Primary Follicular Dendritic Cell Sarcoma of Urinary Bladder

Follicular dendritic cell sarcoma is a rare malignant mesenchymal neoplasm composed of follicular dendritic cells. A subset of cases can be found at extranodal sites. Only 2 follicular dendritic cell sarcomas were previously reported in the urinary bladder. We report a follicular dendritic cell sarcoma that involved the urinary bladder of an 81-year-old woman, who presented with painless gross hematuria. The neoplasm was composed of large epithelioid cells with atypical nuclei, admixed with inflammatory cells. On immunohistochemistry, the neoplastic cells were reactive only for CD21, CD35, and vimentin. The tumor was considered unresectable due to its large size and proximity to the iliac vessels and the patient was treated with bortezomib, cyclophosphamide, and dexamethasone. Although a full course of chemotherapy was not completed due to adverse side effects, the tumor was reduced in size. A subsequent involved-field radiotherapy was performed. Three years postdiagnosis and 2 years after the radiotherapy treatment, a recurrent tumor was identified on imaging in the left posterolateral bladder wall. However, at that time the patient was found to have a newly developed advanced colonic adenocarcinoma. Three and a half months later she developed a bowel perforation and died.