title: Treatment with sunitinib for patients with progressive metastatic pheochromocytomas and sympathetic paragangliomas.
abstract: BACKGROUND
  Patients with progressive metastatic pheochromocytomas (PHEOs) or sympathetic paragangliomas (SPGLs) face a dismal prognosis. Current systemic therapies are limited.
  
  
  OBJECTIVE
  The primary end point was progression-free survival determined by RECIST 1.1 criteria or positron emission tomography with [(18)F]fluorodeoxyglucose/computed tomography ([(18)F]FDG-PET/CT), in the absence of measurable soft tissue targets. Secondary endpoints were tumor response according to RECIST criteria version 1.1 or FDG uptake, blood pressure control, and safety.
  
  
  METHODS
  We conducted a retrospective review of medical records of patients with metastatic PHEO/SPGL treated with sunitinib from December 2007 through December 2011. An intention-to-treat analysis was performed.
  
  
  METHODS
  Seventeen patients with progressive metastatic PHEO/SPGLs treated at the Institut Gustave-Roussy and MD Anderson Cancer Center.
  
  
  METHODS
  Patients treated with sunitinib.
  
  
  RESULTS
  According to RECIST 1.1, eight patients experienced clinical benefit; three experienced partial response, and five had stable disease, including four with predominant skeletal metastases that showed a 30% or greater reduction in glucose uptake on [(18)F]FDG-PET/CT. Of 14 patients who had hypertension, six became normotensive and two discontinued antihypertensives. One patient treated with sunitinib and rapamycin experienced a durable benefit beyond 36 months. The median overall survival from the time sunitinib was initiated was 26.7 months with a progression-free survival of 4.1 months (95% confidence interval = 1.4-11.0). Most patients who experienced a clinical benefit were carriers of SDHB mutations.
  
  
  CONCLUSIONS
  Sunitinib is associated with tumor size reduction, decreased [(18)F]FDG-PET/CT uptake, disease stabilization, and hypertension improvement in some patients with progressive metastatic PHEO/PGL. Prospective multi-institutional clinical trials are needed to determine the true benefits of sunitinib.
authors.affiliation: Department of Endocrine Neoplasia and Hormonal Disorders, Unit 1461, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, Texas 77030, USA.
authors.forename: Montserrat
authors.initials: M
authors.lastname: Ayala-Ramirez
authors.forename: Cecile N
authors.initials: CN
authors.lastname: Chougnet
authors.forename: Mouhammed Amir
authors.initials: MA
authors.lastname: Habra
authors.forename: J Lynn
authors.initials: JL
authors.lastname: Palmer
authors.forename: Sophie
authors.initials: S
authors.lastname: Leboulleux
authors.forename: Maria E
authors.initials: ME
authors.lastname: Cabanillas
authors.forename: Caroline
authors.initials: C
authors.lastname: Caramella
authors.forename: Pete
authors.initials: P
authors.lastname: Anderson
authors.forename: Abir
authors.initials: A
authors.lastname: Al Ghuzlan
authors.forename: Steven G
authors.initials: SG
authors.lastname: Waguespack
authors.forename: Desirée
authors.initials: D
authors.lastname: Deandreis
authors.forename: Eric
authors.initials: E
authors.lastname: Baudin
authors.forename: Camilo
authors.initials: C
authors.lastname: Jimenez
mesh_terms: D000293:Adolescent; D000310:Adrenal Gland Neoplasms / Q000175:diagnosis / Q000188:drug therapy* / Q000473:pathology; D000328:Adult; D000368:Aged; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D007211:Indoles / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D010235:Paraganglioma / Q000175:diagnosis / Q000188:drug therapy* / Q000556:secondary; D010673:Pheochromocytoma / Q000175:diagnosis / Q000188:drug therapy* / Q000556:secondary; D011758:Pyrroles / Q000627:therapeutic use*; D012189:Retrospective Studies; D000077210:Sunitinib; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents; D007211:Indoles; D011758:Pyrroles; D000077210:Sunitinib