Calcifying Fibrous Tumor : Introduction & Clinical
Image Description
Introduction: Calcifying fibrous tumor/pseudotumor (CFT) is a rare benign fibrous lesion that is usually seen in children and young adults with a slight female predilection. It consists of hypocellular, sclerotic collagen, small calcific deposits with psammoma bodies, and scattered lymphoplasmacytic cells..
Clinical Presentation: CFT has a wide anatomic distribution, from soft tissues to visceral organs. It presents as a painless, slow-growing subcutaneous or deep soft tissue mass in the extremities, trunk, inguinal region, and head and neck. Cases have been reported in the gastrointestinal tract (especially stomach), pleura, oral cavity, heart, lung, gall bladder, and adrenal gland.
Association has been reported between the rare intraabdominal cases of CFT and sclerosing angiomatoid nodular transformation of spleen and other IgG4-related sclerosing diseases. A small number of pleural CFTs have shown mutations in ZN717, FRG1 and CDC27 genes and recurrent copy number losses.
Clinical Presentation: CFT has a wide anatomic distribution, from soft tissues to visceral organs. It presents as a painless, slow-growing subcutaneous or deep soft tissue mass in the extremities, trunk, inguinal region, and head and neck. Cases have been reported in the gastrointestinal tract (especially stomach), pleura, oral cavity, heart, lung, gall bladder, and adrenal gland.
Association has been reported between the rare intraabdominal cases of CFT and sclerosing angiomatoid nodular transformation of spleen and other IgG4-related sclerosing diseases. A small number of pleural CFTs have shown mutations in ZN717, FRG1 and CDC27 genes and recurrent copy number losses.