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International Orthopaedics

, Volume 19, Issue 6, pp 383–389 | Cite as

Surgical treatment of extra-abdominal desmoid tumours (aggressive fibromatoses)

  • S. Higaki
  • A. Tateishi
  • T. Ohno
  • S. Abe
  • K. Ogawa
  • T. Iijima
  • T. Kojima
Article

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.

Keywords

Local Excision Massive Haemorrhage Wide Margin Wide Excision Wide Local Excision 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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.

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Copyright information

© Springer-Verlag 1995

Authors and Affiliations

  • S. Higaki
    • 1
  • A. Tateishi
    • 1
  • T. Ohno
    • 1
  • S. Abe
    • 1
  • K. Ogawa
    • 1
  • T. Iijima
    • 2
  • T. Kojima
    • 2
  1. 1.Department of Orthopaedic SurgeryTeikyo University School of MedicineTokyoJapan
  2. 2.Department of Orthopaedic Surgery, Faculty of MedicineUniversity of TokyoTokyoJapan

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