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Periampullary Gangliocytic Paraganglioma

  • Ching-Shu Chiang
  • Bor-Uei Shyr
  • Shih-Chin Chen
  • Yi-Ming Shyr
  • Shin-E WangEmail author
Original Article
  • 27 Downloads

Abstract

Background

Gangliocytic paraganglioma (GP) is rare and difficult to be differentiated from other periampullary neoplasms. The clinical characteristics and optimal treatment of periampullary GPs have not been clarified.

Methods

The data pool for the analysis comprised of cases of periampullary GP encountered in our institution and sporadic cases reported in the English literature.

Results

A total of 117 cases with periampullary GP were studied, including 2 from our institute, and among them, duodenal GP was the most common (53.0%). GP size ranged from 0.7 to 19.0 cm, with a median of 2.2 cm. The most common presenting symptom for overall periampullary GPs was epigastric pain in 49.5% cases, followed by gastrointestinal bleeding in 35.4% cases. Most (84.1%) of the periampullary GPs were benign, whereas 15.9% were malignant. Metastasis was noted in 26.3% of periampullary GPs, with 14.5% showing lymph node metastasis and 1.8% showing liver metastasis. Of the periampullary GP cases included, 30.1% were treated with pancreaticoduodenectomy, 40.6% with local excision, and 17.7% with endoscopic resection.

Conclusions

Periampullary GP should be considered as a tumor with malignant potential. Endoscopic resection is the treatment of choice for most of the duodenal GPs, whereas pancreaticoduodenectomy is recommended for GPs with possible malignancy, such as large size, with submucosal invasion, or pancreatic GP.

Keywords

Gangliocytic paraganglioma Gastrointestinal bleeding Periampullary 

Notes

Financial Support

This work was financially supported by grants from Taipei Veterans General Hospital (V106C-028, VTA106-V1-1-1) and the Ministry of Science and Technology (MOST 106-2314-B-075 -048 -MY2) and the Ministry of Health and Welfare (MOHW107-TDU-B-212-114026A).

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Copyright information

© The Society for Surgery of the Alimentary Tract 2018

Authors and Affiliations

  • Ching-Shu Chiang
    • 1
  • Bor-Uei Shyr
    • 1
  • Shih-Chin Chen
    • 1
  • Yi-Ming Shyr
    • 1
  • Shin-E Wang
    • 1
    Email author
  1. 1.Division of General Surgery, Department of Surgery, Taipei Veterans General HospitalNational Yang Ming UniversityTaipeiTaiwan

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