Follicular dendritic cell tumor: report of 13 additional cases of a distinctive entity.

Follicular dendritic cell (FDC) tumor is an extremely rare malignant neoplasm with approximately 17 well-documented cases in the literature. We report 13 additional cases of this distinctive neoplasm. There were seven men and six women, with a mean age of 46.5 years (range, 27-62 years). There was involvement of cervical lymph nodes (six cases), mediastinum (three cases), axilla, tonsil, spleen, and peripancreatic soft tissues (one case each). The neoplasms were grey to tan, ranging in size from 1 to 13 cm. They were formed by oval to spindle cells with eosinophilic cytoplasm growing in sheets and fascicles, with a focal storiform pattern and whorls reminiscent of those seen in meningioma. The nuclei were oval or elongated with thin nuclear membranes, inconspicuous or small eosinophilic nucleoli, and clear or dispersed chromatin. Typically, the tumor cells were intimately admixed with small lymphocytes, with a prominent perivascular cuffing. Multinucleated tumor cells were present in seven cases. Necrosis, marked cellular atypia, high mitotic rate, and/or abnormal mitoses were present in seven cases. The tumor cells were positive for CD21 (10 of 11), CD35 (10 of 11), Ki-M4p (seven of eight) Ki-FDRC1p (six of seven), vimentin (five of nine), and S100 protein (five of nine). One case stained with actin. In situ hybridization, done in six cases, did not show Epstein-Barr virus RNA sequences. Ultrastructural examination of eight cases showed long, complex, occasionally interdigitating cytoplasmic processes joined by desmosomes. The behavior of these tumors is more akin to that of a low-grade soft tissue sarcoma than a malignant lymphoma and is characterized by local recurrences and occasional metastases. Two patients died of tumor, two were alive with recurrent or metastatic disease, eight were alive with no disease, and one was lost to follow-up.