Michalaki and Gennatas Journal of Medical Case Reports 2012, 6:115 http://www.jmedicalcasereports.com/content/6/1/115 In this report, we describe the case of a patient who had ChRCC and who experienced improvement in his general condition and stable disease on treatment with everolimus after a prolonged response to sunitinib treat- ment. This case report suggests that everolimus is an effective and appropriate treatment for this RCC tumor subtype. Case presentation A 43-year-old Caucasian man presented with painless macroscopic hematuria of sudden onset and gradual deterioration. Ultrasound of his kidneys demonstrated a mass lesion with abnormal echogenicity and vasculariza- tion in the upper pole of his right kidney. A computed tomography scan of the retroperitoneal space showed a malignant space-occupying mass in the upper pole of his right kidney. He underwent right radical nephrectomy in 2003. The nephrectomy specimen showed a 20 × 12 × 8 cm tumor at the upper pole with a homoge- nous, solid light brown cut surface. The tumor was limited to his kidney. The sections stained by hematoxylin and eosin showed sheets of polygonal cells with abundant granular eosinophilic cytoplasm with oval nuclei, convo- luted nuclear membranes, and perinuclear cytoplasmic vacuolization. Histology revealed ChRCC. Three years after nephrectomy, a routine abdominal scan showed a recurrence in his liver (Figure 1) and in Page 2 of 5 bones in the chest area. The disease was considered advanced, and sunitinib 50 mg once daily on the 4/2 sche- dule (four weeks on treatment and two weeks off treat- ment cycle) was commenced. He also started zoledronic acid infusions and was treated with CyberKnife® for bone metastases. A dose reduction to 37.5 mg after three months for gastrointestinal adverse effects was necessary. Our patient had a partial response to treatment and had a maximum response at nine months (Figure 2). Sunitinib was continued for four years despite some increase in bone disease burden but was stopped at 50 months as a consequence of disease progression in his liver. Sorafenib 800 mg daily was started after cessation of sunitinib for progressive disease. Follow-up imaging demonstrated stable disease. However, the next follow-up, three months later, showed progressive disease in his liver and bones. Everolimus 10 mg daily was started. At the time of this report, five months after the commencement of everoli- mus, there is radiologic evidence (Figure 3) of ongoing incremental reduction in liver disease volume, and our patient feels well and is tolerating treatment with no sig- nificant toxicity. To the best of our knowledge, this case is the first report of ChRCC treated successfully with sequential tyrosine kinase and mTOR inhibitor therapy. Notably, the time on treatment with sunitinib exceeded four years. Figure 1 An abdominal scan shows recurrence in the liver (multiple lesions).