A Detailed Clinicopathologic Review of Castleman Disease Demonstrates That Atypical/Dysplastic Follicular Dendritic Cells Positive for SOX11, S-100, CXCL13, or D2-40 Are a Frequent Finding in Hyaline Vascular and Plasma Cell Variants
Robert S Ohgami, Matthew Alcasid, Roger A Warnke, Daniel A Arber. Stanford, Stanford, CA
Background: Castleman disease is a distinct lymphoproliferative disorder with alterations in lymph node follicular architecture. Though Castleman disease has been relatively well studied, a comprehensive clinicopathologic review in the context of current knowledge is lacking.
Design: 36 cases of Castleman disease with available histologic material between 2004-2011 were retrospectively reviewed and major clinical and pathologic features analyzed.
Results: We not only provide a comprehensive review of the clinical and histomorphologic features of these cases of Castleman disease, but also importantly demonstrate that atypical/dysplastic follicular dendritic cells are a relatively common occurrence in Castleman disease, present in 9 of 36 cases (24%). Interestingly, atypical/dysplastic follicular dendritic cells (aFDC) were seen in both hyaline vascular variant (7 of 23 cases) and plasma cell variant (2 of 13). One case of Castleman disease, intermediate between hyaline vascular variant and plasma cell variant, showed no aFDC. No significant association of aFDC with age, sex or HHV8 status was seen. In 7 of 9 cases, atypical/dysplastic follicular dendritic cells were positive for SOX11, S-100, CXCL13, or D2-40 with a subset of cells showing frequent loss of CD21 (Figure 1). No progression to follicular dendritic cell tumors was seen in the 36 cases reviewed here.
Conclusions: Atypical/dysplastic follicular dendritic cells positive for SOX11, S-100, CXCL13, or D2-40 are relatively common in Castleman disease, both hyaline vascular and plasma cell variants. The presence of scattered aFDC should not be interpreted as progression to a follicular dendritic cell tumor.
|Dysplastic/atypical Follicular Dendritic cells|
|Yes (n=9)||No (n=27)|
|Age, median (range)||35 (17-63)||45 (17-75)|