Published in:
01-02-2019 | Case Report - Brain Tumors
Disseminated central nervous system hemangioblastoma in a patient with no clinical or genetic evidence of von Hippel-Lindau disease—a case report and literature review
Authors:
Simer J. Bains, Pitt F. Niehusmann, Torstein R. Meling, Cathrine Saxhaug, Mark Züchner, Petter Brandal
Published in:
Acta Neurochirurgica
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Issue 2/2019
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Abstract
Background
Hemangioblastomas (HB) are benign tumors of the central nervous system (CNS) that can appear sporadic or as part of von Hippel-Lindau (VHL) disease. It is often curable with surgical resection, but upon relapse, the disease exhibits a treatment-refractory course.
Case report
A patient treated for sporadic cerebellar HB relapsed 12 years post-surgery. She developed disseminated disease throughout the CNS, including leptomeningeal manifestations. Repeat surgery and craniospinal radiation therapy were unsuccessful.
Conclusion
This case is in line with previous publications on disseminated non-VHL HB. Available treatment options are inefficient, emphasizing the need for improved understanding of HB biology to identify therapeutic targets.