title: Multidisciplinary management of locally advanced and widely metastatic paraganglioma in a patient with life-threatening compressive symptoms.
abstract: BACKGROUND
  Patients presenting with locally advanced, metastatic paraganglioma with life-threatening compressive symptoms of critical anatomic structure pose a significant management challenge.
  
  
  METHODS
  We present a case of a 15-year-old patient with enlarging right neck mass causing dysphagia and respiratory compromise from near complete obstruction of the oropharynx.
  
  
  RESULTS
  Evaluation of the patient's family history led to the identification of a mutation in the succinate dehydrogenase subunit B (SDSD) gene (G725A). Laboratory and imaging workup revealed an 8.8 × 6.6 × 4.1 cm metabolically and biochemically active right neck mass, a tumor in the left para-aortic region, and multiple bony lesions consistent with widely metastatic disease. Multidisciplinary management included preoperative clinical optimization, coil embolization, and palliative resection of the neck mass.
  
  
  CONCLUSIONS
  Although the currently available treatment options for patients with advanced metastatic paraganglioma render no survival benefit, a multidisciplinary management approach aimed at relief of tumor-related symptoms and catecholamine excess should be undertaken.
authors.affiliation: Endocrine Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, Maryland.
authors.forename: Vladimir
authors.initials: V
authors.lastname: Neychev
authors.affiliation: Thoracic and Gastrointestinal Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, Maryland.
authors.forename: David
authors.initials: D
authors.lastname: Straughan
authors.affiliation: Section on Endocrinology and Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland.
authors.forename: Karel
authors.initials: K
authors.lastname: Pacak
authors.affiliation: Endocrine Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, Maryland.
authors.forename: Electron
authors.initials: E
authors.lastname: Kebebew
mesh_terms: D000293:Adolescent; D000402:Airway Obstruction / Q000209:etiology*; D014408:Biomarkers, Tumor / Q000235:genetics; D002345:Carotid Body Tumor / Q000556:secondary / Q000601:surgery; D003680:Deglutition Disorders / Q000209:etiology*; D006801:Humans; D033183:Interdisciplinary Communication; D008297:Male; D009154:Mutation; D009362:Neoplasm Metastasis; D010236:Paraganglioma, Extra-Adrenal / Q000175:diagnosis / Q000235:genetics / Q000556:secondary* / Q000601:surgery*; D013385:Succinate Dehydrogenase / Q000235:genetics; D016896:Treatment Outcome
chemical_list: D014408:Biomarkers, Tumor; C108095:SDHB protein, human; D013385:Succinate Dehydrogenase
keywords: compressive symptoms; management approach; metastatic disease; paraganglioma; succinate dehydrogenase gene (SDHB)