Follicular Dendritic Cell Sarcoma: An Underrecognized Disease Entity with Highly Heterogeneous Clinical, Pathologic, and Histogenetic Characteristics
Olga L Bohn, Jocelyn Maragulia, Oscar Lin, Andrew D Zelenetz, Julie Teruya-Feldstein, Maria E Arcila. Memorial Sloan-Kettering Cancer Center, New York, NY
Background: Follicular dendritic cell sarcomas (FDCS) are rare neoplasms arising from stromal-derived follicular dendritic cells of B-lymphoid follicles. Most published cases remain confined to single case reports or small-group case series and, consequently, the clinical and pathologic understanding of this disease remains limited and discohesive. In this study we describe the clinical and pathologic characteristics of patients diagnosed at our institution and evaluate similar characteristics in cases extracted from published literature.
Design: Twenty one cases of FDCS diagnosed at our institution were retrieved from the files and clinicopathologic characteristics were recorded including age, gender, site of involvement, size of the tumor, histologic characteristics, treatment, recurrence status, time of last follow up and survival data. Similar characteristics were extracted from 102 cases as recorded in literature.
Results: For the combined cohort of 123 patients, 66 were male and 57 female with a median age of 44 yrs (range 9-87). The Abdominal/pelvic region was the most commonly affected area in this cohort (55/123, 45%) followed by the head and neck region (44/123, 36%). Tumors varied widely in size, ranging from 1.2 to 21 cm (n=94) and 54(44%) were reported to have high grade histologic features at diagnosis. Follow-up data were available in 106 cases, covering a period of 1-324 mo (mean, 29 mo; median 20 mo). Of the informative cases, 47 (38%) had recurrence or metastasis, and 12 (10%) died of the disease. Size ≥ 5 cm and high grade histology were significantly associated with decreased disease free survival and overall survival (p<0.05). Treatment protocols varied widely among all cases but most included a combination of surgical resection plus radiation and/or chemotherapy.
Conclusions: Follicular dendritic cell sarcoma is a highly heterogeneous disease with variable pathologic findings and clinical outcomes. Size>5cm and high grade histology are associated with poor prognosis.
Tuesday, March 5, 2013 1:00 PM
Poster Session IV # 268, Tuesday Afternoon