Clinicopathological characteristics of extranodal follicular dendritic cell sarcoma: A report of two cases

Abstract

Follicular dendritic cell sarcoma (FDCS) is an extremely rare tumor, which mainly originates from FDCs in the lymph nodes. Sometimes FDCS can arise from outside the lymph nodes due to the existence of acquired lymphoid tissue, which becomes the histological basis of the tumor. The diagnosis of FDCS, particularly extranodal FDCS, presents a challenge for pathologists and hematopathologists. The present study presents two cases of extranodal FDCS based on clinical features and histomorphology. Soft tissue of the chest wall was involved in case 1 and right tonsil tissue in case 2. Case 1 underwent surgery, and was in good health post-operatively. During the 5-month post-operative follow-up period, the patient was healthy in all respects. Case 2 received surgery combined with radiotherapy, and the follow-up data reported that the patient remained alive, without signs of recurrence or metastasis during the 4-month post-operative follow-up period. Additionally, a total of 102 cases of extranodal FDCS were retrieved from the literature, which were extracted and reviewed carefully. The rates of recurrence, metastasis and mortality were 14.63 (12/82), 17.07 (14/82) and 8.29% (15/82), respectively. The overall survival rates of the 102 cases, showing 2-year total survival rates, were 70%, the same with that of 5-year total survival rates. The 2-year tumor-free total survival rates were 68%, and the 5-year equivalents were 32%. Female patients had a poorer prognosis than male patients (P<0.05). Kaplan-Meier estimation presented no statistically significant differences between disease-free survival rates or overall survival rates and age, tumor size or treatment (P>0.05).