The Desmoid Tumor Research Foundation

July 2019

Extra-abdominal desmoid tumours – further evidence for the watchful waiting policy

Author Information

Krieg AH(1), Wirth C(1), Lenze U(1), Kettelhack C(2), Coslovsky M(3), Baumhoer D(4), Klenke FM(5), Siebenrock KA(5), Exner GU(6), Bode-Lesniewska B(7), Fuchs B(8), Cherix S(9), Hefti F(1).

(1)Bone and Soft Tissue Tumour Centre of the Univ of Basel (KWUB), Basel Switzerland / Paediatric Orthopaedic Dept, Univ Children's Hospital Basel (UKBB), Basel, Switzerland. (2)KWUB, Basel Switzerland / Dept of Surgery, Univ Hospital Basel, Switzerland. (3)Dept for Clinical Research, Clinical Trial Unit, Univ of Basel, Switzerland. (4)KWUB, Basel Switzerland / Bone Tumour Reference Centre at the Institute of Pathology, Univ Hospital Basel, Switzerland. 5)Dept of Orthopaedic Surgery, Inselspital, Bern Univ Hospital, Bern, Switzerland. (6)Orthopaedic Centre Zurich, Hospital Hirslanden im Park, Zurich, Switzerland. (7)Institute of Pathology and Molecular Pathology, Univ Hospital Zurich, Switzerland. (8)Orthopaedic Dept, Hospital Winterthur, Switzerland. (9)Centre des Sarcomes, Service d'Orthopedie et de Traumatologie, Centre Hospitalier Universitaire Vaudois (CHUV), Lausanne, Switzerland.

Publication

Swiss Med Wkly

Summary

The goal of this retrospective study was to evaluate the results of different treatment modalities for extra-abdominal desmoid tumours at four sarcoma treatment centres. The mean follow-up time for the 96 patients included in the study (63.5% female; mean age 38.9 years) was 8.4 years (2.0-40.5 years). The initial treatments were surgery (n = 44), surgery with radiation (n = 16), watchful waiting (n = 15), radiation only (n = 9), or systemic treatment (n = 12). The local recurrence rate was 45.5% in patients with primary surgical treatment and 37.5% following surgery combined with irradiation. Patients who were treated with radiation alone showed regressive (33.3%) or stable disease (66.6%). Systemic treatment alone resulted in disease progression in 41.7% of our patients. In the watchful waiting group, 73.3% showed stable disease, 20.0% showed spontaneous regression, and 6.7% showed progression after a mean follow-up of 4.1 years (2.0-11.5 years). Our results suggest that a watchful waiting approach should be the first line treatment in asymptomatic desmoid tumours. However, radiation can help improve local control rates in patients who have undergone surgery. Progression and local recurrence rates following systemic treatment were comparable to those observed in surgery combined with radiation.

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