We report the youngest case of solitary fibrous tumor (SFT) with extensive involvement of the nasolacrimal duct system and discuss current literature regarding this tumor type. A 12-year-old female presented with a 6-month history of an enlarging right medial orbit mass. CT orbits revealed a well-circumscribed, enhancing lesion adjacent to the right nasolacrimal system. Pathology confirmed SFT (1.2 × 1.1 × 1.1 cm) with spindle cell morphology and a mitotic rate of 5 per 10 high power fields. Immunohistochemistry (IHC) was positive for CD-34 and signal transducer and activator of transcription 6 and negative for S-100, consistent with SFT. Next-generation sequencing confirmed NGFI-A-binding protein 2::signal transducer and activator of transcription 6 gene fusion. To our knowledge, only 17 cases of SFT involving the lacrimal sac have been reported, of which, the average age was 43.5 years. Notably, SFTs with a high mitotic rate carry a heightened risk of malignant transformation. Given our patient's mitotic rate of 5 per 10 high power fields, positive surgical margins, and young age, close follow-up is imperative.
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