A chronic eyelid lesion in a child: multi-disciplinary approach to diagnosis, treatment and management of a highly atypical histiocytic lesion. (17th February 2022)

• Record Type: Journal Article
• Authors: Ramgopal, Archana; Segal, Julia; Mukhtar, Sabrina; Yu, Jenny; Picarsic, Jennifer; Tersak, Jean M.; Allen, Steven W.
• Is Part Of: Pediatric hematology and oncology. Volume 39:Number 2(2022)
• Page Start: 180
• Page End: 186
• Publication Date: 2022-02-17
• Subjects: Histiocytosis -- eyelid -- lesion -- pediatric -- HS histiocytic sarcoma -- CT computed tomography -- PET positron emission tomography; Pediatric hematology -- Periodicals; Tumors in children -- Periodicals; Blood -- Diseases -- Periodicals; Hematologic Diseases -- Child; Hematologic Diseases -- Infant; Neoplasms -- Child; 618.9215
• DOI: 10.1080/08880018.2021.1960657
• Languages: English
• ISSNs: 0888-0018

Abstract

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.