Aim: This study was interested in extremity leiomyosarcoma with focus on clinical outcome after surgery with or without adjuvant therapy. Patients and Methods: A retrospective case series of all... Show moreAim: This study was interested in extremity leiomyosarcoma with focus on clinical outcome after surgery with or without adjuvant therapy. Patients and Methods: A retrospective case series of all patients with leiomyosarcoma, surgically treated between 2000 and 2015 and a minimum follow-up of 2 years, was drawn from institutional databases in Belgium and the Netherlands. Postoperative complications were reported with the Radiation Therapy Oncology Group (RTOG) and the Henderson classification. Results: Seventy-five patients were operated on, of whom 47 underwent (neo)adjuvant therapy. Infection was observed in 11 patients, seven associated with (neo)adjuvant radiotherapy. Dermatological complaints vvere observed in 26 patients, 10 associated with (neo)adjuvant radiotherapy. Overall survival was 60%. Local recurrence occurred in 11 (15%) patients. Conclusion: This study describes favourable clinical outcome following (neo)adjuvant radiotherapy. In the future, larger databases on leiomyosarcoma should enhance the power of these findings and define the benefits of adjuvant therapy in leiomyosarcoma. Show less
Groot, V. de; Verhelst, E.; Hogendoorn, P.C.W.; Keizer, R.J.W. de 2019
The aim of this thesis is to gain more insight in the molecular pathology of soft tissue sarcomas, especially leiomyosarcoma and myxoid liposarcoma. The group of soft tissue sarcomas... Show moreThe aim of this thesis is to gain more insight in the molecular pathology of soft tissue sarcomas, especially leiomyosarcoma and myxoid liposarcoma. The group of soft tissue sarcomas constitute a large number of different, mostly rare, neoplasms, each with their own histopathology, genetic profile and clinical features. Here we focus on the molecular profile of these two soft tissue tumours and performed studies to identify potential targets for specific therapeutic strategies. Leiomyosarcomas are characterized by a complex genetic profile which makes it difficult to identify targets for treatment. We here show that MED12 mutations are restricted to uterine smooth muscle tumours, and describe the occurrence of non-recurrent translocations in two cases of soft tissue leiomyosarcomas. Next, we show high expression of the Bcl family members in leiomyosarcomas, and using cell lines we could demonstrate that these proteins play a role in chemoresistance. Myxoid liposarcomas have a well-defined recurrent translocation. We show expression of the cancer-testis antigen NY-ESO-1 in a large proportion of tumours, providing options for immunotherapy. We present a novel, spontaneously immortalized myxoid liposarcoma cell line which was used for a largescale drug screen, resulting in the identification of a promising novel target. Show less
Lou, S.; Cleven, A.H.G.; Balluff, B.; Graaff, M. de; Kostine, M.; Briaire-de Bruijn, I.; ... ; Bovee, J.V.M.G. 2016
