gms | German Medical Science

Deutscher Kongress für Orthopädie und Unfallchirurgie (DKOU 2016)

25.10. - 28.10.2016, Berlin

Desmoid tumors – from extensive surgery to watchful waiting – Results of different treatment modalities in one country

Meeting Abstract

  • presenting/speaker Andreas Krieg - Universitätskinderspital (UKBB), Knochen- +Weichteiltumorzentrum der Universität Basel (KWUB), Basel, Switzerland
  • Christoph Kettelhack - Viszeralchirurgie, Universitätsspital Basel, Basel, Switzerland
  • Klaus-Arno Siebenrock - Inselspital Bern, Klinik für Orthopädische Chirurgie und Traumatologie, Bern, Switzerland
  • Frank M. Klenke - Inselspital, Universitätsspital Bern, Klinik für Orthopädische Chirurgie, Bern, Switzerland
  • Ulrich Exner - Orthopädie Zentrum Zürich, Zürich, Switzerland
  • Bruno Fuchs - Orthopädie, Universitätsklinik Balgrist, Zürich, Switzerland
  • Stephane Cherix - Orthopädie, Universitätsspital Lausanne, Lausanne, Switzerland
  • Fritz Hefti - Universitäts-Kinderspital beider Basel (UKBB), Abt. für Orthopädie, Knochen und Weichteiltumorzentrum der Universität (KWUB), Basel, Switzerland

Deutscher Kongress für Orthopädie und Unfallchirurgie (DKOU 2016). Berlin, 25.-28.10.2016. Düsseldorf: German Medical Science GMS Publishing House; 2016. DocWI51-1486

doi: 10.3205/16dkou370, urn:nbn:de:0183-16dkou3703

Veröffentlicht: 10. Oktober 2016

© 2016 Krieg et al.
Dieser Artikel ist ein Open-Access-Artikel und steht unter den Lizenzbedingungen der Creative Commons Attribution 4.0 License (Namensnennung). Lizenz-Angaben siehe http://creativecommons.org/licenses/by/4.0/.


Gliederung

Text

Objectives: Extraabdominal desmoid tumors (DT) are benign and rare with a persistent treatment dilemma due to their high recurrence rate and their heterogenous behaviour. In the past wide local excision was the usual treatment, however the risk of local recurrence was high (> 40%). The goal of the retrospective study is to evaluate the mid- and longterm results of extraabdominal desmoid tumors with respect to the different treatment modalities in Switzerland.

Methods: From 1970 through 2013 105 patients with DT were treated with surgery (n = 49) or surgery plus radiation (n = 16). Further treatment options in the three sarcoma centers were radiation alone (n = 9), systemic therapy (n = 13) or watchful waiting (n = 18). Patient demographics, tumor site, histology, history of previous recurrences, as well as follow-up status with recurrence rates of the different treatment modalities were recorded retrospectively

Results and Conclusion: There is a mean follow-up of 8.2 years in the current study. All patients presented with tumors located at the extremities in 51%, pelvis in 6%, trunk in 17% and at the abdominal (15%) or thoracic (11%) wall. By immunohistochemistry, 86% of patients in this study were positive for beta-catenin. Overall 49 % (n = 24) of DT patients developed a recurrence after primary surgical treatment (n = 49). In the surgical plus radiation group 38% presented a recurrent DT. The overall recurrence rate in the surgery and surgery plus radiation group was 46%, 36% in patients with wide margins, 50 % after marginal resection and 80% after intralesional resection. In the group of patients who received radiation alone, all patients presented stable or regredient disease. The patients with systemic therapy showed in 33% progression and required further treatment. In the watchful waiting group we observed 67% stable diseases, 21 % spontaneous regressions and 12% progression.

The results of our observation group could encourage us to wait with careful observation instead of resecting primary DT. Beside surgery, radiation is an additional treatment option for better local control. The goal of any medical therapies - stabilization of the disease - has been achieved in a similiar rate than with alternative treatment options as surgery plus radiation. The results of this study should therefore be the basis for clear treatment guidelines for patients with DT. In a second stage, a prospective study should be carried out in order to evaluate the adequacy of these strategies.