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Paediatrics
Rare blood neoplasm masking as a concerning soft tissue tumour: blastic plasmacytoid dendritic cell neoplasm
  1. Sarah Braungart1,2,
  2. Jens Stahlschmidt3,
  3. Chris Lethaby4 and
  4. Aiden Moore2
  1. 1Paediatric Surgery, NHS Greater Glasgow and Clyde, Glasgow, UK
  2. 2Paediatric Surgery, Leeds Teaching Hospitals NHS Trust, Leeds, UK
  3. 3Histopathology, Leeds Teaching Hospitals NHS Trust, Leeds, UK
  4. 4Paediatric Oncology, Leeds Teaching Hospitals NHS Trust, Leeds, UK
  1. Correspondence to Sarah Braungart; sarah.braungart{at}nhs.scot

Abstract

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive malignancy with fewer than 100 paediatric cases reported. This report details a boy who presented in young childhood with a skin lesion on his left buttock that clinically resembled a sarcomatous lesion. Histopathological work-up including flow cytometry, immunophenotyping and phenotypic correlation were consistent with the diagnosis of BPDCN. The diagnosis of BPDCN radically altered this patient’s treatment pathway. The case highlights the importance of multidisciplinary team evaluation in soft tissue masses and careful consideration of alternative diagnoses. For this child, the diagnosis of BPDCN prevented an extensive local resection.

  • Malignant and Benign haematology
  • Paediatric oncology
  • Pathology
  • Paediatric Surgery
  • Surgical oncology

https://doi.org/10.1136/bcr-2024-262050

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    Footnotes

    • X @Sarah_Braungart

    • Contributors Guarantor for the manuscript: SB. The following authors were responsible for drafting of the text, sourcing and editing of clinical images, investigation results, drawing original diagrams and algorithms, and critical revision for important intellectual content: SB, JS, CL and AM. The following authors gave final approval of the manuscript: SB, JS, CL and AM.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

    • Competing interests None declared.

    • Provenance and peer review Not commissioned; externally peer reviewed.