Universität zu Köln

Berthold, Frank ORCID: 0000-0002-7613-1723, Spix, Claudia, Kaatsch, Peter and Lampert, Fritz (2017). Incidence, Survival, and Treatment of Localized and Metastatic Neuroblastoma in Germany 1979-2015. Pediatr. Drugs, 19 (6). S. 577 - 594. NORTHCOTE: ADIS INT LTD. ISSN 1179-2019

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Abstract

Background A comprehensive clinical long-term survey over the complete spectrum of neuroblatoma disease is lacking in the literature. Objective Our objective was to describe the incidence, risk profiles, therapies, and outcomes for the total cohort of German patients with neuroblastoma including all clinical stages and risk groups. Methods Epidemiological, clinical, and outcome data of neuroblastoma patients who participated in one of the six consecutive national trials between 1979 and 2015 were analyzed retrospectively. Results Of all German neuroblastoma patients known to the national childhood cancer registry, ninety seven percent enrolled in one of the trials. The absolute neuroblastoma rate has increased slightly, whereas the median age at diagnosis has decreased. Except for the screening period (1995-2000), the risk factors lactate dehydrogenase (LDH), ferritin, chromosome 1p, and the MYCN oncogene have remained largely constant, with the exception of an increase in MYCN amplification at stage 4 for those aged C18 months between trials NB97 (27%) and NB2004 (35%). The 10-year overall survival increased in patients with stage 1-3 neuroblastoma from 83 to 91%, for stage 4S from 80 to 85%, and for stage 4 aged C18 months from 2 to 38%. The fraction of patients in stages 1-3 who never received chemotherapy (neither for frontline nor at recurrence) increased from 35 to 60%. The proportion of macroscopically complete surgical resections of the primary tumor decreased for the total population as well as for patients with stage 4 aged C18 months. The impact of chemotherapy response on the outcome was trial dependent. The overall proportion of toxic death during the time of the protocol therapy was 6% for stage 4 patients aged C18 months and 2% for low-/intermediate-risk patients. The most frequently reported late sequelae in stage 4 patients aged C18 months were renal dysfunctions, hypothyroidism, major hearing impairment, and second malignancies. Conclusion The body of data for incidences, risk profiles, and survival rates from this survey of more than 37 years provides a useful perspective for future studies on neuroblastoma sub-cohorts.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Berthold, Frank UNSPECIFIED orcid.org/0000-0002-7613-1723 UNSPECIFIED Spix, Claudia UNSPECIFIED UNSPECIFIED UNSPECIFIED Kaatsch, Peter UNSPECIFIED UNSPECIFIED UNSPECIFIED Lampert, Fritz UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-210500
DOI:	10.1007/s40272-017-0251-3
Journal or Publication Title:	Pediatr. Drugs
Volume:	19
Number:	6
Page Range:	S. 577 - 594
Date:	2017
Publisher:	ADIS INT LTD
Place of Publication:	NORTHCOTE
ISSN:	1179-2019
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language HIGH-RISK NEUROBLASTOMA; STEM-CELL TRANSPLANTATION; HIGH-DOSE CHEMOTHERAPY; ONCOLOGY-GROUP; STAGE-4 NEUROBLASTOMA; INDUCTION CHEMOTHERAPY; CHILDREN; CANCER; THERAPY; IMMUNOTHERAPY Multiple languages Pediatrics; Pharmacology & Pharmacy Multiple languages
Refereed:	Yes
URI:	http://kups.ub.uni-koeln.de/id/eprint/21050