Extranodal follicular dendritic cell sarcoma of the pharyngeal region:a report of 3 cases with review of literature / 临床与实验病理学杂志

ABSTRACT

Purpose To report the c1inicopatho1ogica1 characteristics,diagnosis and differentia1 diagnosis of extranoda1 fo11icu1ar den-dritic ce11 sarcoma( FDCS)of the pharyngea1 region. Methods The c1inica1 features,histopatho1ogica1 changes and immunohisto-chemica1 findings were ana1yzed in three cases of FDCS with review of the re1ated 1iterature. Results Case 1,a 70-year-o1d man pres-ented with the comp1aint of a pain1ess mass in pharyngea1 region accompanied by shortness of breath for the past 2 months. Case 2,a 40-year-o1d woman presented with the comp1aint of pharyngea1 foreign body sensation and b1oody sputum for the past 1 month. Case 3, a 38-year-o1d man presented with the comp1aint of intermittent epistaxis for the past 2 months. 3 cases showed simi1ar morpho1ogiesthe neop1astic ce11s were ovoid to spind1e-shaped,with indistinct ce11 borders,dispersed granu1ar chromatin,and scattered sma11 nuc1e-o1i. Notab1y,severa1 nuc1ear inc1usions were identified,and rare binuc1ear and mu1tinuc1eated ce11s were a1so present. There were main1y 3 kinds of growth patterns in the tumorsdiffuse sheets,fascic1es,and storiform arrangements admixed with sma11 1ymphocytes, which sometimes gathered into a mass. Immunohistochemica11y,tumor ce11s( 3/3 )were strong1y and diffuse1y positive for fo11icu1ar dendritic ce11 markers CD21,CD23 and CD35. Tumor ce11s(3/3)were a1so diffuse1y positive for fascin and D2-40. Some tumor ce11s (1/3)were diffuse1y positive for CXCL-13. Ki-67 pro1iferation index was estimated at 6%-20%. Conclusions Extranoda1 FDCS of the pharyngea1 region is rare and misdiagnosis is frequent1y made. A comprehensive eva1uation of c1inica1 manifestations,patho1ogic features and immunohistochemica1 findings are essentia1 for definitive diagnosis.