Blastic plasmacytoid dendritic cell neoplasm without cutaneous involvement: A rare disease with a rare presentation

Blastic plasmacytoid dendritic cell neoplasm: a report of four cases and review of the literature

“…Cutaneous lesions vary widely, from one or two nodules to disseminated cutaneous lesions associated with superficial lymphadenopathy and occasionally systemic symptoms, such as weakness and weight loss. Lack of cutaneous involvment is rare 20–22 . Atypical presentation has been described, with some patients presenting with early liver metastasis 23 or with a renal mass 24 .…”

“…Lack of cutaneous involvment is rare. [20][21][22] Atypical presentation has been described, with some patients presenting with early liver metastasis 23 or with a renal mass. 24 The present report describes the first case of BPDCN presenting with a breast nodule (patient 4).…”

Blastic plasmacytoid dendritic cell neoplasm of the skin associated with myelodysplastic syndrome

“…This disease is highly aggressive, with a clinical presentation characterized by early skin involvement and rapid dissemination. Approximately 85% of the reported cases demonstrated cutaneous involvement at presentation, whereas a lack of cutaneous involvement is rare, with approximately 30 cases that have been reported [4,5]. Atypical presentation has been described; some patients presented with early liver metastasis [6] or with a renal mass [7].…”

Pathology Quiz Case: Plasmacytoid Dendritic Cell Neoplasm

“…2 Among hematologic malignancies, the overall incidence of BPCDN is extraordinarily low (0.4 -0.7%), with two-thirds of all cases affecting elderly persons Ͼ55 years of age, including an increased susceptibility in male subjects (3:1). [3][4][5][6] Frequently an initial finding, skin lesions are almost always (90 -99%) present in BPDCN prompting otherwise asymptomatic patients to seek medical attention and treatment. Evaluation of these patients reveals variable involvement of bone marrow, peripheral blood, spleen, lymph nodes, and extrahematopoietic sites, highlighting the phenotypic heterogeneity of this neoplasm.…”

“…Evaluation of these patients reveals variable involvement of bone marrow, peripheral blood, spleen, lymph nodes, and extrahematopoietic sites, highlighting the phenotypic heterogeneity of this neoplasm. [3][4][5][6][7] Without immediate initiation of appropriate treatment, rapid dissemination and systemic symptoms appear. Despite an often indolent initial presentation, BPDCN is almost invariably a highly aggressive disease with median survival ranging from 12 to 20 months from time of diagnosis.…”