Blastic plasmacytoid dendritic cell neoplasm with centrocyte‐like morphology clinically simulating a melanocytic nevus

Chu, Christopher H.; Rudnick, Eric; Motaparthi, Kiran

doi:10.1111/cup.13099

Cited by 3 publications

(2 citation statements)

References 18 publications

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“…The neoplastic cells have scant cytoplasm and eccentrically positioned nuclei with ovoid or slightly irregular contours, fine chromatin, and one or more small nucleoli. [ 10 , 34 – 36 ] Mitotic figures are variable, and necrosis can be present (Fig. 2C ).…”

Section: Pathologic Featuresmentioning

confidence: 99%

Blastic plasmacytoid dendritic cell neoplasm: a comprehensive review in pediatrics, adolescents, and young adults (AYA) and an update of novel therapies

Cuglievan

Connors

et al. 2023

Leukemia

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Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy that can involve the bone marrow, peripheral blood, skin, lymph nodes, and the central nervous system. Though more common in older adults, BPDCN has been reported across all age groups, including infants and children. The incidence of pediatric BPDCN is extremely low and little is known about the disease. Pediatric BPDCN is believed to be clinically less aggressive but often with more dissemination at presentation than adult cases. Unlike adults who almost always proceed to a hematopoietic stem cell transplantation in first complete remission if transplant-eligible, the majority of children can be cured with a high-risk acute lymphoblastic leukemia-like regimen. Hematopoietic stem cell transplantation is recommended for children with high-risk disease, the definition of which continues to evolve, or those in relapse and refractory settings where outcomes continue to be dismal. Novel agents used in other hematologic malignancies and CD123 targeted agents, including chimeric antigen receptor T-cells and monoclonal/bispecific antibodies, are being brought into research and practice. Our goal is to provide a comprehensive review of presentation, diagnosis, and treatment by review of pediatric cases reported for the last 20 years, and a review of novel targeted therapies and therapies under investigation for adult and pediatric patients.

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Section: Pathologic Featuresmentioning

confidence: 99%

Blastic plasmacytoid dendritic cell neoplasm: a comprehensive review in pediatrics, adolescents, and young adults (AYA) and an update of novel therapies

Cuglievan

Connors

et al. 2023

Leukemia

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“…After excluding myelomonocytic neoplasm and cutaneous lymphoma, confirmation with PDC markers was required for BPDCN diagnosis. [ 7 ] More recently, a study suggested initial dual immunostaining for TCF4 and CD123 for any skin lesion suspected of BPDCN. If the tumor cells showed dual expression of TCF4 and CD123, further immunostaining was not required.…”

Section: Discussionmentioning

confidence: 99%

A Rare Case of Blastic Plasmacytoid Dendritic Cell Neoplasm in a Child Mimicking Lymphoma/Leukemia Cutis

et al. 2022

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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.

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Blastic plasmacytoid dendritic cell neoplasm with immunoblastoid morphology and MYC rearrangement and overexpression

et al. 2019

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Blastic plasmacytoid dendritic cell neoplasm with centrocyte‐like morphology clinically simulating a melanocytic nevus

Cited by 3 publications

References 18 publications

Blastic plasmacytoid dendritic cell neoplasm: a comprehensive review in pediatrics, adolescents, and young adults (AYA) and an update of novel therapies

Blastic plasmacytoid dendritic cell neoplasm: a comprehensive review in pediatrics, adolescents, and young adults (AYA) and an update of novel therapies

A Rare Case of Blastic Plasmacytoid Dendritic Cell Neoplasm in a Child Mimicking Lymphoma/Leukemia Cutis

Blastic plasmacytoid dendritic cell neoplasm with immunoblastoid morphology and MYC rearrangement and overexpression

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