CAT-VHL Exploring the Role of Carbonic Anhydrase IX as Diagnostic and Theranostic Target in Von-Hippel Lindau Disease

Inclusion Criteria: * Voluntarily given informed consent * Age ≥18 years old * Performance Status ECOG/WHO score 0-2 * For females of reproductive potential, negative pregnancy test and use of highly effective contraception for 30 days following IMP administration * For males of reproductive potential, use of highly effective contraception for 30 days following IMP administration. And, for the primary cohort: * Diagnosis of VHL disease requiring surveillance following confirmation of pathogenic variant at genetic test Alternatively, for the secondary cohort: \- Clinical and/or pathological diagnosis of hemangioblastoma, pheochromocytoma, pancreatic neuroendocrine tumor or clear cell renal cell carcinoma requiring surgery. Exclusion Criteria: * Performance Status ECOG/WHO score \>2 * Women who are pregnant or breastfeeding or are planning pregnancy during the study * Men who are planning fatherhood during the study * Exposure to any murine or chimeric antibodies within 5 years prior to the planned IMP administration * Exposure to any experimental diagnostic or therapeutic drug within 30 days from the planned IMP administration * Surgery, biopsy, ablative procedure, radiotherapy or any other local treatment for any primary tumor within 4 weeks prior to the planned IMP administration * Exposure to any systemic agent within 4 weeks prior to the planned IMP administration or in case of continuing adverse effects with grade \>1 from such therapy * Current exposure to systemi…