title: Primary intracranial soft tissue sarcoma in children and adolescents: a cooperative analysis of the European CWS and HIT study groups.
abstract: 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.
authors.affiliation: Division of Pediatric Hematology and Oncology, Department of Pediatrics and Adolescent Medicine, Medical University of Graz, Auenbruggerplatz 38, 8036 Graz, Austria. martin.benesch@klinikum-graz.at
authors.forename: Martin
authors.initials: M
authors.lastname: Benesch
authors.forename: André O
authors.initials: AO
authors.lastname: von Bueren
authors.forename: Tobias
authors.initials: T
authors.lastname: Dantonello
authors.forename: Katja
authors.initials: K
authors.lastname: von Hoff
authors.forename: Torsten
authors.initials: T
authors.lastname: Pietsch
authors.forename: Ivo
authors.initials: I
authors.lastname: Leuschner
authors.forename: Alexander
authors.initials: A
authors.lastname: Claviez
authors.forename: Uta
authors.initials: U
authors.lastname: Bierbach
authors.forename: Gabriele
authors.initials: G
authors.lastname: Kropshofer
authors.forename: Rudolf
authors.initials: R
authors.lastname: Korinthenberg
authors.forename: Norbert
authors.initials: N
authors.lastname: Graf
authors.forename: Meinolf
authors.initials: M
authors.lastname: Suttorp
authors.forename: Rolf Dieter
authors.initials: RD
authors.lastname: Kortmann
authors.forename: Carsten
authors.initials: C
authors.lastname: Friedrich
authors.forename: Nicolas
authors.initials: N
authors.lastname: von der Weid
authors.forename: Peter
authors.initials: P
authors.lastname: Kaatsch
authors.forename: Thomas
authors.initials: T
authors.lastname: Klingebiel
authors.forename: Ewa
authors.initials: E
authors.lastname: Koscielniak
authors.forename: Stefan
authors.initials: S
authors.lastname: Rutkowski
mesh_terms: D000293:Adolescent; D001932:Brain Neoplasms / Q000134:cerebrospinal fluid / Q000175:diagnosis / Q000628:therapy; D002648:Child; D018572:Disease-Free Survival; D005060:Europe; D005260:Female; D006801:Humans; D007391:International Cooperation; D008137:Longitudinal Studies; D008297:Male; D012189:Retrospective Studies; D012509:Sarcoma / Q000134:cerebrospinal fluid / Q000175:diagnosis / Q000628:therapy; D016896:Treatment Outcome