Journal
INTERNATIONAL JOURNAL OF SURGICAL PATHOLOGY
Volume 29, Issue 7, Pages 808-814Publisher
SAGE PUBLICATIONS INC
DOI: 10.1177/10668969211003660
Keywords
ALK-rearrangement associated renal cell carcinoma; VCL-ALK renal cell carcinoma; renal cell carcinoma; anaplastic lymphoma kinase; ALK
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ALK rearrangement-associated renal cell carcinoma (ALK-rearranged RCC) is a new provisional entity in the 2016 World Health Organization classification of RCCs. This study reports 2 cases of ALK-rearranged RCC, one with a VCL-ALK fusion and the other with an EML4-ALK fusion, showing distinct features.
Anaplastic lymphoma kinase (ALK) rearrangement-associated renal cell carcinoma (ALK-rearranged RCC) is a new provisional entity that has been included in the 2016 World Health Organization classification of RCCs. We report 2 cases of ALK-rearranged RCC, 1 with a vinculin-ALK (VCL-ALK) fusion and the other with an EML4-ALK fusion. The VCL-ALK RCC occurred in a 14-year-old girl with sickle cell trait and showed features similar to previously described VCL-ALK RCCs, including medullary epicenter, solid architecture, and polygonal cells with cytoplasmic vacuoles. The EML4-ALK RCC occurred in a 14-year-old boy with no evidence of sickle cell trait and had multiple less-specific growth patterns comprising tubular, solid, and tubulopapillary architectures in the desmoplastic stroma, reminiscent of collecting duct carcinoma. Both tumors demonstrated cytoplasmic and membranous ALK protein expression by immunohistochemistry. Fluorescence in situ hybridization confirmed the ALK gene rearrangements in both cases. On review in the literature, we found that solid architecture and cytoplasmic vacuoles were present significantly more frequently in VCL-ALK RCC than in non-VCL-ALK RCC, supporting the distinctive nature of the former.
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