ABSTRACT
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare tumor that affects elderly individuals and presents a poor prognosis. Skin is the most common site of involvement, accounting for 89% of the cases. Extracutaneous organs, especially bone marrow, lymph nodes, and peripheral blood, can be involved at the time of diagnosis. We report a case of BPDCN in a child, presenting with a cutaneous lesion mimicking lymphoma or leukemia cutis. The histologic findings revealed a dense diffuse infiltration by monomorphic agranular medium-sized blast cells with sparing of the grenz zone, whose first immunophenotypic profile raised the possibility of diagnosing B lymphoblastic lymphoma or leukemia. However, the absence of CD10 expression and strongly positive expression for CD4, CD56, CD45RA, and the plasmacytoid dendritic cell-associated antigens, including CD123, supported the definite diagnosis of BPDCN. The patient responded well to a systemic combination chemotherapy regimen, modified from the Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) protocol for anaplastic large cell lymphoma (ALCL), that differed from the established recommendation using ALL protocol. Owing to the patient's excellent treatment outcome, this regimen could represent an effective alternative regimen for BPDCN in children.
