A registry for soft tissue sarcoma and other soft tissue tumours in children, adolescents, and young adults: CWS-SoTiSaR

children, adolescents, and young adults with soft tissue sarcomas and tumours :

Rhabdomyosarcoma: embryonal rhabdomyosarcoma, alveolar rhabdomyosarcoma, rhabdomyosarkoma of other subtypes (not otherwise specified, NOS)

„RMS-like“-tumours: extraosseous Ewing’s Sarcoma, peripheral primitive neuroectodermal tumour, synovial sarcoma, undifferentiated sarcoma

"Non-RMS- like"-tumours: Angiomatoid fibrous histiocytoma, Alveolar soft part sarcoma, Clear cell sarcoma, Desmoplastic small and round cell tumour, Extraskeletal chondrosarcoma, Epitheloid sarcoma, embryonal sarcoma of the liver (should be treatetd as RMS), Endometrial stromal sarcoma, Congenital (infantile) fibrosarcoma, Fibrosarcoma, Extraosseous Giant cell tumour, Hamartoma, Inflammatory myofibroblastic tumour or inflammatory myofibroblastic sarcoma, Low grade fibromyxoid sarcoma, Leiomyosarcoma, Liposarcoma, Malignant fibrous histiocytoma (MFH), Myofibroblastic sarcoma, Malignant mesenchymoma, Malignant peripheral nerve sheath tumour (incl. eurofibrosarcoma& malig. schwannoma), Malignant rhabdoid tumour, Myxofibrosarcoma, Plexiform fibrohistiocytic tumour, Pigmented neuroectodermal tumour of childhood , Vascular sarcoma, such as Hemangioendothelioma, Hemangiopericytoma, Angiosarcoma

Other soft tissue tumours: Chordoma, Dermatofibrosarcoma protuberans, Myo-/ Fibromatoses including Juvenile nasopharyngeal angiofibroma (JNF), Gastrointestinal stromal tumour, Lipoblastoma, Pleuropulmonary blastoma

The establishment of the registry will ensure that epidemiological and clinical information about chil-dren, adolescents and young adults ≤21 yrs of age with different types of soft tissue tumours including extremely rare histiotypes (e.g. non-rhabdomyosarcomatous soft tissue sarcoma) and non malignant types (e.g. fibromatosis). The registry is the backbone project of the CWS Study group that will ensure an adequate data base for further clinical and laboratory research. By the means of data collection and validation, the CWS Study group can assess the quality of treatment and provide an advisory service. The registry will constitute a basis for innovative clinical phase-I, -II and -III trials prepared in coopera-tion with the European paediatric Soft Tissue sarcoma Group (EpSSG) and the DGOH/AIO for se-lected groups of patients (i.e. high risk RMS, primary resected synovial sarcoma or “adult type” soft tissue sarcoma). The feasibility of any cooperation however depends on the existence of a registry for standardised treated patients which encompasses all histiotypes and risk groups of STS. The registry will also provide a clinical data base for a sarcoma tissue repository and biological studies. The ability to correlate outcomes of prognosis with biological markers and treatment is critical. Centrally coordi-nated network will facilitate multidisciplinary research and data sharing. The resulting system can be a model for comprehensive research studying other rare diseases.

• Prospective registration pf all newly diagnozed patients with soft tissue tumours / sarcoma
• Collect information about incidence of different types of soft tissue tumours as a rare disease
• Collect prospectively information on treatment and outcome to determine whether a relationship exists between outcomes and specific interventions
• Assess the quality of treatment by the means of data collection, data check and an advisory service provided by the registry and the CWS reference centres
• Create a database for the reassessment of the present therapy stratification system and find new risk factors by linkage of bio-logical studies to long-term outcome
• Identify sarcoma specific surrogate endpoints
• Provide a basis for innovative clinical phase-I and -II and -III trials being prepared in cooperation with the European paediatric Soft Tissue sarcoma Group (EpSSG) and the Arbeitsgemein-schaft Internistische Onkologie (AIO) of the DGOH, which will include only selected groups of patients (i.e. high risk rhabdomy-osarcoma (RMS), primary resected synovial sarcoma and “adult type” soft tissue sarcoma). Their feasibility is depending on the existence of a registry for standardised treated patients with all types and risk groups of soft tissue sarcoma (STS).
• Provide a clinical data basis for a sarcoma tumour and tissue repository
• Facilitate the conduct of other clinical and laboratory-based sar-coma research.
• Serve as an information resource for sarcoma researchers, clini-cians and patients
• Conduct long-term follow up to assess late morbidities and qual-ity of life ( in cooperation with the late effects groups)
• Implement high-quality information systems by optimising the linkage between data from the registry, data from the clinical tri-als conducted by the CWS Group and data from biological stud-ies including comprehensive profiling (genome, transcriptome and proteome)
• Serve as a focal point for related registries in the countries coop-erating within the CWS Study Group (Austria, Poland, Sweden, Switzerland)

• Pathologically proven diagnosis of a soft tissue tumour (centrally reviewed), age less than 21 years of age (20 years and 364 days)*, available for long term follow up, written informed consent for data collection. * In case of rhabdomyosarcoma, patients older than 21 years of age can be included as well.
• Written informed consent

Coordination
Dr. med. Tobias Dantonello, Dr. med. Monika Sparber-Sauer, Dr. med. Monika Scheer
Klinikum Stuttgart - Olgahospital
Klinik für Kinder- und Jugendmedizin - Pädiatrie 5,
Studienzentrale CWS
Kriegsbergstrasse 62
70174 Stuttgart
Telefon +49 (711) 278 73870
Fax +49 (711) 278 72749
cws@olgahospital-stuttgart.de

Documentation

Simone Feuchtgruber, Erika Hallmen, Iris Veit-Friedrich
Klinikum Stuttgart - Olgahospital
Klinik f. Kinder-und Jugendmedizin, Pädiatrie 5,
Studienzentrale CWS
Kriegsbergstrasse 62
70174 Stuttgart
Telefon +49 (711) 278-73811/73875
Fax +49 (711) 278 72749
cws.dokumentation@olgahospital-stuttgart.de