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Journal of Gastrointestinal Surgery

, Volume 11, Issue 10, pp 1351–1354 | Cite as

Gangliocytic Paraganglioma: Case Report and Review of the Literature

  • Agnes Witkiewicz
  • Avi Galler
  • Charles J. Yeo
  • Samuel D. Gross
Article

Abstract

Gangliocytic paraganglioma is a rare tumor, which occurs nearly exclusively in the second portion of the duodenum. Generally, this tumor has a benign clinical course, although rarely, it may recur or metastasize to regional lymph nodes. Only one case with distant metastasis has been reported. We present a case of duodenal gangliocytic paraganglioma treated first by local resection followed by pylorus-preserving pancreaticoduodenectomy. Examination of the first specimen revealed focal nuclear pleomorphism and mitotic activity, in addition to the presence of three characteristic histologic components: epithelioid, ganglion, and spindle cell. In the subsequent pancreaticoduodenectomy specimen, there was no residual tumor identified in the periampullary area, but metastatic gangliocytic paraganglioma was present in two of seven lymph nodes. This case report confirms the malignant potential of this tumor. We review the published literature on gangliocytic paragangliomas pursuing a malignant course. We conclude that surgical therapy of these neoplasms should not be limited to local resection, as disease recurrence, lymph node involvement, and rarely distant metastasis may occur.

Keywords

Gangliocytic paraganglioma Epithelioid cells Ganglion cells Spindle cell 

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Copyright information

© The Society for Surgery of the Alimentary Tract 2007

Authors and Affiliations

  • Agnes Witkiewicz
    • 1
  • Avi Galler
    • 2
  • Charles J. Yeo
    • 2
    • 3
  • Samuel D. Gross
    • 2
  1. 1.Department of PathologyThomas Jefferson UniversityPhiladelphiaUSA
  2. 2.Department of SurgeryThomas Jefferson UniversityPhiladelphiaUSA
  3. 3.Jefferson Medical CollegeThomas Jefferson UniversityPhiladelphiaUSA

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