Nuchal aggressive fibromatosis in childhood: Two instructive case reports

Aggressive fibromatosis is a rare, benign tumour with a capacity for infilt ration of surrounding structures and a propensity for local recurrence. The cornerstone of therapy is surgery, with various other treatment modalities having ill-defined roles. Assessment of the efficacy of these intervention s is difficult. The natural history of the condition is variable and differ ent treatment modalities are often used concurrently. Childhood cases pose particular management problems because of their tendency to occur in the he ad and neck region and the potential for treatment-related morbidity. Two c hildren presented after surgery with recurrent disease threatening the airw ay. One remitted spontaneously and remains disease free at 20 years. The ot her achieved a complete remission with radiotherapy and toremifene. The rol e of non-surgical treatment, particularly radiotherapy, is reviewed.