0544 3 Diagnostic_Pasta:-

In 1832, Mc Ferlane first described a particular type of fibrous neoplasm originated in the muscular aponeuroses, characterized by a biologic behaviour intermediate between that of sarcomas and fibromas. In 1983, Müller coined the term desmoid tumor (1,2). In 1923, a case of extra-abdominal desmoid tumor was described by Nichols (3). Desmoid tumors also called extra-abdominal or aggressive fibromatosis – are rare fibromatous tumors of fibroblast origin which involve muscular connective tissue, muscular fascia and aponeuroses. They are locally very aggressive and tend to infiltrate adjacent tissue even it is that thought their clinical behaviour appears to be quite different from that of sarcomas. In fact, desmoid tumors are low-grade tumors which grow slowly but constantly, do not metastasize either through lymph nodes or through blood circulation; however, they have high rates of local recurrence (4,5). As the scarcity of cases limited the performing of statistically significant perspective studies, desmoid tumors although more frequently diagnosed nowadays still stimulate the curiosity of the scientific community. Overall, desmoid tumors are reported to account for less than 0.03% of all neoplasms, with only 2-4 new cases per million of inhabitants/year (6). They are fibroproliferative disorders arising from musculoaponeurotic tissue and their high local recurrence rates suggested a reactive etiology; however, recently it has been shown that, even if this tumor does not metastasize, a true neoplastic process underlies fibroblast proliferation due to desmoid cell clonality (7). Three main theories have been proposed to explain the pathogenesis of desmoid tumors: mechanical theory, endocrine theory, genetic theory. According to the first theory, desmoid tumors may be associated with trauma, stretch injuries of the abdominal wall and especially enlarging pregnant uterus; the SUMMARY: Diagnostic and behavioural parameters differentiating proliferative muscolo-fascial low grade lesions. Case reports.