Primary intracranial soft tissue sarcoma in children and adolescents: a cooperative analysis of the European CWS and HIT study groups
Purely intracranial soft tissue sarcomas (ISTS) are very rare among children. A retrospective database analysis of the Cooperative Weichteilsarkom Studiengruppe (CWS) and brain tumor (HIT) registries was conducted to describe treatment and long-term outcome of children and adolescents with ISTS. Nineteen patients from Germany, Austria and Switzerland were reported between 1988 and 2009. Median age at diagnosis was 9.7 years (range, 0.5–17.8). Central pathological review was performed in 17 patients. Eleven patients underwent a total and five a subtotal tumor resection. A biopsy was done in one patient. In two patients no data concerning extent of initial resection was available. Radiotherapy was performed in 15 patients (first-line, n = 11; following progression, n = 4). All but one patient received chemotherapy (first-line, n = 7, following progression, n = 5; first-line and following progression, n = 6). With a median follow-up of 5.8 years (range, 0.6–19.8) ten patients were alive in either first or second complete remission. Seven patients died due to relapse or progression and two were alive with progressive disease. Estimated progression-free and overall survival at 5 years were 47 % (±12 %) and 74 % (±10 %), respectively. About 50 % of patients with ISTS remain relapse-free after 5 years. Multimodality treatment including complete tumor resection and radio-/chemotherapy is required to achieve sustained tumor control in patients with ISTS. Early initiation of postoperative non-surgical treatment seems to be important to prevent recurrence. Due to the intracranial localization local therapy should follow the recommendations used in brain tumors rather than in soft tissue sarcomas, whereas chemotherapy should be guided by histological subtype.
KeywordsSoft tissue sarcomas Children Adolescents Intracranial Chemotherapy Radiotherapy
The authors thank Wibke Treulieb and Christine Lindow (HIT study centre Hamburg, Germany), Gabriele Pammer (HIT study centre Graz, Austria), Erika Hallmen, Iris Veit-Friedrich and Simone Feuchtgruber (CWS study centre Stuttgart, Germany) for excellent data management, Claudia Bremensdorfer for administrative support and Franz Quehenberger (Medical University of Graz) for statistical support. The HIT and CWS studies and the Reference Centre for Neuropathology at the University of Bonn are supported by Grants from the Deutsche Kinderkrebsstiftung (German Childhood Cancer Foundation). The Austrian HIT study centre is supported by the Steirische Kinderkrebshilfe.
Conflict of interest
All authors declare no conflict of interest.
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