Brecht, Ines B., Bremensdorfer, Claudia, Schneider, Dominik T., Fruehwald, Michael C., Offenmueller, Sonja, Mertens, Rolf, Vorwerk, Peter, Koscielniak, Ewa, Bielack, Stefan S., Benesch, Martin, Hero, Barbara, Graf, Norbert ORCID: 0000-0002-2248-323X, von Schweinitz, Dietrich and Kaatsch, Peter (2014). Rare Malignant Pediatric Tumors Registered in the German Childhood Cancer Registry 2001-2010. Pediatr. Blood Cancer, 61 (7). S. 1202 - 1210. HOBOKEN: WILEY. ISSN 1545-5017

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Abstract

Background Recurrence occurs in almost 50% of patients with intracranial ependymoma, and their outcome following recurrence is poor. Methods We retrospectively reviewed the medical records of 22 patients with intracranial ependymoma and subsequent relapse(s) (59 recurrences) treated at Children's Hospital Los Angeles or New York University between January 1997 and December 2012. Results Median duration of follow-up was 52 months (7-171 months). Median age at initial diagnosis was 4 years (0.3-19 years) with 8 patients younger than 3 years at presentation. Eleven patients had anaplastic and 11 cellular pathologies. Eighteen patients had infratentorial tumors at diagnosis and 3 (all infratentorial) had metastatic spinal cord involvement at presentation. Cerebrospinal fluid involvement was not identified at diagnosis or relapse. Median time to first recurrence was 16 months (1.3 to 115 months). The number of recurrences in each patient ranged from 1 to 9 (median = 2). Thirty-seven recurrences (63%) were detected asymptomatically by surveillance imaging. Fifteen recurrences (26%) arose outside the initial tumor site. Recurrences were treated by surgical resection (45), with irradiation (30), and with various oral chemotherapies (23) with (7) or without (16) conventional chemotherapy. The 5 and 10 year overall survival rates from first recurrence were 0.37 +/- 0.14 and 0.25 +/- 0.14. Conclusion Prolonged (5-10 year) survival from first relapse was noted in over one-quarter of our patients. It remains unclear whether early radiographic diagnosis, differing treatment modalities beyond radical surgical resection or possibly unrecognized biological differences contributed towards this prolonged survival. Pediatr Blood Cancer 2014;61:1195-1201. (c) 2014 Wiley Periodicals, Inc.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Brecht, Ines B.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Bremensdorfer, ClaudiaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schneider, Dominik T.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Fruehwald, Michael C.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Offenmueller, SonjaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Mertens, RolfUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Vorwerk, PeterUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Koscielniak, EwaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Bielack, Stefan S.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Benesch, MartinUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hero, BarbaraUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Graf, NorbertUNSPECIFIEDorcid.org/0000-0002-2248-323XUNSPECIFIED
von Schweinitz, DietrichUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kaatsch, PeterUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
URN: urn:nbn:de:hbz:38-435544
DOI: 10.1002/pbc.24997
Journal or Publication Title: Pediatr. Blood Cancer
Volume: 61
Number: 7
Page Range: S. 1202 - 1210
Date: 2014
Publisher: WILEY
Place of Publication: HOBOKEN
ISSN: 1545-5017
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
COOPERATIVE-STUDY GROUP; CHILDREN; ADOLESCENTS; CLASSIFICATION; CARCINOMA; CAREMultiple languages
Oncology; Hematology; PediatricsMultiple languages
URI: http://kups.ub.uni-koeln.de/id/eprint/43554

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