National Cancer Institute®
Last Modified: July 1, 2002
UI - 12032534
AU - Franklin SL; Eisenhofer G; Geffner ME
TI - Simultaneous development of pheochromocytomas in prepubertal siblings with von Hippel-Lindau syndrome.
SO - J Pediatr 2002 May;140(5):625-8
AD - Division of Endocrinology, Department of Pediatrics, Mattel Children's Hospital at UCLA, Los Angeles, California 90095, USA.
We report 2 siblings with a known von Hippel-Lindau mutation who simultaneously developed asymptomatic pheochromocytomas at significantly younger ages than are typically seen in this setting.
UI - 11865075
AU - Wiesener MS; Eckardt KU
TI - Erythropoietin, tumours and the von Hippel-Lindau gene: towards identification of mechanisms and dysfunction of oxygen sensing.
SO - Nephrol Dial Transplant 2002 Mar;17(3):356-9
UI - 12084291
AU - Maranchie JK; Walther MM
TI - Early identification of patients with von Hippel-Lindau disease at risk for pheochromocytoma.
SO - Curr Urol Rep 2001 Feb;2(1):24-30
AD - Urologic Oncology Branch, National Cancer Institute, 10101 Grosvenor Place, #518, North Bethesda, MD 20852, USA. Maranchj@mail.nih.gov
von Hippel-Lindau disease (VHL) is an autosomal dominant familial syndrome that predisposes to the formation of tumors in multiple organ systems, including adrenal and extra-adrenal pheochromocytomas. However, fewer than 30% of VHL families develop pheochromocytomas. In recent years, this clinical heterogeneity has been correlated with missense mutations. The VHL patient requires vigilant, lifelong biochemical and radiographic screening for pheochromocytoma. Half of VHL pheochromocytomas present bilaterally, and there is a high incidence of recurrence after surgery. Because of the morbidity of bilateral total adrenalectomy with subsequent steroid replacement therapy, the recent therapeutic trend has been toward observation and minimally invasive adrenal-sparing procedures.
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