Pheochromocytoma and multifocal functioning paraganglioma in a 9-year-old boy with von Hippel-Lindau disease.
We report the case of a 9-year-old boy presenting with multifocal paraganglioma partially involving the right suprarenal gland, abdominal paraganglia, and the left para-aortal retropleural region. Von Hippel-Lindau disease (VHL) was diagnosed exclusively by VHL-gene analysis. Other clinical signs of VHL were lacking. After a complaint-free period of 7 months the boy developed a metachronous paraganglioma adjacent to the opposite adrenal gland. Patients with VHL disease should undergo adrenal-sparing surgery.
Tröbs
Ralf-Bodo
RB
Department of Pediatric Surgery, Universitätsklinikum Leipzig AöR, Leipzig, Germany.
Reichardt
Peter
P
Friedrich
Thomas
T
Klöppel
Rainer
R
Bennek
Joachim
J
D000310:Adrenal Gland Neoplasms / Q000150:complications*; D002648:Child; D006801:Humans; D008297:Male; D010235:Paraganglioma / Q000150:complications; D010673:Pheochromocytoma / Q000150:complications*; D006623:von Hippel-Lindau Disease / Q000150:complications*