title: Pediatric extraadrenal paraganglioma.
abstract: BACKGROUND
  Pediatric paraganglioma is rare and extraadrenal paraganglioma has not been characterized in children.
  
  
  METHODS
  The authors reviewed the medical records and pathology samples of children with extraadrenal paraganglioma treated at our institution between December 1978 and September 2000. Clinical presentation, treatment, and outcome were evaluated.
  
  
  RESULTS
  Eight patients (median age, 11.4 years) were identified, 4 were boys and none had a family history of paraganglioma or associated syndromes. Primary tumors arose in the retroperitoneum (n = 3), carotid body (n = 2), jugulotympanic ganglion (n = 1), cervical-paraspinal region (n = 1), and lung (n = 1). Extraadrenal paraganglioma had not been suspected at presentation in any patient. Of 5 patients who underwent gross total resection at the time of diagnosis, 4 remain disease free, 1 had microscopic residual tumor and died of disease. Three patients had initially unresectable disease, 2 are disease free after neoadjuvant therapy and delayed surgery, and 1 has persistent disease after tumor embolization and radiotherapy.
  
  
  CONCLUSIONS
  Pediatric extraadrenal paraganglioma occurs most commonly in the retroperitoneum and head and neck, and the diagnosis usually is not suspected at the time of presentation. Surgery is the mainstay of treatment, and outcome is good after gross total resection. Neoadjuvant therapy can facilitate complete resection of initially unresectable tumors.
authors.affiliation: Department of Hematology-Oncology, St. Jude Children's Research Hospital, Memphis, TN 38105-2794, USA.
authors.forename: Tanya M
authors.initials: TM
authors.lastname: Tekautz
authors.forename: Charles B
authors.initials: CB
authors.lastname: Pratt
authors.forename: Jesse J
authors.initials: JJ
authors.lastname: Jenkins
authors.forename: Sheri L
authors.initials: SL
authors.lastname: Spunt
mesh_terms: D000293:Adolescent; D002648:Child; D005260:Female; D006801:Humans; D008297:Male; D010236:Paraganglioma, Extra-Adrenal / Q000175:diagnosis / Q000628:therapy; D016896:Treatment Outcome