The gradual expansion of multiple intramedullary metastatic renal cell carcinoma in a patient with von Hippel-Lindau disease with multiple intramedullary hemangioblastomas: illustrative case.

backgroundPatients with von Hippel-Lindau disease (VHLD) often have multiple intramedullary tumors, including hemangioblastoma and rarely intramedullary metastatic renal cell carcinoma (RCC). However, the incidence of intramedullary metastatic clear-cell RCC (CCRCC) comorbid with hemangioblastoma is not clear. OBSERVATIONS: The authors report a rare case of intramedullary metastatic CCRCC comorbid with multiple hemangioblastomas in a patient with VHLD. The preoperative differential diagnosis by MRI was difficult. She underwent three surgical removals. After removal of hemangioblastoma at the first surgery, the juxtaposed tumor grew fast during 3 years. Moreover, 4 years after the second surgery, one of the two adjacent increasing tumors grew faster than another. The pathological diagnoses of both fast-growing tumors were intramedullary metastatic CCRCC, not hemangioblastoma. This patient has no neurological deficits and is now being followed up as an outpatient without any further treatment. LESSONS: Although a rare metastatic intramedullary CCRCC is difficult to differentiate from comorbid hemangioblastomas in patients with VHLD, it is important to note that the tumor growth rate differs between hemangioblastoma and metastatic CCRCC, which can be followed using sequential MRI. Care must be taken not to dismiss the timing of the removal of metastatic intramedullary tumors. https://thejns.org/doi/10.3171/CASE25459.