A case of a large solitary fibrous tumor in the thigh, displaying NAB2ex4-STAT6ex2 gene fusion

Abstract

A solitary fibrous tumor (SFT) is documented in several body sites. However, there are few reports on the radiological and corresponding histopathological, including immunohistochemical, features of SFT in the lower extremities. A 58-year-old male presented with a lump in his right thigh of 6months duration. Plain radiograph revealed a soft tissue lesion in his right thigh, involving the adjacent mid-diaphysis and showing focal cortical thickening and calcification. Magnetic resonance imaging scans displayed two well-defined, T1-isointense and T2 heterogeneously hyperintense lesions, measuring together 15cm in the intermuscular plane and the juxtacortical location along the mid-diaphyseal region of the right femur. Radiologically, the differential diagnoses considered were undifferentiated pleomorphic sarcoma and synovial sarcoma. Microscopic examination of the core biopsy and the resected tumor revealed a tumor composed of cells with oval to spindle-shaped nuclei in a variably collagenized stroma, including hyalinized blood vessels and focal dystrophic calcification. Mitotic figures were 4/10 high power fields. Immunohistochemically, the tumor cells were positive for CD34, BCL2, and STAT6. Diagnosis of malignant SFT was offered. The tumor displayed NAB2ex4-STAT6ex2 gene fusion on molecular testing. This constitutes a relatively uncommon case report of a large SFT in the thigh, including its radiological and pathological features, confirmed by STAT6 immunostaining. An SFT should be considered in cases of slow-growing, well-defined soft tissue tumors, which are isointense on T1 and heterogeneously hyperintense on T2-weighted sequences, and display calcification and cortical thickening of the adjacent bones. Various differential diagnoses and their treatment-related implications in such cases are discussed herewith.