title: Necessity for lifelong follow-up of patients with familial paraganglioma syndrome: A case report.
abstract: BACKGROUND
  Patients with established familial paraganglioma (PGL) syndrome may have multiple metachronous lesions. This article illustrates, via imaging and findings, the need for lifetime follow-up of patients with familial PGL syndromes.
  
  
  METHODS
  Patients' medical charts and radiological images were reviewed in a retrospective analysis.
  
  
  RESULTS
  Over the course of 18 years, this patient developed 2 simultaneous carotid PGLs, a cardiac PGL, and a biochemically active interaortocaval PGL.
  
  
  CONCLUSIONS
  PGLs do not necessarily occur simultaneously in patients with familial PGL syndrome. Lifelong observation is needed to detect these lesions before they become large and symptomatic. Lack of biochemical activity is not a predictor of future lesions being inactive. Cardiac PGLs are rare and require resection.
authors.affiliation: Department of Otolaryngology - Head and Neck Surgery, New York University Langone Medical Center, Clinical Cancer Center, New York, New York.
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Persky
authors.affiliation: Department of Vascular Surgery, New York University Langone Medical Center, Clinical Cancer Center, New York, New York.
authors.forename: Mark
authors.initials: M
authors.lastname: Adelman
authors.affiliation: Department of Cardiovascular Surgery, New York University Langone Medical Center, Clinical Cancer Center, New York, New York.
authors.forename: Elias
authors.initials: E
authors.lastname: Zias
authors.affiliation: Department of Otolaryngology - Head and Neck Surgery, New York University Langone Medical Center, Clinical Cancer Center, New York, New York.
authors.forename: David
authors.initials: D
authors.lastname: Myssiorek
mesh_terms: D000328:Adult; D002345:Carotid Body Tumor / Q000175:diagnosis / Q000235:genetics* / Q000601:surgery*; D005500:Follow-Up Studies; D006338:Heart Neoplasms / Q000235:genetics / Q000601:surgery; D006801:Humans; D008297:Male; D010236:Paraganglioma, Extra-Adrenal / Q000235:genetics / Q000601:surgery; D010375:Pedigree; D013577:Syndrome; D016896:Treatment Outcome
keywords: biochemically active; cardiac paraganglioma; familial paraganglioma; follow-up; multifocality; paraganglioma syndrome 1 (PGL 1); succinate dehydrogenase subunit D (SDHD)