Clinicopathological features of sinonasal SMARCB1-deficient adenocarcinoma
YUE Chang-li
XIONG Yan
LIU Hong-gang
PIAO Ying-shi
Abstract：Objective To investigate the clinicopathological features of primary SMARCB1-deficient sinonasal adenocarcinomas(SNACs).Methods The clinicopathological data of 6 cases of SMARCB1-deficient SNACs were collected,and the clinicopathological characteristics were observed and analyzed,and related literatures were reviewed.Results Six patients were male,aged 34-78 years,with a median age of 60 years.The tumor cells were uniform,showing oncocytoid in 5 cases(5/6),plasmacytoid in 3 cases(3/6),signet-ring and clear cell like in 1 case,respectively(1/6).Tubules in the form of punched-out luminal spaces and yolk sac tumor like were noted in 1 case(1/6),cribriform in 4 cases(4/6)and yolk sac tumor like structures(1/6),and mucoid matrix was commonly found(5/6).Immunohistochemical staining showed that all cases were completely negative for SMARCB1,5 cases shown SMARCA2 loss,and 1 case was weak expression,while ARID1A and SMARCB4 were retained,with moderate expression for CK7(4/6)and focal expression for p63(2/6)and Sall4(2/6),and no expression for CDX2,PLAP and S100.The SMARB1 gene deletion was detected in 3 cases by FISH.Among the 4 cases followed up,3 cases died within 16 months after operation,and 1 case recurred in three times and survived.Conclusion SMARCB1-deficient SNAC is rare and its prognosis is poor.Combination of histological morphology and immunohistochemistry is helpful for its differential diagnosis.
Keywords：Nasal cavity and paranasal sinusSMARCB1-deficient adenocarcinomaClinicopathological featuresDifferential diagnosis
Publication Date：2025-03-28
Online Publishing Date：2025-08-15(First online date of this platform, not the publication date of the document)
Pages：5( 263-267 )