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

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:

• Histiocytosis
• eyelid
• lesion
• pediatric
• HS histiocytic sarcoma
• CT computed tomography
• PET positron emission tomography

Conflict of interest and funding source

All authors have no conflicts of interest to disclose and no funding or pharmaceutical support was secured for this study.

Financial disclosure

All authors have no financial relationships relevant to this article to disclose.

Author’s Contribution

1 Data Interpretation

2 Literature Search

3 Manuscript Preparation

Abbreviations

Histiocytic sarcoma (HS)

Acknowledgments

We would like to acknowledge Srikala Narayanan, MD, Pediatric Radiologist at UPMC, for figure interpretation and compilation. In addition, thank you to Mr. Chris Woods, Imaging application specialist at Cincinnati Children’s Hospital Medical Center (CCHMC) for figure compilation. The University of Pittsburgh holds a Physician-Scientist Institutional Award from the Burroughs Wellcome Fund^AR

Financial disclosure

All authors have no financial relationships relevant to this article to disclose.