Primary cutaneous blastic plasmacytoid dendritic cell neoplasm in a child: A challenging diagnosis and management

Abstract

A 13-year-old boy was referred with a 10-month history of an asymptomatic dark violaceous infiltrated nodular lesion on the left cheek (Figure 1). He had been previously treated with topical and systemic steroids with partial improvement. A skin biopsy revealed a dense dermal infiltrate of monomorphous, poorly differentiated medium-sized cells with blastic morphology (Figure 2A-B). Immunohistochemical staining revealed positive CD4, CD56, and CD123 cells that were negative for CD3, CD8, CD20, CD10, PAX5, TdT, LMP1, and MPO; Ki67 index was 40% (Figure 2C). The diagnosis of blastic plasmacytoid dendritic cell neoplasm (BPDCN) was made. Bone marrow (BM) biopsy and cerebrospinal fluid (CSF) examination were normal. Positron emission tomography-computed tomography (PET/CT) revealed hypermetabolic activity (SUVmax 4.68) localized to the skin. After careful risk-benefit deliberation with a multidisciplinary team, systemic treatment with a high-risk acute lymphoblastic leukemia (ALL) chemotherapy protocol was initiated (vincristine, dexamethasone, asparaginase, daunomycin, cytarabine, cyclophosphamide, and methotrexate). The patient is currently receiving treatment and has had good response without complications after 18 months' follow-up. How to cite this article: Rivas-Calderón MK, Cheirif-Wolosky O, Rosas-Romero ME, et al. Primary cutaneous blastic plasmacytoid dendritic cell neoplasm in a child: A challenging diagnosis and management.

Pediatric Blastic Plasmacytoid Dendritic Cell Neoplasm, Clinical Features and Immunophenotype: A Case Report

“…These lesions vary from hematoma-like macules to purplish plaques or nodules; focal lesions often affect the head and lower extremities, although they may spread throughout the body [ 11 , 12 ]. As shown in Figure 1 , the characteristic lesions of the cutaneous involvement are non-specific and are easily confused with other differential diagnoses [ 3 , 4 , 13 ].…”

“…A variety of treatments have been used in adult patients, including chemotherapy regimens based on protocols for non-Hodgkin's lymphoma, myeloid leukemia, and ALL, with and without HSCT. In addition, the low incidence of the disease makes it difficult to define an optimal therapeutic approach; however, in children, treatment with high-risk ALL protocols is the most commonly used strategy and is associated with the best-reported outcomes [ 4 , 5 , 13 ].…”