This week's artwork was donated by Lonny, a pediatric cancer patient who received treatment at The Children's Hospital of Philadelphia . The von Hippel-Lindau tumor suppressor protein. Loss of pVHL function leads to deregulation of HIF target genes, which play critical roles in angiogenesis. VHL disease is a rare genetical disease with a poor long-term prognosis. Clinical features include retineous angiomas, CNS hemangioblastomas, pheochromocytomas and renal cysts.
Renal cysts are bilateral and frequently lead to malignant transformation.
Surgical treatment of renal cysts is preferably delayed until a minimum of 3 cm diameter has been reached. Treatment has to be as conservative as possible, knowing that uni- or bilateral nephrectomy often has to be carried out later. The father complained of a malignant hypernephroma, which metastasized in the upper jaw 6 months later. As in this patient, renal cell carcinomas are fatal and are exhibited at an early age. Consequentially, patients with familial von Hippel-Lindau disease exhibiting cases of renal cell carcinoma should be examined very carefully in the head and neck region to detect and treat metastases as early as possible.