Challenges in Von Hippel–Lindau’s disease: PRRT in patients on hemodialysis

Von Hippel–Lindau’s disease (VHL) is a hereditary tumor syndrome characterized by its prototype lesions, hemangioblastomas, and renal cell carcinomas. Treatment for renal cell carcinomas can ultimately result in long-term dialysis. Pancreatic neuroendocrine tumors (pNET) can also occur in the course of the disease. Currently, peptide receptor radionuclide therapy (PRRT) is the standard treatment for progressive neuroendocrine tumors. However, little is known about treatment with PRRT in patients on dialysis, an infrequent presentation in patients with VHL. We present a 72-year-old man with VHL... Mehr ...

Verfasser: Ayub, N.
Braat, A. J.A.T.
Timmers, H. J.L.M.
Lam, M. G.E.H.
van Leeuwaarde, R. S.
Dokumenttyp: Artikel
Erscheinungsdatum: 2022
Schlagwörter: Adolescent/young adult / Male / White / Netherlands / Kidney / Endocrine-related cancer / Nephrology / Oncology / Radiology/Rheumatology / Insight into disease pathogenesis or mechanism of therapy / March / 2022 / Internal Medicine / Endocrinology / Diabetes and Metabolism / Journal Article
Sprache: Englisch
Permalink: https://search.fid-benelux.de/Record/base-28790899
Datenquelle: BASE; Originalkatalog
Powered By: BASE
Link(s) : https://dspace.library.uu.nl/handle/1874/446053

Von Hippel–Lindau’s disease (VHL) is a hereditary tumor syndrome characterized by its prototype lesions, hemangioblastomas, and renal cell carcinomas. Treatment for renal cell carcinomas can ultimately result in long-term dialysis. Pancreatic neuroendocrine tumors (pNET) can also occur in the course of the disease. Currently, peptide receptor radionuclide therapy (PRRT) is the standard treatment for progressive neuroendocrine tumors. However, little is known about treatment with PRRT in patients on dialysis, an infrequent presentation in patients with VHL. We present a 72-year-old man with VHL on hemodialysis and a progressive pNET. He received four cycles of PRRT with a reduced dose. Only mild thrombopenia was seen during treatments. The patient died 9 months after the last PRRT because of acute bleeding in a hemangioblastoma. Hemodialysis is not a limiting factor for PRRT treatment and it should be considered as it seems a safe short-term treatment option for this specific group.