Universität zu Köln

Meany, Holly J., London, Wendy B., Ambros, Peter F., Matthay, Katherine K., Monclair, Tom, Simon, Thorsten, Garaventa, Alberto ORCID: 0000-0002-5368-6363, Berthold, Frank, Nakagawara, Akira, Cohn, Susan L., Pearson, Andrew D. J. and Park, Julie R. (2014). Significance of Clinical and Biologic Features in Stage 3 Neuroblastoma: A Report from the International Neuroblastoma Risk Group Project. Pediatr. Blood Cancer, 61 (11). S. 1932 - 1940. HOBOKEN: WILEY. ISSN 1545-5017

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Abstract

BackgroundInternational Neuroblastoma Staging System (INSS) Stage 3 neuroblastoma is a heterogeneous disease. Data from the International Neuroblastoma Risk Group (INRG) database were analyzed to define patient and tumor characteristics predictive of outcome. ProcedureOf 8,800 patients in the INRG database, 1,483 with INSS Stage 3 neuroblastoma and complete follow-up data were analyzed. Secondary analysis was performed in 1,013 patients (68%) with MYCN-non-amplified (NA) tumors. Significant prognostic factors were identified via log-rank test comparisons of survival curves. Multivariable Cox proportional hazards regression model was used to identify factors independently predictive of event-free survival (EFS). ResultsAge at diagnosis (P<0.0001), tumor MYCN status (P<0.0001), and poorly differentiating/undifferentiated histology (P=0.03) were independent predictors of EFS. Compared to other Stage 3 subgroups, outcome was inferior for patients 547 days with MYCN-NA neuroblastoma (P<0.0001), and within this cohort, serum ferritin 96ng/ml was associated with inferior EFS (P=0.02). For patients <547 days of age with MYCN-NA tumors, serum ferritin levels were prognostic of overall survival (OS) (P=0.04) and chromosome 11q aberration was prognostic of EFS (P=0.03). ConclusionsAmong patients with INSS Stage 3 neuroblastoma patients, age at diagnosis, MYCN status and histology predict outcome. Patients <547 days of age with MYCN-NA tumors that lack chromosome 11q aberrations or those with serum ferritin <96ng/ml have excellent prognosis and should be considered for therapy reduction. Prospective clinical trials are needed to identify optimal therapy for those patients 547 days of age with undifferentiated histology or elevated serum ferritin. Pediatr Blood Cancer 2014;61:1932-1939. (c) 2014 Wiley Periodicals, Inc.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Meany, Holly J. UNSPECIFIED UNSPECIFIED UNSPECIFIED London, Wendy B. UNSPECIFIED UNSPECIFIED UNSPECIFIED Ambros, Peter F. UNSPECIFIED UNSPECIFIED UNSPECIFIED Matthay, Katherine K. UNSPECIFIED UNSPECIFIED UNSPECIFIED Monclair, Tom UNSPECIFIED UNSPECIFIED UNSPECIFIED Simon, Thorsten UNSPECIFIED UNSPECIFIED UNSPECIFIED Garaventa, Alberto UNSPECIFIED orcid.org/0000-0002-5368-6363 UNSPECIFIED Berthold, Frank UNSPECIFIED UNSPECIFIED UNSPECIFIED Nakagawara, Akira UNSPECIFIED UNSPECIFIED UNSPECIFIED Cohn, Susan L. UNSPECIFIED UNSPECIFIED UNSPECIFIED Pearson, Andrew D. J. UNSPECIFIED UNSPECIFIED UNSPECIFIED Park, Julie R. UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-425342
DOI:	10.1002/pbc.25134
Journal or Publication Title:	Pediatr. Blood Cancer
Volume:	61
Number:	11
Page Range:	S. 1932 - 1940
Date:	2014
Publisher:	WILEY
Place of Publication:	HOBOKEN
ISSN:	1545-5017
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language CHILDRENS ONCOLOGY GROUP; PATHOLOGY CLASSIFICATION; MYCN AMPLIFICATION; N-MYC; 13-CIS-RETINOIC ACID; PROGNOSTIC IMPACT; CHROMOSOME 1P; CANCER GROUP; TUMORS; AGE Multiple languages Oncology; Hematology; Pediatrics Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/42534