Canceration from aggressive fibromatosis to fibrosarcoma——a report of one case and literature review

Abstract

Objective To determine the clinical manifestation,pathologic behavior,therapy and related factors of rare aggressive fibromatosis.Methods Canceration from aggressive fibromatosis to fibrosarcoma in one case of aggressive fibromatosis was analyzed and relevant literatures were reviewed.Results Aggressive fibromatosis was a benign or semimaligrant,non-metastatic proliferations of fibrous tissue that infiltrated surrounding tissues and tended to recur after surgical resection.Pathology showed fibroblastic monoconal proliferation between the cellular center and the collagen periphery.Electron microscope showed an abundant collagen network enclosing a pelymorphous cellular proliferation.Immunohistochemistry defined vimentin and actin positive desmoid tumors.Complete surgical excision was a chief treatment.Several operations were needed in most cases.Recurrence rates may be as high as 10%～70%.Chemotherapy and radiotherapy may be used together with surgery in recurrence or unsatisfactory surgical margin.The disease recurred after operation and one radiation therapy,reoperation and pathology showed fibrosarcoma.Conclusion Aggressive fibromatosis is a rare benign,non-metastatic proliferation tumour of fibrous tissue with a tendency of local recurrent after surgical resection.The cornerstone of therapy is surgery.Radiation therapy and chemotherapy can be used as adjuvant therapy.It is scarce that aggressive fibromatosis cancerates to fibrosarcoma.Maybe surgical stimulus and radiation therapy are the main factors to promote it to cancerate. Key words: Aggressive fibromatosis; Fibrosarcoma; Radiation therapy