Intramedullary spinal cord metastasis of clear cell renal carcinoma in a Von Hippel–Lindau patient

BACKGROUND: Intramedullary spinal cord metastasis is uncommon and represents only 0.6% of all spinal tumors. Renal cell carcinoma is even less frequent in this group than in lung and breast cancer. Patients with Von Hippel– Lindau disease (VHLd) present spinal hemangioblastoma more frequently. CASE DESCRIPTION: A 59-year-old female patient presented with medullary syndrome. There was a previous history of VHLd, with a cerebellar hemangioblastoma resection years ago. The radiological investigation showed a cervical intramedullary solid-cystic lesion. The patient has submitted a tumor resection, and a pathological and immunohistochemistry study confirmed clear cell renal carcinoma metastasis. CONCLUSION: In patients with VHLd, the presence of an intramedullary solid-cystic lesion may not represent always a hemangioblastoma. Other diagnostic possibilities must be evaluated, despite being epidemiologically less frequent. Inside the group of patients with VHLd, only a previous case of intramedullary spinal cord renal cell carcinoma was reported in the literature.