Primary Intraosseous Solitary Fibrous Tumor of the Mandible: Report of a Diagnostically Challenging Case with NAB2::STAT6 Fusion and Review of the Literature.

Abstract

Solitary fibrous tumor (SFT) represents an uncommon mesenchymal neoplasm affecting primarily the extremities and deep soft tissues with, overall, benign but locally aggressive biologic behavior and an underlying pathognomonic NAB2::STAT6 fusion. Intraosseous SFTs are infrequent, and involvement of the jawbones is exceedingly rare. A 54-year-old woman presented with an asymptomatic, well-demarcated, multilocular radiolucency of the left posterior mandible featuring focally irregular borders, root resorption and lingual cortex perforation. The lesion had shown progressive growth over a 6-year period. Microscopically, a proliferation of predominantly ovoid and spindle-shaped cells with indistinct cell membrane borders, elongated, plump or tapered, hyperchromatic nuclei, and lightly eosinophilic cytoplasm was noted. Marked cytologic atypia, pleomorphism and mitoses were absent. A secondary population of epithelioid cells exhibiting ovoid or elongated vesicular nuclei, and abundant, pale eosinophilic or vacuolated cytoplasm was also present. The supporting stroma was densely fibrous with areas of marked hyalinization and variably-sized, ramifying, thin-walled vessels. By immunohistochemistry, lesional cells were strongly and diffusely positive for STAT6 and CD99, and focally immunoreactive for MDM2 and SATB2. Ki-67 was expressed in less than 5% of lesional cells, while most interspersed epithelioid cells were positive for the histiocyte marker, CD163. Molecular analysis disclosed a NAB2::STAT6 fusion confirming the diagnosis of SFT. The patient underwent segmental mandibulectomy. Herein, we report the first case of primary intraosseous SFT of the mandible with complete documentation of its characteristic immunohistochemical and molecular features. Diagnosis of such unusual presentations may be further complicated by the challenging histomorphologic diversity of SFT.