Atypical presentation of blastic plasmacytoid dendritic cell neoplasm: a potential diagnostic pitfall in nasal cavity

Publication date: Available online 3 May 2018
Source:Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology
Author(s): Fang Yu, Ke Sun, Zhaoming Wang
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare disease with an aggressive clinical course resulting in median survival times of 12-14 months¹. It represents approximately 0.8% of primary cutaneous lymphomas². According to the WHO Classification of Tumors of Hematopoietic and Lymphoid Tissue in 2008, BPDCN is defined as an acute myeloid leukemia related precursor neoplasm, derived from precursors of the plasmacytoid dendritic cell. It is characterized microscopically by its dense monomorphous infiltrates of medium-sized blastoid morphology and expression of CD4 and CD56 as well as the absence of any common myeloid, T-lymphoid, B-lymphoid, and NK-lymphoid lineage markers³. A few series of BPDCN have been reported in the literature, but very few cases of BPDCN have been described with lack of skin manifestation at the time of diagnosis⁴. Here we report a rare BPDCN that happened in nasal cavity.



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