Follicular dendritic cell sarcoma of gastrointestinal tract with two emerging distinct subtypes: a case report and systemic review

Abstract

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.

An incidental discovery of a gastric follicular dendritic cell sarcoma: A rare case report and a literature review

“…FDCS typically manifests as larger masses that measure 7.8 cm on average. 10 The tumor, in our case, was large measuring 12 × 4.5 cm.…”

“…One or more FDC markers can, however, occasionally be lost in the gastrointestinal FDCS (24%, 8/33 cases). 10 Both morphological characteristics and immunohistochemistry are crucial in the diagnosis of FDCS since the histomorphology and immunohistochemistry of FDCS might be varied. 10 In the present case, CD21 and CD23 were expressed by tumor cells.…”

“… 10 Both morphological characteristics and immunohistochemistry are crucial in the diagnosis of FDCS since the histomorphology and immunohistochemistry of FDCS might be varied. 10 In the present case, CD21 and CD23 were expressed by tumor cells. Clusterin and podoplanin (D2–40) are other markers which also have high sensitivity for FDCS.…”

Fine needle aspiration cytology of follicular dendritic cell sarcoma of the stomach masquerading as gastrointestinal stromal tumor: A case report of a unique entity with emphasis on cytomorphology

“…1 There is no male or female predominance of cases. 9,11,12 FDCS typically presents as a slow growing, asymptomatic mass 2 and can occur in a variety of extranodal locations. 2,4,12,13 The pathogenesis of FDCS is incompletely understood, but it has been associated with Castleman disease.…”

“…Of the 21 cases of classic FDCS in the gastrointestinal tract published in the literature, 33% were erroneously diagnosed as GIST, carcinoma, or lymphoma, and another 24% only carried an initial impression of sarcoma. 9 The typical course for FDCS is indolent, often behaving as a low to intermediate grade malignant neoplasm, 11 notable for a local recurrence rate of 28%. An aggressive clinical course with metastasis to the lung, liver, or lymph nodes is also possible, 2 occurring in 27% of cases.…”