Decarolis, Boris ORCID: 0000-0002-1607-0543, Simon, Thorsten, Krug, Barbara, Leuschner, Ivo, Vokuhl, Christian, Kaatsch, Peter, von Schweinitz, Dietrich, Klingebiel, Thomas, Mueller, Ingo, Schweigerer, Lothar, Berthold, Frank and Hero, Barbara (2016). Treatment and outcome of Ganglioneuroma and Ganglioneuroblastoma intermixed. BMC Cancer, 16. LONDON: BIOMED CENTRAL LTD. ISSN 1471-2407

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Abstract

Background: Ganglioneuroma (GN) and ganglioneuroblastoma intermixed (GNBI) are mature variants of neuroblastic tumors (NT). It is still discussed whether incomplete resection of GN/GNBI impairs the outcome of patients. Methods: Clinical characteristics and outcome of localized GN/GNBI were retrospectively compared to localized neuroblastoma (NB) and ganglioneuroblastoma-nodular (GNBN) registered in the German neuroblastoma trials between 2000 and 2010. Results: Of 808 consecutive localized NT, 162 (20 %) were classified as GN and 55 (7 %) as GNBI. GN/GNBI patients presented more often with stage 1 disease (68 % vs. 37 %, p < 0.001), less frequently with adrenal tumors (31 % vs. 43 %, p = 0.001) and positive mIBG-uptake (34 % vs. 90 %, p < 0.001), and had less often elevated urine catecholamine metabolites (homovanillic acid 39 % vs. 62 %, p < 0.001, vanillylmandelic acid 27 % vs. 64 %, p < 0.001). Median age at diagnosis increased with grade of differentiation (NB/GNBN: 9; GNBI: 61; GN-maturing: 71; GN-mature: 125 months, p < 0.001). Complete tumor resection was achieved at diagnosis in 70 % of 162 GN and 67 % of 55 GNBI, and after 4 to 32 months of observation in 4 GN (2 %) and 5 GNBI (9 %). Eleven patients received chemotherapy without substantial effect. Fifty-five residual tumors (42 GN, 13 GNBI) are currently under observation (median: 44 months). Five patients (3 GN, 2 GNBI) showed local progression; all had tumor residuals > 2 cm. No progression occurred after subtotal resection. Two patients died of treatment, none of tumor progression. Conclusions: GN/GNBI account for one quarter of localized NT and differ from immature tumors in their clinical features. Chemotherapy is not effective. Subtotal resection appears to be a sufficient treatment.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Decarolis, BorisUNSPECIFIEDorcid.org/0000-0002-1607-0543UNSPECIFIED
Simon, ThorstenUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Krug, BarbaraUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Leuschner, IvoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Vokuhl, ChristianUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kaatsch, PeterUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
von Schweinitz, DietrichUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Klingebiel, ThomasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Mueller, IngoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schweigerer, LotharUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Berthold, FrankUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hero, BarbaraUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
URN: urn:nbn:de:hbz:38-269513
DOI: 10.1186/s12885-016-2513-9
Journal or Publication Title: BMC Cancer
Volume: 16
Date: 2016
Publisher: BIOMED CENTRAL LTD
Place of Publication: LONDON
ISSN: 1471-2407
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
NEUROBLASTIC TUMORS; RETROPERITONEAL GANGLIONEUROMA; MALIGNANT-TRANSFORMATION; RISK NEUROBLASTOMA; MATURATION; RECOMMENDATIONS; CLASSIFICATION; TERMINOLOGY; REGRESSION; CHILDHOODMultiple languages
OncologyMultiple languages
Refereed: Yes
URI: http://kups.ub.uni-koeln.de/id/eprint/26951

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