Solitary fibrous tumor
A rare soft tissue tumor characterized by a well-circumscribed mass potentially occurring at any anatomical site histopathologically showing spindled to ovoid cells arranged around a branching and hyalinized vasculature and variable stromal collagen deposition. Immunohistochemistry reveals CD34 and/or STAT6 expression. NAB2-STAT6 gene fusions are pathognomonic for this tumor which may be malignant or benign. Clinically most patients present with a slow-growing painless mass. Large tumors may cause paraneoplastic syndromes such as Doege-Potter syndrome with the induction of severe hypoglycemia or acromegaloid features.
Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.
Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http: //www.orphadata.org. Data version September 2023.
Newly diagnosed with
Solitary fibrous tumor?
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