Outstanding Response to Sunitinib in a Patient with Unresectable Retroperitoneal Paraganglioma

#1729

Introduction: Pheochromocitomas and Paragangliomas (PCs/PGLs) are rare neuroendocrine tumors arising from the adrenal medulla and extra-adrenal ganglia. Around 30-40% of these neoplasms are genetically determined. PCs/PGLs arising from the sympathetic chain can synthesize bioactive amines leading to typical syndromes, in contrast to parasympathetic PGLs that are mainly non-secretory.

Aim(s): To describe a case of locally advanced paragangliomas treated with Sunitinib and radical surgery

Materials and methods: A 51-year-old woman referred to our center in July 2014 for abdominal pain and weight loss. A CT scan showed a large lesion in the upper left section of abdomen of 18 cm. The mass displaced vascular structures and embraced pancreas. A CT-guided biopsy documented the presence of retroperitoneal PGL. SUTNET, a single arm, monocentric, phase II study, designed to investigate safety and activity of Sunitinib in advanced and/or unresectable PCs/PGLs, was ongoing in our institution. Patient was enrolled in December 2014. Sunitinib was administered at the standard dose of 50 mg/day (4-weeks-on/2-weeks-off). Due to hematological toxicity, after 16 cycles, dose was reduced to 37.5 mg.

Conference: 14th Annual ENETSConcerence (2017)

Presenting Author: Pusceddu S

Authors: Prinzi N, Femia D, Lo Russo G, Vernieri C, Concas L,

Keywords: Paragangliomas, Sunitinib,