Ontology highlight
ABSTRACT: Background
Paragangliomas (PGLs) are neuroendocrine neoplasms arising from chromaffin cells of sympathetic or parasympathetic paraganglia. Systemic therapies have been used only in metastatic PGLs. Antiangiogenic agents, such as sunitinib, could be a viable therapeutic choice in the subgroup of patients with SDH-positive PGLs. We describe the case of a man with Familial Paraganglioma Syndrome type 1 (FPGL) related to a novel mutation in SDHD gene treated with sunitinib. Furthermore, we performed a systematic review of the literature aimed to address the following question: is sunitinib treatment effective in patients with advanced/progressive/metastatic PGL?Methods
We performed a data search using MEDLINE, Cochrane Library, and Scopus between April 2019 and Se
SUBMITTER: Sesti F
PROVIDER: S-EPMC8247584 | biostudies-literature | 2021
REPOSITORIES: biostudies-literature