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Published on: October 14, 2021
Follicular dendritic cell sarcoma of gastrointestinal tract with two emerging distinct subtypes: a case report and
Hongxing Gui1, Jigisha Chaudhari2, Rifat Mannan2
1Department of Pathology and Laboratory Medicine, Pennsylvania Hospital of the University of Pennsylvania Health System, 801 Spruce Street, 10th Floor Spruce building, Philadelphia, PA, 19107, USA. Hongxing.gui@pennmedicine.upenn.edu.
Background:
Follicular dendritic cell sarcoma (FDCS) is a rare neoplasm of mesenchymal origin. FDCS of gastrointestinal tract (GI) are exceedingly uncommon.
Case Presentation:
We report the first case of classic type FDCS in a 34-year-old male with Birt-Hogg-Dubé syndrome, which presented as a mass at the ileo-cecal junction. He received no further treatment after resection and remained disease free for 3.5 years. We further analyze and review the clinical and pathologic findings of 33 cases of GI tract FDCS reported in the literature.
Conclusions:
There are two distinct subtypes of FDCS in the GI tract: the classic type occurs in relatively younger patients (mean = 45.3 years) without Epstein-Barr virus (EBV) association, and behaves more aggressively; the inflammatory subtype presents as colonic polypoid tumor in older patients (mean = 60.7 years) and is EBV positive. The clinical outcome in the latter group appears favorable although mortality rate is not necessarily low.
