Affordable Access

Access to the full text

Clinical response to pazopanib in a patient with endolymphatic sac tumor not associated with von Hippel-Lindau syndrome

Authors
  • Nelson, Thomas1
  • Hu, Jethro2
  • Bannykh, Serguei3
  • Fan, Xuemo3
  • Rudnick, Jeremy2
  • Vail, Eric4
  • 1 Department of Neurology, Cedars-Sinai Medical Center, 127 S San Vicente Blvd, Suite A6600, Los Angeles, CA 90048, USA
  • 2 90048, USA
  • 3 Division of Neuropathology, Department of Pathology, Cedars-Sinai Medical Center, 8700 Beverly Blvd, Los Angeles, CA 90048, USA
  • 4 Division of Molecular Pathology, Department of Pathology, Cedars-Sinai Medical Center, 8700 Beverly Blvd, Los Angeles, CA 90048, USA
Type
Published Article
Journal
CNS Oncology
Publisher
Future Medicine
Publication Date
Mar 06, 2020
Volume
9
Issue
1
Identifiers
DOI: 10.2217/cns-2019-0019
PMID: 32141326
PMCID: PMC7163403
Source
PubMed Central
Keywords
License
Green

Abstract

Endolymphatic sac tumors (ELSTs) are rare, locally invasive, vascular tumors of the temporal bone. These lesions are associated with von Hippel-Lindau syndrome but may arise sporadically. Early surgical intervention is recommended to prevent permanent neurologic deficits; however, many ELSTs are unresectable or are subtotally resected due to neurovascular compromise. Chemotherapeutic salvage therapy in trials of neoplasms of associated syndromes has targeted angiogenesis with variable response. We present the case of a sporadic ELST, previously minimally responsive to bevacizumab, treated with pazopanib, a multi-kinase inhibitor and antiangiogenic, with good response. Cases such as our patient may demonstrate the utility of novel antiangiogenics in the treatment of these rare neoplasms, particularly when the tumor is unresectable or necessitates subtotal resection.

Report this publication

Statistics

Seen <100 times