A chronic eyelid lesion in a child: multi-disciplinary approach to diagnosis, treatment and management of a highly atypical histiocytic lesion

Pediatr Hematol Oncol. 2022 Mar;39(2):180-186. doi: 10.1080/08880018.2021.1960657. Epub 2021 Aug 11.

Abstract

Malignant histiocytic neoplasm with histiocytic sarcoma phenotype is a rare malignant neoplasm, distinguished by malignant cells with phenotypic characteristics of mature tissue histiocytes. Histiocytic sarcoma typically presents as a primary malignancy, although can also present as a secondary malignancy, and is rarely seen in the pediatric population. Due to the rarity of this condition, diagnosis of histiocytic sarcoma is difficult and considered a diagnosis of exclusion. We describe a unique case of a chronic upper eyelid lesion with biopsy findings of a highly atypical histiocytic neoplasm initially concerning for histiocytic sarcoma; however, after integration of clinical findings, non-progressive and quiescent molecular profile, concluded to be an atypical juvenile xanthogranuloma in a child treated with excision and observation alone. This report highlights the importance of an integrated team approach to diagnosis of unusual histiocytic neoplasms.

Keywords: CT computed tomography; HS histiocytic sarcoma; Histiocytosis; PET positron emission tomography; eyelid; lesion; pediatric.

Publication types

  • Case Reports

MeSH terms

  • Child
  • Eyelids / pathology
  • Histiocytes / pathology
  • Histiocytic Sarcoma* / diagnosis
  • Histiocytic Sarcoma* / pathology
  • Histiocytic Sarcoma* / therapy
  • Humans