introduction: desmoid tumors are very rare, benign fibrous neoplasms arise from the musculoaponeurotic structures throughout the body.   case report: the patient was a   seven-year old boy with a large mandibular mass growing over a period of six months. his ct-scan showed a large mass, 13 cm in diameter in the cheek area extending to the neck and trachea. biopsy was compatible with desmoid fib...

Two molecular recombinants (EU-8 and K-3) constructed from ring-necked pheasant virus and UR2AV, the helper virus associated with avian sarcoma virus UR2, caused a high incidence of a hitherto unreported pathological condition in chick skeletal muscle. A disease spectrum was observed in which muscle was infiltrated by proliferating fibroblasts and caused white streaks, white diffuse areas, or w...

Infantile myofibromatosis (IM) is a rare benign tumor in children. It was first described as congenital fibrosarcoma. Later, sporadic cases were discussed, and in 1981, 61 cases were examined and named as IM. Three different forms were described: solitary, multicentric and visceral IM.1 Three quarters of soft tissue tumours in children and adolescents are benign, 95 % of them are fibromatosis, ...

Spectrum of Fibroblastic and Myofibroblastic Tumors When one considers soft tissue tumors in pediatrics, tumors of vascular (29%), neurogenic (15%), and myogenic (striated muscle, 14%) origin occur more often than fibroblastic-myofibroblastic tumors (12%). The spectrum of fibroblastic/myofibroblastic tumors is quite divergent from both clinical and histopathologic viewpoints (Table 1). These tu...

The pathogenesis of generalized fibromatosis still remains unknown, despite reports on association with estrogen receptors. This disease is classified into three types as follows: solitary fibromatosis, congenital generalized fibromatosis without visceral involvement, and congenital generalized fibromatosis with both cutaneous and visceral involvement. In most cases, the disease is sporadic, bu...

Introduction: Aggressive fibromatosis (desmoid tumor) is a locally invasive soft tissue lesion, composed of a monoclonal proliferation of benignappearing spindle (fibroblast like) cells. A subset of lesions contain a somatic mutation in the adenomatous polyposis coli (APC) gene (1). Familial Adenomatosis Polyposis (FAP) is an inherited pre-neoplastic condition that predisposes to colonic neopla...

PURPOSE To explore the molecular bases of potential new pharmacologic targets in aggressive fibromatosis (desmoid tumor). EXPERIMENTAL DESIGN Tumor specimens from 14 patients surgically treated for aggressive fibromatosis (6 familial adenomatous polyposis and 8 sporadic cases), analyzed for adenomatous polyposis coli (APC) and CTNNB1 (beta-catenin) mutations, were further investigated for bet...

BACKGROUND Abdominoperineal excision is performed in patients with locally advanced, low rectal carcinoma. Reconstruction of the dorsal vagina and perineum using the vertical rectus abdominis myocutaneous flap following extensive surgery results in favorable surgical outcome and quality of life. However, the rectus abdominis muscle, as part of the anterior abdominal wall, may develop fibrous le...

Objective: The purpose of this study is to explore the clinical characteristics and therapeutic methods of aggressive fibromatosis (AF) in skull. Methods: The clinical data and operative therapy of one case of aggressive fibromatosis in skull in our department were examined and reviewed. The associated literatures were reviewed and discussed. Results: It was indicated that the main manifestatio...