Changes to This Summary (09/26/2014)
The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.
Added text to state that patients with von Hippel-Lindau syndrome can also develop pancreatic cysts, cystadenomas, and pancreatic neuroendocrine tumors (NETs). Also added text to state that pancreatic cysts and cystadenomas are not malignant, but pancreatic NETs possess malignant characteristics and are typically resected if they are 3 cm or larger (cited Libutti et al. as reference 54). Also added that a review of the natural history of pancreatic NETs shows that these tumors may demonstrate nonlinear growth characteristics (cited Weisbrod et al. as reference 55).
Added text to state that a case series of 14 patients with histories of surgical resection demonstrated effective disease control with minimal decrease in renal function after thermal ablation (cited Yang et al. as reference 62).
Added level of evidence 2.
Added text to state that a series from the National Cancer Institute found that 20 of 255 patients with hereditary leiomyomatosis and renal cell cancer (HLRCC) had adrenal nodules, some of which did not appear to be adenomas on the basis of imaging characteristics; because many of these lesions were fluorodeoxyglucose avid, resections were performed and all showed evidence of both micronodular and macronodular adrenal hyperplasia, suggesting that adrenal nodules could be an additional manifestation of HLRCC (cited Shuch et al. as reference 104).
Added text to state that there are some published recommendations to perform skin exams on a regular basis, but there is no consensus regarding frequency of skin exams, and these recommendations have not been prospectively validated.
Added level of evidence 5.
Added level of evidence 4.
Added level of evidence 4.
Added text to state that loss of oxidative phosphorylation resulting from biallelic inactivation of FH renders HLRCC tumors almost entirely reliant on aerobic glycolysis for meeting cellular adenosine triphosphate and other bioenergetics requirements. Consequently, aerobic glycolysis is being explored as a therapeutic strategy (cited Xie et al. and Yamasaki et al. as references 125 and 126, respectively). Revised text to state that a phase II study examining the combination of bevacizumab and erlotinib for the treatment of advanced HLRCC is ongoing and is based partly on the premise that this combination might inhibit effective glucose delivery to tumor cells (cited Linehan et al. as reference 127).
This summary is written and maintained by the PDQ Cancer Genetics Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ NCI's Comprehensive Cancer Database pages.