A patient with germline von Hippel-Lindau (VHL) gene alteration and history of multiple tumors present with classical paraneoplastic syndrome (PNS) associated with renal cell carcinoma (RCC). She underwent open nephron sparing surgery with resolution of symptoms. She remained without recurrence of RCC for the initial 2 years of her follow-up. To the best of our knowledge, this case represents the first in which PNS was specifically resolved using a partial nephrectomy in a patient with VHL. This case report provides initial evidence for the potential role of nephron sparing surgery in the management of paraneoplastic symptoms associated with hereditary RCC.
Urology case reports. 2017 Apr 27*** epublish ***
John M DiBianco, Julie Y An, Sally Tanakchi, Zachary Stanik, Aidan McGowan, Mahir Maruf, Abhinav Sidana, Amit Lodha Jain, Akhil Muthigi, Arvin K George, Christopher Bayne, W Marston Linehan, Shawna L Boyle, Adam R Metwalli
School of Medicine and Health Sciences, The George Washington University, Washington, DC, USA., Urologic Oncology Branch, Center for Cancer Research, National Cancer Institute, Bldg. 10, Hatfield Clinical Research Center, Rm 2 W-3952 10 Center Drive MSC 1210, 20892-1210, Bethesda, MD, USA., Laboratory of Pathology, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA.