Dunlap Quinn A, Day Kristine E, Borak Samuel G, Woodworth Bradford A
Department of Surgery, Division of Otolaryngology, University of Alabama at Birmingham, Birmingham, Alabama, USA.
Allergy Rhinol (Providence). 2014 Jan;5(1):50-2. doi: 10.2500/ar.2014.5.0085.
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy that possesses a heterogenous clinical and immunophenotypic presentation. The current case report describes an interesting and unique presentation of BPDCN as a primary paranasal sinus tumor without evidence of cutaneous or systemic involvement. As such, the report further contributes to the ongoing debate regarding the true putative origin of the neoplasm, as well as highlights the optimal diagnostic modalities, paramount importance of early diagnosis, and vast heterogeneity exhibited by this fascinating malignancy. The atypical presentation described here indicates the manifestations of BPDCN are more heterogenous than previously documented and thus can not be definitively ruled out in the absence of bone marrow, peripheral blood, or cutaneous involvement. Furthermore, atypical neoplastic presentations mandate flow cytometry and adjunctive immunohistochemistry for the definitive diagnosis of BPDCN, and early diagnosis of such neoplasms are critical for rapid initiation of treatment and improved outcomes.
母细胞性浆细胞样树突状细胞肿瘤（BPDCN）是一种罕见的血液系统恶性肿瘤，具有异质性的临床和免疫表型表现。本病例报告描述了BPDCN作为原发性鼻窦肿瘤的一种有趣且独特的表现，无皮肤或全身受累证据。因此，该报告进一步推动了关于该肿瘤真正假定起源的 ongoing 争论，同时突出了最佳诊断方式、早期诊断的至关重要性以及这种迷人的恶性肿瘤所表现出的巨大异质性。此处描述的非典型表现表明BPDCN的表现比先前记录的更为异质化，因此在没有骨髓、外周血或皮肤受累的情况下不能绝对排除。此外，非典型肿瘤表现需要流式细胞术和辅助免疫组化来明确诊断BPDCN，而此类肿瘤的早期诊断对于迅速开始治疗和改善预后至关重要。
