MYOFIBROBLASTIC TUMORS - SHORT SUMMARY OF THE CLINICAL-FEATURES, DIAGNOSIS, AND DIFFERENTIAL-DIAGNOSIS

This review summarizes myofibroblastic tumours that have been characte rized in the last years. These lesions include: fibromatoses in adults and infants (infantile digital fibromatosis and infantile myofibromat osis); myofibroma of adults, an almost exclusively solitary lesion in the skin which is characterized morphologically as a biphasic lesion c omposed of spindle-shaped eosinophilic tumour cells and more primitive mesenchymal tumour cells associated with a haemangiopericytoma-like v asculature; dermatomyofibroma (plaque-like dermal fibromatosis), a ban d-like myofibroblastic proliferation in young female patients, which i s mainly located in the periaxillar region and in which distinction fr om more aggressive, plaque-like variant of dermatofibrosarcoma protube rans is mandatory; myofibroblastoma of the breast, a well-circumscribe d lesion composed of spindle shaped, desmin-positive tumour cells, whi ch is seen mainly in elderly male patients and has to be distinguished from other spindle cell lesions of the breast;angiomyofibroblastoma, a well-circumscribed myofibroblastic neoplasm of the vulva and vagina composed of ovoid to round myoid tumour cells with scattered multinucl eated cells, which forms a continuous morphological spectrum with the clinically more aggressive angiomyxoma in this location;intranodal myo fibroblastoma, a distinctive proliferation of myofibroblastic cells as sociated with so-called amianthoid fibres, which is seen most commonly in inguinal lymph nodes; myofi broblastoma/myofibroblastic tumour of soft tissues, a variably well-circumscribed myofibroblastic lesion whi ch lacks atypia and is composed of actin and/or desmin positive tumour cells, and poorly delineated sarcomas with myofibroblastic differenti ation (myofibrosarcoma).