Abstract
The expression of TFE3 (transcription factor E3) in solitary fibrous tumours (SFTs) and their histologic mimickers was investigated, and the diagnostic value and clinical significance of TFE3 nuclear expression in SFTs were explored. Immunohistochemical analysis for TFE3 was performed on 50 cases of SFTs that were surgically resected. The controls were sample tissues from malignant peripheral nerve sheath tumour, synovial sarcoma, dedifferentiated liposarcoma, spindle cell lipoma, and dermatofibrosarcoma protuberans. The survival of patients with TFE3-positive and TFE3-negative expressions was assessed through the Kaplan-Meier analysis. In 44 of 50 (88%) SFTs, nuclear immunoreactivity for TFE3 was detected. The TFE3 expression was negative in all samples of synovial sarcoma, malignant peripheral nerve sheath tumour, dermatofibrosarcoma protuberans, and spindle cell lipoma and weakly positive in 2 of 10 cases of dedifferentiated liposarcoma. Fluorescence in situ hybridization (FISH) confirmed that the expression of the TFE3 protein is not caused by gene translocation. There was no statistical significance between the association of the TFE3 expression and SFT patient prognosis. Therefore, TFE3 is capable of enhancing the differential diagnosis of SFTs and their histologic mimickers and can be potentially used as a diagnostic marker. The findings also offer valuable insights into SFT diagnosis, aetiology, and associated molecular mechanisms.
Highlights
Solitary fibrous tumour (SFT) is a ubiquitous fibroblastic mesenchymal tumour, which has the potential to affect any region of the body
The histological features of SFTs are different from spindle cell lipoma, dermatofibrosarcoma protuberans, synovial sarcoma, and malignant peripheral nerve sheath tumours
To assess the specificity of Transcription factor E3 (TFE3) to SFTs, we carried out the tests on samples of malignant peripheral nerve sheath tumour, synovial sarcoma, dedifferentiated liposarcoma, spindle cell lipoma, and dermatofibrosarcoma protuberans
Summary
Solitary fibrous tumour (SFT) is a ubiquitous fibroblastic mesenchymal tumour, which has the potential to affect any region of the body. It was first described in the pleura by Klemperer and Rebin in 1931, and later in 1942, these tumours were observed in multiple sites [1, 2]. SFTs are morphologically diverse, characterized by an alternate distribution of hypocellular and hypercellular areas, and haphazard growth pattern of spindle cells mixed with fibrous bundles and haemangiopericytoma-like vessels. The histological features of SFTs are different from spindle cell lipoma, dermatofibrosarcoma protuberans, synovial sarcoma, and malignant peripheral nerve sheath tumours
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