Orbital Histiocytic Sarcoma in a 2-Year-Old Child

Abstract

Histiocytic sarcoma (HS) is an exceedingly rare hematologic malignancy that typically presents in the skin, gastrointestinal tract, and lymph nodes. Few cases of HS have been reported in the head and neck. We describe the case of a two-year-old girl presenting with two weeks of left lower eyelid swelling. Diagnostic testing and biopsy revealed a large inferior orbital mass causing severe bony destruction with extension into the sinuses. Pathologic analysis revealed classic features of histiocytic sarcoma. To the best of our knowledge, no previous case of histiocytic sarcoma occurring in the orbit of a child has been reported. We present an exceedingly rare case of histiocytic sarcoma in a young child presenting with eyelid swelling.

Pediatric Lymphoid and Histiocytic Lesions in the Head and Neck

“…Lymph node is the most common site of disease, but many extranodal sites can be involved. Rare cases of HS in head and neck in children are reported in the literature including maxilla and mandible [ 60 ] and orbit [ 61 ]. In children, HS can follow lymphoblastic leukemia [ 62 ].…”

Orbital Apex Syndrome in a Patient With Histiocytic Sarcoma

“…1,2 Histiocytic sarcoma usually affects the lymph node, skin, and gastrointestinal tract, while the orbit is more rarely involved, and to our knowledge, only 1 case was reported with the pre-sentation of eyelid swelling. 2,3 The differential diagnosis for malignant tumors that commonly invade the orbit includes lymphoma, squamous cell carcinoma, melanoma, and metastatic disease. Although there is no consensus on the imaging features of histiocytic sarcoma, this case indicates it should be considered when facing a rapidly aggressive mass lesion with bone destruction.…”

“…To our knowledge, there are few reports of orbital apex syndrome caused by histiocytic sarcoma, which is a rare lymphohematopoietic system tumor . Histiocytic sarcoma usually affects the lymph node, skin, and gastrointestinal tract, while the orbit is more rarely involved, and to our knowledge, only 1 case was reported with the presentation of eyelid swelling . The differential diagnosis for malignant tumors that commonly invade the orbit includes lymphoma, squamous cell carcinoma, melanoma, and metastatic disease.…”