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Multiple functioning paraganglioma associated with polycythemia.

T Imai1, H Funahashi, Y Sato

  • 1Department of Surgery II, Nagoya University School of Medicine, Japan.

Journal of Surgical Oncology
|December 1, 1988
PubMed
Summary
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This case report details a rare instance of multiple retroperitoneal paragangliomas in a young girl, complicated by polycythemia. Surgical removal led to symptom resolution, though recurrence is possible.

Area of Science:

  • Endocrinology
  • Oncology
  • Surgical Case Reports

Background:

  • Retroperitoneal paragangliomas are rare neuroendocrine tumors.
  • Polycythemia, an elevated red blood cell count, can be associated with certain tumors.
  • Multiple tumors present a unique surgical and diagnostic challenge.

Observation:

  • A 13-year-old girl presented with headache, palpitations, and faintness.
  • Twenty-one retroperitoneal paragangliomas, exceeding 1 cm, were identified.
  • Polycythemia was diagnosed preoperatively and persisted post-surgery despite normal erythropoietin levels.

Findings:

  • Surgical excision of all identified paragangliomas was performed in two stages (1979, 1983).
  • Post-operative symptoms resolved, and bilateral adrenal glands appeared normal.

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  • The nature of the tumors (benign vs. malignant) remains indeterminate, with a likelihood of recurrence after 8 symptom-free years.

    • Implications:

    • This case highlights the extreme rarity of multiple functioning paragangliomas associated with polycythemia.
    • The persistent polycythemia despite normal erythropoietin levels warrants further investigation.
    • The management of extensive retroperitoneal paragangliomas underscores the need for long-term surveillance due to recurrence risk.