Renal hemangioblastoma and renal cell carcinoma with fibromyomatous stroma and hemangioblastoma-like areas belong to the spectrum of one entity
Trpkov K, Salut N, Ribera-Cortada I, Xipell ET, Puigsureda IT, Yilmaz A, Husain AR, Nohr E, Box A, Siadat F, Baranova K, Saleeb RM, Stöhr R, Hartmann A, Agaimy A (2025)
Publication Type: Journal article
Publication year: 2025
Journal
DOI: 10.1007/s00428-025-04325-w
Abstract
Several reports have documented renal cell carcinomas (RCC) with fibromyomatous stroma and with areas of hemangioblastoma (RCC FMS-HB). The hemangioblastoma component in RCC FMS-HB tumors was similar to the morphology of primary renal hemangioblastoma, suggesting that RCC FMS-HB and renal hemangioblastoma share common features. Seven study cases were collected, comprised of three RCC FMS-HB (two sporadic and one in a tuberous sclerosis patient) and four pure renal hemangioblastomas. A molecular analysis was successful in 6/7 cases. There were four males and three females, with an age range of 27 to 74 years, and a mean age of 51 years. The tumors measured from 2.6 to 5.5 cm (median 3.5 cm), and all were organ-confined, stage pT1. Median size for RCC FMS-HB was 2.8 cm, and for renal hemangioblastoma, it was 3.5 cm. The hemangioblastoma component in all RCC FMS-HBs and in all renal hemangioblastomas was positive for inhibin-α and S100 but was negative for CK7. In contrast, the clear-cell epithelial component in RCC FMS-HB was diffusely reactive for CK7 and was negative for inhibin-α and S100. Both components were reactive for CAIX, vimentin, and GPNMB. TSC/MTOR pathway alterations were found in 6/6 successfully evaluated cases, including 3/3 RCC FMS-HB and 3/3 renal hemangioblastomas, with mutations in TSC1 (2/6), TSC2 (2/6), and MTOR (2/6). The shared morphologic, immunohistochemical, and molecular features of RCC FMS-HB and renal hemangioblastoma indicate that they belong to a common biological spectrum, and that renal HB is a distinct entity from its VHL-altered central nervous system counterpart.
Authors with CRIS profile
Robert Stöhr
Medizinische Fakultät
Arndt Hartmann
Lehrstuhl für Allgemeine Pathologie und Pathologische Anatomie
Abbas Agaimy
Institute of Pathology
Involved external institutions
University of Calgary
Canada (CA)
Universitat de Barcelona (UB) / University of Barcelona
Spain (ES)
London Health Sciences Centre
Canada (CA)
University of Toronto
Canada (CA)
Canada (CA)
Universitat de Barcelona (UB) / University of Barcelona
Spain (ES)
London Health Sciences Centre
Canada (CA)
University of Toronto
Canada (CA)
How to cite
APA:
Trpkov, K., Salut, N., Ribera-Cortada, I., Xipell, E.T., Puigsureda, I.T., Yilmaz, A.,... Agaimy, A. (2025). Renal hemangioblastoma and renal cell carcinoma with fibromyomatous stroma and hemangioblastoma-like areas belong to the spectrum of one entity. Virchows Archiv. https://doi.org/10.1007/s00428-025-04325-w
MLA:
Trpkov, Kiril, et al. "Renal hemangioblastoma and renal cell carcinoma with fibromyomatous stroma and hemangioblastoma-like areas belong to the spectrum of one entity." Virchows Archiv (2025).
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