Evaluation of the long term outcome of patients with extremity desmoids
Introduction
The first description of a desmoid tumour has been attributed to McFarlane1 when he removed an oval shaped tumour that felt exceedingly hard and was difficult to divide with the scalpel from the abdominal wall of a 26 year old female in January 1832. Desmoid tumours or aggressive fibromatosis are rare tumours making up about 3% of all soft tissue tumours.2 They are benign and arise from fascia and musculoaponeurotic tissues. From a clinical perspective, three different presentations exist: those that occur involving the abdominal wall in females of child bearing age, those that are associated with polyposis coli and extremity desmoids. Treatment of abdominal wall desmoids is best achieved by wide surgical excision with abdominal wall replacement using mesh to close the resultant defect.3 Management of mesenteric desmoids associated with polyposis coli can be either surgical or conservative, reserving the latter for lesions where the risk of injury to the superior mesenteric vessels is felt to be considerable on the basis of adequate preoperative investigation.4., 5. Conservative treatment of extremity desmoids may also be reasonable for lesions that would otherwise require amputation. However, surgery or radiotherapy or both are regarded as the treatment of choice for lesions at this site.6
A recent review of 22 published series including 780 patients6 indicated that local control of desmoids was better after radiotherapy or radiotherapy with surgery then surgery alone. Follow up of patients in many of these studies, however, was short and included abdominal desmoids. The aim of the study was to examine the long term outcome of patients with primary extremity desmoids and to compare patient episodes treated by surgery alone with those treated by radiotherapy either alone or combined with surgery at any stage of the patient's disease.
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Patients and methods
All patients who were treated for extremity desmoid tumours between 1959 and 1996 were identified through the Scottish Bone and Soft Tissue Tumour Registry. Only those followed up at an Oncology Clinic with an interest in these tumours at the Western Infirmary were included in the study. All patients followed were traced back to their first treatment episode at their original hospital. Pathology was reviewed by a single pathologist with an interest in soft tissue tumours. If the patient missed
Results
During the study period 42 patients with extremity desmoids—29 females and 13 males—were followed at the Oncology Clinic. Median age at presentation was 27 years (range 1–69 years). Nineteen patients presented with a painless mass while 17 had a mass with pain. Six patients presented with pain only, following which a mass was identified on clinical examination or imaging. Patient characteristics and site of presentation are shown in Table 1. Management of the primary lesion was undertaken by 37
Discussion
This study demonstrates a high relapse rate following both radiotherapy and surgery in the treatment of extremity desmoids. Median time to follow up was long at 130 months and although radiotherapy did not reduce the rate of relapse, it significantly prolonged the time to recurrence for these locally aggressive tumours. Excision was incomplete in the majority of patients and this had a significant impact on recurrence. The probability of relapse was not affected by whether treatment was for
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