[Sporadical extraperitoneal desmoid tumors--review and report on 4 cases]

Desmoid tumors are very rare mesenchymal tumors with a partially aggressive growth pattern and high relapse rates. Without metastases, they are classified as semi-malignant tumours. The treatment of this neoplasia is discussed controversially. Up to now, there exists no broad consensus on the treatment regimes. Traditionally, the primary radical surgical resection is often preferred. This might be combined with an (neo-)adjuvant radiation therapy. Recently an increasing importance of radiotherapy has been described. Several studies favour radiotherapy as the single form of treatment. Furthermore several medication regimes have been described in the literature.

We report the follow-up of 4 patients who were treated for desmoid tumours. In 2 cases surgery was the primary treatment. After R0-resection and adjuvant radiation therapy, one of the patients developed 7 relapses. In another patient, 5 relapses were observed after surgical therapy.

Therapeutic guidelines with broad acceptance do not exist up to now due to missing data of prospective studies with sufficient case numbers. In our opinion wide surgical resection should be performed in primary occurring cases with resectable tumours. On account of the previous experiences, it can be concluded that therapy of primary non respectable or recurrent desmoid tumours is not only a domain of a surgical approach. At present an adequate treatment should be planned interdisciplinary by surgeons, radiation therapists and oncologists. For generally accepted guidelines for this rare disease, general therapeutic options based on prospectively randomised studies have to be elaborated.