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Pediatric extraadrenal paraganglioma.

Tanya M Tekautz1, Charles B Pratt, Jesse J Jenkins

  • 1Department of Hematology-Oncology, St. Jude Children's Research Hospital, Memphis, TN 38105-2794, USA.

Journal of Pediatric Surgery
|October 3, 2003
PubMed
Summary
This summary is machine-generated.

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Pediatric extraadrenal paraganglioma is rare, often presenting in the retroperitoneum and head/neck. Complete surgical resection offers the best outcome for these rare pediatric tumors.

Area of Science:

  • Pediatric Oncology
  • Surgical Pathology
  • Rare Tumors

Background:

  • Pediatric paraganglioma is a rare condition.
  • Extraadrenal paraganglioma in children is not well-characterized.

Purpose of the Study:

  • To characterize pediatric extraadrenal paraganglioma.
  • To evaluate clinical presentation, treatment, and outcomes.

Main Methods:

  • Retrospective review of medical records and pathology samples.
  • Study period: December 1978 to September 2000.
  • Eight pediatric patients with extraadrenal paraganglioma were identified.

Main Results:

  • Tumors most commonly arose in the retroperitoneum and head/neck.

Related Experiment Videos

  • Diagnosis was not suspected at presentation in any patient.
  • Gross total resection led to disease-free survival in 4 of 5 patients; neoadjuvant therapy aided resection of unresectable tumors.

    • Conclusions:

    • Pediatric extraadrenal paraganglioma typically presents in the retroperitoneum and head/neck.
    • Diagnosis is often delayed.
    • Surgical resection is the primary treatment, with favorable outcomes after complete removal.