Michalaki and Gennatas Journal of Medical Case Reports 2012, 6:115 http://www.jmedicalcasereports.com/content/6/1/115 C A S E R E P O R T Chromophobe renal cell carcinoma with prolonged response to targeted therapy: a case report Vasiliki Michalaki* and Constantine Gennatas JOURNAL OF MEDICAL CASE REPORTS Open Access Abstract Introduction: Chromophobe renal cell carcinoma is universally accepted as a distinct subtype of renal cell carcinoma. There are conflicting reports on prognosis, and few data on response to treatment exist. Currently, we do not have any effective treatment for the metastatic disease apart from surgical procedures. Current strategies are based on results obtained in the context of clear cell-type renal cell carcinoma. Separate trials for rare histologies seem unfeasible and are unlikely to be performed. For these cases, clinical observations are an important part for advancing therapeutic insight. In recent years, novel tyrosine kinase inhibitors have been shown to have significant clinical benefit in advanced renal cell carcinoma. Case presentation: We present the case of a 43-year-old Caucasian man with advanced chromophobe renal cell carcinoma treated with the tyrosine kinase inhibitor sunitinib and subsequently with sorafenib and the mammalian target of the rapamycin inhibitor everolimus, achieving a prolonged response and significant clinical benefit. We report an unexpectedly high efficacy of everolimus as a third-line treatment in a patient with metastatic chromophobe renal cell carcinoma. Conclusions: Up to now, no published data from randomized clinical studies have addressed the question of efficacy of everolimus as a third-line treatment after failure of tyrosine kinase inhibitors. To the best of our knowledge, this case is the first report of chromophobe renal cell carcinoma treated successfully with sequential tyrosine kinase and mammalian target of rapamycin inhibitor therapy. Notably, the time on treatment with sunitinib exceeded four years. The case presented here implies that everolimus could be a viable option for patients with metastatic chromophobe renal cell carcinoma. Introduction Renal cell carcinoma (RCC) accounts for approximately 1% of all cancers, and the incidence of kidney cancer, unlike other genitourinary malignancies, is rapidly increas- ing at 2.5% per year. Chromophobe RCC (ChRCC) repre- sents 5% of neoplasms of renal tubular epithelium. ChRCC is a distinct type of renal cancer, is presumably derived from the intercalated cells of the collecting duct system, and exhibits a better prognosis than other types of RCC. An enhanced understanding of the underlying biology of RCC has led to systemic therapy targeted at the vascu- lar endothelial growth factor (VEGF) pathway as well as * Correspondence: vmichalaki@usa.net Oncology Clinic, Second Department of Surgery, Areteion Hospital, University of Athens, 76 V. Sofias av, 115 28, Athens, Greece the the mammalian target of the rapamycin (mTOR) pathway. Agents blocking these pathway elements have demonstrated robust efficacy, offer new strategic options for patients with metastatic RCC, and have largely replaced cytokines as the standard of care in this disease. Trials with small-molecule VEGF and platelet-derived growth factor (PDGF) receptor inhibitors, such as suniti- nib and sorafenib, have shown significant clinical activity in randomized trials in advanced clear cell RCC (CCRCC). Both drugs have been approved by the US Food and Drug Administration for the treatment of metastatic RCC. Data regarding the activity of sunitinib and sorafenib in advanced ChRCC are lacking because recent trials were restricted mostly to patients with CCRCC. © 2012 Michalaki and Gennatas; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.