Summary
Extra-abdominal desmoid tumours (EADT) are benign lesions but difficult to cure because of their infiltrative nature and tendency to recur. Among many treatments recommended in the past, wide excision has been successful, even in difficult cases. We have analyzed retrospectively 41 cases of histologically confirmed EADTs. A total of 98 operations were performed on these patients: 29 wide excisions on 22 patients, 52 intra-lesional excisions with wide margins on 16 patients, and 17 incomplete excisions on 3 patients. One patient, with intra-pelvic lesions, died of a massive haemorrhage 3 days after surgery. Forty patients were followed from between 3 and 29 years. One, who had a multicentric EADT for 21 years, died from the disease. The significant factors concerning local recurrence after wide procedures were an unsatisfactory initial wide local excision, disease affecting 4 or more muscles and the invasive nature of the recurrences. We recommend wide local excision of these tumours in all anatomical areas that allow this procedure. When major nerves and vessels are involved, we recommend an intralesional excision with wide margins in order to preserve limb function. Radiation therapy should be confined to cases in which wide local procedures are not feasible. Overall, 37 of our patients (90%) were cured of the disease, 2 had their disease controlled, and 2 died.
Résumé
Le fibrome desmoïde extra-abdominal (FDEA) est une lésion bénigne. Cependant, elle est difficile à traiter en raison de sa nature infiltrative et de sa tendance à la recidive Parmi les traitements recommandés autrefois, la grande excision était une méthode sûre même dans les cas difficiles. Rétrospectivement, nous avons analysé 41 cas confirmés histologiquement de FDEA. Un total de 98 opérations ont été pratiquées chez ces patients. Nous avons fait 29 grandes excisions sur 22 patients, 52 excisions intra-lésionelles avec de grandes marges sur 16 patients et 17 excisions incomplètes sur 3 patients. Un patient atteint d'une lesion intra-pelvienne est décédé trois jours après l'opération. On a observé 40 patients pendant une période de 3 à 29 ans. Un de ces patients atteint de FDEA multicentrique, suivi 21 ans, est décédé de la maladie. Les principaux facteurs contribuant à la recurrence locale sont les suivants: (1) La qualité de l'excision initiale, (2) La maladie affectant 4 muscles ou plus, (3) La nature invasive des récidives du FDEA. Nous recommandons la grande excision locale de ces tumeurs dans toutes les régions anatomiques le permettant. Sinon, lorsque des nerfs ou vaisseaux importants sont présents, nous recommandons l'excision intra-lésionelle avec une grande marge afin de préserver le fonctionnement des membres (inférieures + supérieurs); la radio-thérapie devrait être utilisée seulement si les grandes excisions ne peuvent pas être réalisées. Globalement, 37 patients (90%) ont pu être guéris de cette maladie. Deux patients ont vu leur maladie contrôlée, et 2 autres patients sont décédés comme mentionné ci-dessus.
Similar content being viewed by others
References
Abramowitz D, Zornooza J, Ayala AG et al. (1983) Soft tissue desmoid tumours: radiographic bone changes. Radiology 146: 11–13
Barber HM, Galasco CSB, Woods CG (1973) Multicentric extra-abdominal desmoid tumours: a report of two cases. J Bone Joint Surg [Br] 55: 858–863
Cole NM and Guiss LW (1969) Extra-abdominal desmoid tumours. Arch Surg 98: 530–533
Dahn I, Jonsson N, Lundh G (1963) Desmoid tumours. A series of 33 cases. Acta Chir Scand 126: 305–314
Das Gupta TK, Brasfield RD, O'Hara J (1969) Extra-abdominal desmoids: A clinicopathological study. Ann Surg 170: 109–121
Dominguez-Malagon HR, Alfeiran-Ruiz A, Chavarria-Xicotencatl P et al. (1992) Clinical cellular effects of cholchicine in fibromatosis. Cancer 69: 2478–2483
Enneking WF (1983) Musculoskeletal Tumor Surgery. Churchill Livingston, New York, pp 760–772
Enzinger FM, Shiraki M (1967) Musculo-aponeurotic fibromatosis of the shoulder girdle (extra-abdominal desmoid), analysis of 30 cases followed up for 10 or more years. Cancer 21: 1133–1140
Enzinger, Weiss (1988) Soft tissue tumours, 2nd edn. Mosby, St Louis, pp 145–163
Hunt RTN, Morgan HC, Ackerman LV (1960) Principles in the management of extra-abdominal desmoids. Cancer 13: 825–836
Kiel KD, Suit HD (1984) Radiation therapy in the treatment of aggressive fibromatosis (desmoid tumour) Cancer 54: 2051–2055
Kinzbrunner B, Ritter S, Domingo J et al. (1983) Remission of rapidly growing desmoid tumour after tamoxifen therapy. Cancer 52: 2201–2204
Khorsand J, Karakousis CP (1985) Desmoid tumours and their management. Am J Surg 149: 215–218
Kofoed H, Kamby C, Agnostaki L (1985) Aggressive fibromatosis. Surg Gynaecol Obstet 160: 124–127
McDougall A, McGarrity G (1979) Extra-abdominal desmoid tumours. J Bone Joint Surg [Br] 61: 373–377
Musgrove JE, McDonald JR (1948) Extra-abdominal desmoid tumours: Their differential diagnosis and treatment. Arch Pathol 45: 513–540
Nichols RW (1923) Desmoid tumours. A report of 31 cases. Arch Surg 7: 227–236
Patel SR, Evans HL, Benjamin R (1993) Combination chemotherapy in adult desmoid tumours. Cancer 72: 3244–3247
Phelan JT, Grace JT, Pickeren JW (1968) Juvenile fibromatosis. Surgery 64: 492–497
Pettit VD, Chamness JT, Ackerman LV (1954) Fibromatosis and fibrosarcoma following irradiation therapy. Cancer 7: 149–158
Rock MG, Pritchard DJ, Reiman HM et al. (1984) Extra-abdominal desmoid tumours. J Bone Joint Surg [Am] 66: 1369–1374
Stout AP (1961) The fibromatoses. Clin Orthop 19: 11–18
Wara WM, Phillips TL, Hill DR et al. (1977) Desmoid tumours — Treatment and prognosis. Radiology 124: 225–226
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Higaki, S., Tateishi, A., Ohno, T. et al. Surgical treatment of extra-abdominal desmoid tumours (aggressive fibromatoses). International Orthopaedics 19, 383–389 (1995). https://doi.org/10.1007/BF00178355
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00178355