Treatment of Aggressive Fibromatosis: the Experience of a Single Institution |
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| Authors: | M. El-Haddad M. El-Sebaie R. Ahmad E. Khalil M. Shahin R. Pant M. Memon A. Al-Hebshi Y. Khafaga M. Al-Shabanah A. Allam |
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| Affiliation: | 2. Department of Orthopedic Surgery, King Faisal Specialist Hospital, Riyadh, Saudi Arabia;3. Department of Medical Oncology, King Faisal Specialist Hospital, Riyadh, Saudi Arabia;1. Vitreoretinal Division, King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia;2. Department of Ophthalmology, King Saud University, Riyadh, Saudi Arabia;1. Department of Thoracic Surgery, Peking Union Medical College Hospital, CAMS & PUMC, Beijing, 100730, China;2. Department of Pathology, Peking Union Medical College Hospital, CAMS & PUMC, Beijing, 100730, China;1. Angiology and Vascular Surgery Department, Hospital Universitario de Getafe, Madrid, Spain;2. Research Department, Hospital Universitario de Getafe, Madrid, Spain;1. Steno Diabetes Center A/S, Gentofte, Denmark;2. Novo Nordisk A/S, Bagsværd, Denmark;3. King Abdul Aziz University Hospital, Jeddah, Saudi Arabia;4. EHU, Oran, Algeria;5. Algerian Ministry of Health, Algiers, Algeria;6. Novo Nordisk A/S, Algiers, Algeria;7. APDP, Lisbon, Portugal;8. Centre de Recherche Public de la Santé, Strassen, Luxembourg;9. Copenhagen University Hospital Gentofte, Department of Medicine, Denmark;1. Rheumatology and Rehabilitation Department, Faculty of Medicine, Cairo University, Cairo, Egypt;2. Rheumatology and Rehabilitation Department, Dr. Erfan and Bagedo General Hospital, Jeddah, Saudi Arabia;3. Radiology Department, Faculty of Medicine, Cairo University, Cairo, Egypt;4. Radiology Department, Dr. Erfan and Bagedo General Hospital, Jeddah, Saudi Arabia;5. Pediatric neurology Department, Dr. Erfan and Bagedo General Hospital, Jeddah, Saudi Arabia;6. Rheumatology and Rehabilitation Department, Suez Canal University, Ismailia, Egypt;7. Rheumatology Department, University of Twente, Enschede, The Netherlands |
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| Abstract: | AimsAggressive fibromatosis is a locally aggressive infiltrative low-grade tumour, although pathologically benign, and it does not metastasise, yet it can cause serious local distressing symptoms by virtue of local destruction and impairment of local function. The aim of this study was to emphasise the role of radiotherapy and adequate surgery in the treatment of fibromatosis in patients presenting with newly diagnosed or recurrent disease and to analyse our treatment results over 15 years for this rare tumour type.Materials and methodsFifty-four patients with confirmed diagnosis of aggressive fibromatosis treated at King Faisal Specialist Hospital between 1990 and 2006 were identified from our local cancer registry. Forty-seven patients had surgery: complete resection (R0) in 20 patients, incomplete surgery (R1/2) in 27 patients, and seven patients had biopsy only. Forty-five patients were treated with radiotherapy: 38 patients were treated with postoperative radiotherapy, three patients were treated with preoperative radiotherapy and four patients had radiotherapy as the only treatment. The radiotherapy dose ranged between 45 and 60 Gy (median 50.4 Gy). Three patients did not receive any form of treatment apart from biopsy, but were still included in the final analysis.ResultsFifty-two per cent (28/54 patients) of our patient population had tumour recurrence when first presented to King Faisal Specialist Hospital. The median age was 29.5 years (range 2–63 years). The most common site of involvement was the extremities (28 patients). Among the 54 patients (with primary and recurrent presentation) there were 10 local recurrences, all of which were within the original primary site. The 5-year progression-free survival and overall survival rates for the whole group were 75 and 95%, respectively. Univariate and multivariate Cox regression analysis showed that the depth of invasion significantly affected progression-free survival.ConclusionAggressive fibromatosis is effectively treated with surgery and postoperative radiotherapy. Patients first presenting with tumour recurrence may still have local tumour control comparable with newly diagnosed patients. |
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