A rare case of blastic plasmacytoid dendritic cell neoplasm with a nasopharyngeal mass as the main feature and review of literature
SHI Qian-yun
WANG Xuan
YE Sheng-bin
FENG Xiao
ZHANG Ru-song
SHI Qun-li
MA Jie
Abstract：Objective To improve the understanding,diagnosis and differential diagnosis of blastic plasmacytoid dendritic cell neoplasm (BPDCN).Methods The clinical features,immunophenotype,molecular analysis,treatment and prognosis of 1 case of BPDCN with a nasopharyngeal mass as the main clinical feature were analyzed,and the literature was reviewed.Results A 33-year-old female patient clinically presented a nasopharyngeal mass.Morphology of the nasopharyngeal biopsy showed that the tumor cells were composed of small to medium sized ceils with scant cytoplasm and irregular nuclei.The chromatin was dispersed and the nucleolus was not obvious.Bone marrow puncture after admission showed diffuse small to medium sized tumor cells with a large number of necrosis.For difficuh diagnosis of the nasopharyngeal biopsy and bone marrow biopsy,the patient was enquired again,and then a forearm subcutaneous mass was found and skin biopsy was subsequently performed.Immunohistochemical examination showed that the tumor cells were positive for CD4,CD56,CD123,and negative for CD3,CD20,CD79α.In nasopharyngeal biopsy,hybridization in situ showed EBER negative,T cell receptor gene rearrangement was negative.The diagnosis of BPDCN was finally confirmed with invasion of the nasopharynx,bone marrow and skin.Conclusion BPDCN of nasal biopsy in patient clinically presented as a nasopharyngeal mass is easily misdiagnosed.We pathologists should raise awareness for this rare condition.Several differential diagnosis such as NK/T cell lymphoma should be excluded.
Keywords：Blastic plasmaeytoid dendritic cell neoplasmNasopharynxPathologic diagnosisDifferential diagnosis
Publication Date：2017-01-01
Online Publishing Date：2025-08-15(First online date of this platform, not the publication date of the document)
Pages：5( 598-602 )