Clinical And Pathological Features Of Aggressive Fibromatosis In Larynx

Clinical and pathological features of aggressive fibromatosis in larynxObjective Aggressive fibromatosis in larynx is a rare borderline fibroblast neoplasm exhibiting local aggressiveness. At present, because of our insufficient clinical cognition, the managements of them are inappropriate, which affects the prognosis of the patients with aggressive fibromatosis in larynx, and leads to poor life quality. Our study on this problem is to understand its biological behavior and evaluate the clinical features and the treatments of the desease. Methods Based on one patient with aggressive fibromatosis in larynx treated in our hospital, clinical and pathological findings were analyzed. The nosogenesis, clinical and pathological features,feasible ways of treatment and the prognosis of the aggressive fibromatosis in larynx were discussed. Results The lesion manifested with voice hoarseness and dyspneic respiration. Grossly, the glottic rim was infiltrated by a hard, grey-white tissue showing a tentacular outline. Tissue sections featured a moderately cellular lesion composed of collagenoblast and collagenous fibers. The collagenoblast is quite swelling, but is not mature, with orbicular-ovate or tapered fusiform nucleus, without obform, and mitotic figure is rare. Some may arrange like fascicularis, vorticose,or paliform,some also arrange disorderly, having no certain direction. The spindle cells were immunopositive for actins and vimentin, and negative for keratins, epithelial membrane antigen, desmin, and S-100 protein. After an operation was given to the patient, without radiotherapy, clinical symptom of the patient has been improved. Following up for 10 months, there is no clinical evidence of lesion recurrence until now. Conclusions The aggressive fibromatosis in larynx is a rare benign tumor-like pathologic change ,but it has obvious malignant tumor behavior. The patient may often manifest the sound change at first, or with swallow difficulty. Computed tomography and pathological features may be helpful for the diagnosis. Pathologic diagnosis is the final standard of diagnosing aggressive fibromatosis. As the recurrence rate of aggressive fibromatosis in larynx is high, the tumor should be treated as the low potential malignancy soft connective tissue tumor, and the surgery should be the chief therapic method. Surgery is the optimal of therapy, and wide excision or radical excision with clear margins is the best therapy to avoid the high risk of local recurrence.