that patients with angiomatosis 0022-3468/$ – see front matter D 2007 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2007.02.029 B Hartmut P.H. Neumann is supported by the Deutsche Krebshilfe (grant no. 70-3313-Ne 1), the Deutsche Forschungsgemeinschaft (grant no. NE 571/5-2), and the European Union (LSHC-CT-2005-518200). * Corresponding author. Tel.: +40 30 450 552001; fax: +49 30 450 552900. E-mail address: jan.langrehr@charite.de (J.M. Langrehr). Index words: von Hippel–Lindau disease; Pancreatic head resection; Adrenal surgery Journal of Pediatric Surgery (2007) 42, 1291 – 1294 www.elsevier.com/locate/jpedsurg