Recurrent Orbital Adult-Type Fibrosarcoma in a 3-Year-Old Girl

Abstract

A 3-year-old Filipino girl presented with progressive left-sided ptosis and proptosis after resection of a superomedial orbital tumor 2.5 years ago. She had been followed with serial MRI, which demonstrated interval growth of a recurrent lesion. Repeat resection was undertaken via an eyelid-splitting anterior orbitotomy. The patient's pediatric oncologist and sarcoma specialist did not recommend adjuvant chemotherapy, and the family declined proton radiotherapy. The patient will continue to be monitored with serial imaging. Histopathology, immunohistochemistry, and reverse transcriptase polymerase chain reaction were most consistent with a diagnosis of adult-type fibrosarcoma. This is the first reported case of adult-type fibrosarcoma presenting in the orbit of a child. Fibrosarcoma is a fibroblast-derived tumor that commonly presents in the extremities, usually in patients in their fourth to sixth decades. It is rarely seen in the orbit, with the largest case series to date only including 5 patients.

Diagnostic challenges in primary orbital fibrosarcoma: a case report

“…To our knowledge, only one case has been reported of adult-type FS in the orbit using immunohistochemistry and cytogenetic studies; unlike in this case, the tumor occurred in a 3-year-old child and did not affect the orbital rim or floor. 10 …”