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Background: Anaplastic lymphoma kinase (ALK)-rearranged renal cell carcinoma (ALK-RCC) is a rare provisional entity characterized by fusion of the ALK gene with various fusion partner genes, resulting in oncogenic activation. Case presentation: We report a case of a 39-year-old Chinese man with ALK-RCC who underwent partial nephrectomy for a left renal tumor measuring 2.8 × 2.7× 2.6 cm. Histopathological examination revealed a renal epithelial tumor with atypical morphology. Immunohistochemistry demonstrated the occurrence of renal epithelial differentiation and excluded other entities. Next-generation sequencing confirmed a KIF5B-ALK gene fusion. Conclusion: This case presents the clinical data, morphology, immunohistochemistry, and molecular characteristics of a rare subtype of ALK-rearranged renal cell carcinoma with KIF5B-ALK fusion. The findings may enhance the clinical and pathological understanding of ALK-RCC among clinicians and pathologists. Managing ALK-RCC is challenging due to its rarity and limited treatment options. Targeted therapies directed at the ALK gene may have a role in patients with ALK-RCC.
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Yao et al. (2026) studied this question.
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