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Treatment with sunitinib for patients with progressive metastatic pheochromocytomas and sympathetic paragangliomas

Montserrat Ayala-Ramirez1, Cecile N Chougnet, Mouhammed Amir Habra

  • 1Department of Endocrine Neoplasia and Hormonal Disorders, Unit 1461, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, Texas 77030, USA.

The Journal of Clinical Endocrinology and Metabolism
|September 12, 2012
PubMed
Abstract

Insights

Sunitinib showed clinical benefit in some patients with metastatic pheochromocytomas (PHEOs) or paragangliomas (SPGLs), including tumor shrinkage and improved blood pressure. Further trials are needed to confirm these findings for progressive metastatic neuroendocrine tumors.

Area of Science:

  • Oncology
  • Endocrinology
  • Radiology

Background:

  • Metastatic pheochromocytomas (PHEOs) and sympathetic paragangliomas (SPGLs) have a poor prognosis with limited systemic therapy options.
  • Current treatments for advanced PHEOs/SPGLs are insufficient, highlighting the need for novel therapeutic strategies.

Purpose of the Study:

  • To evaluate the efficacy and safety of sunitinib in patients with progressive metastatic PHEO/SPGL.
  • To assess progression-free survival (PFS) and objective tumor response as primary and secondary endpoints.

Main Methods:

  • Retrospective review of 17 patients with metastatic PHEO/SPGL treated with sunitinib between December 2007 and December 2011.
  • Evaluation of progression-free survival using RECIST 1.1 criteria and [(18)F]FDG-PET/CT, alongside assessment of tumor response, blood pressure control, and safety.

Main Results:

  • Eight patients (47%) achieved clinical benefit, including partial response or stable disease, with some showing significant reduction in [(18)F]FDG uptake on PET/CT.
  • Hypertension improved in six patients, and median overall survival was 26.7 months with a PFS of 4.1 months.
  • Patients with SDHB mutations were more likely to experience clinical benefit.

Conclusions:

  • Sunitinib demonstrates potential in reducing tumor size, decreasing [(18)F]FDG uptake, stabilizing disease, and improving hypertension in select patients with metastatic PHEO/PGL.
  • Prospective, multi-institutional clinical trials are essential to validate the therapeutic benefits of sunitinib in this patient population.

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