Sorafenib in the treatment of metastatic paraganglioma: A case report.

Abstract

e13523 Background: Paragangliomas (extra-adrenal pheochromocytomas) are rare chromaffin cell tumors that can often be cured by resection. Excess release of catecholamines is characteristic for paragangliomas. Paragangliomas are mostly benign if diagnosed and treated early. Overall 0–36% of paraganglioma patients develop metastatic disease, depending on the type of tumor. Methods: We present a case of a 43-year-old woman who admitted to our department with a history of episodic headaches, diaphoresis and weakness. Hypertension and tachycardia were diagnosed. She had elevated plasma catecholamine levels and a right paraaortic mass (9x9.5cm) was visualized on CT. The mass was excised and the diagnosis of paraganglioma was confirmed. In postoperative follow-up the patient's blood pressure and catecholamine levels were normalized. After twenty months follow up, local recurrence and metastases were detected in thorax, abdomen, and skeletal system. Plasma catecholamine levels were high. Results: Chemotherapy with CVD (adriamycine (7mg/m2), dacarbazine (400mg/m2), cyclophosphamide (500mg/m2), vincristine (1.4mg/m2) on day 1 and 2, repeated every 21 days; were administered and no improvement was observed in PET-CT following a treatment of three cycles. Sorafenib (800mg/m2/day) was applied for three-months. Finally the patient’s plasma catecholamine levels and metastatic lesions regressed. Conclusions: Treatment options of metastatic paragangliomas (MP) are removal by excision or ablation, chemotherapy, or targeted pharmaceuticals. Benefits of CVD chemotherapy for MP appears to be short-term and do not include an increase in patient survival. As targeted therapy, treatment of MP with everolimus or imatinib were attempted with no significant benefit, and thus are not recommended. However partial response was reported with Sunitinib some cases. We used sorafenib as another multiple TKI and detected complete response. So far, sorafenib has not been applied in the treatment of MP in literature. Sorafenib can be preferred in the treatment of MP. Therefore, more comprehensive clinical trials are needed.