Paragangliomas are rare, often vascular, endocrine tumors that are sometimes malignant. Antiangiogenic agents may play a role in treating advanced disease. We report a case of a 49-year-old woman with metastatic and rapidly progressive secretory paraganglioma (with germ line SDHB p.V140F–c.418G>T mutation) who responded rapidly and durably to the VEGFR- and multi-targeted kinase inhibitor, lenvatinib, despite progression through prior cytotoxic and kinase inhibitor therapy. She incurred dramatic response within 48 h of lenvatinib initiation, but developed parallel transient mild tumor lysis syndrome and, later, localized radiation ‘recall’ pneumonitis. Follow-up assessments demonstrated a confirmed response evaluation criteria in solid tumors response as well as symptomatic and biochemical improvement. Lenvatinib may represent a promising therapeutic for further development in treating metastatic paraganglioma.
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