Blastic Plasmacytoid Dendritic Cell Neoplasm Presenting as a Mammary Gland Tumor in a Pediatric Patient: A Case Report

Int J Surg Pathol. 2023 Nov 13:10668969231211338. doi: 10.1177/10668969231211338. Online ahead of print.

Abstract

Emanating from a discrete category within the lympho-hematopoietic tumor system, as established by the World Health Organization in 2008, the blastic plasmacytoid dendritic cell neoplasm constitutes an uncommon malignant hematological disorder. It is routinely misidentified on account of its conspicuous dermatological manifestation, yet may insidiously permeate bone marrow and lymph nodes, involving peripheral blood and diverse extra-nodal tissues. Instances of mammary gland encroachment are extraordinarily infrequent. The current document delineates a case of a 14-year-old female patient contending with blastic plasmacytoid dendritic cell neoplasm, whose primary symptom was a mammary nodule, and whose breast and bone marrow/blood involvement were synchronous, in attempt to increase clinical vigilance.

Keywords: CD123-targeting therapies; blastic plasmacytoid dendritic cell neoplasm; hematopoietic stem cell transplantation; hyperCVAD regimen; tagraxofusp.