Aggressive Fibromatosis, Aetiology, Diagnosis and Treatment

Abstract

*@ Summary Aggressive fibromatosisrepresents (AF) a semimalignant tumour which has a locally destructive and invasive growth tendency but does not metastasize because of its high recurrence rate, the tumour remains a surgical problem. AF is rare group of fibroproletlative tumour started initially as painful masses developing slowly affecting mainly the limbs, neck, trunk, and the abdomen in that order. The disease process of (AF) regardless of the site of presentation, sex, or the age of the patient, endocrine, genetic and physical factors seem to play an important role in the development of the disease. Recent innovation in the field of molucular biology showed the abnormalities in (AF) is at the level of chromosomes in the form of gene mutation and chromosomal anomalies. Other studies showed that the pathogenesis of (AF) is related or modulates by hormone. Physical trauma seemed to have role in the development of the tumour. Whether (AF) is benign or malignant tumour is still in the field of theories. Many investigators put this type of tumour in the category of malignant lesion even in the absence of distal metastasis. The modalities of treatment of (AF) is widely different but surgery makes the major bulk, provided there is a good preoperative demonstration of the lesion by contrast solution and MR imaging, in the recurrent types of (AF) the combination of other modalities of treatment, radiotherapy, chemotherapy, cytotoxic and noncytotocix with and without surgery could be the most appropriate way of management. In this paper the character, pathogenesis, development method of detecting and the different modalities of treatment have been reviewed from literature.