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One Tracer, Whole Syndrome: CAIX-Targeted PET Unmasks the Multisystem Tumor Landscape of Von Hippel-Lindau Disease.

Created on 24 Sep 2026

Authors

Esra Arslan, Halil Kömek, Canan Can, Elife Akgün, Okan Türk, Uğur Yücetaş, Tevfik Fikret Cermik, Ali Rıza Kural

Published in

Clinical nuclear medicine. Sep 24, 2026. Epub Sep 24, 2026.

Abstract

Von Hippel-Lindau (VHL) is hereditary syndrome characterized with benign and malignant neoplasms resulting from dysregulation of hypoxia-sensitive pathway. Fifty-year-old man with VHL underwent multimodal imaging for remaining CNS disease. ⁶⁸Ga-DOTATATE PET/CT showed single cerebellar lesion, while ¹⁸F-FDG PET/CT remained negative despite active disease; [⁶⁸Ga]Ga-DPI-4452 CAIX-targeted PET/CT revealed multisystem tumor burden; cerebellar and spinal hemangioblastomas, recurrent and contralateral ccRCC lesions and epididymal involvement. This case demonstrates the ability of CAIX-targeted imaging to visualize the pervasive hypoxia-induced phenotype underlying VHL-associated neoplasms, detect biologically active but metabolically latent disease, and may represent promising molecular imaging approach for phenotype-focused evaluation of inherited tumor syndromes.

PMID:
42779534
Bibliographic data and abstract were imported from PubMed on 24 Sep 2026.

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