Universität zu Köln

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:	Creators Email ORCID ORCID Put Code Decarolis, Boris UNSPECIFIED orcid.org/0000-0002-1607-0543 UNSPECIFIED Simon, Thorsten UNSPECIFIED UNSPECIFIED UNSPECIFIED Krug, Barbara UNSPECIFIED UNSPECIFIED UNSPECIFIED Leuschner, Ivo UNSPECIFIED UNSPECIFIED UNSPECIFIED Vokuhl, Christian UNSPECIFIED UNSPECIFIED UNSPECIFIED Kaatsch, Peter UNSPECIFIED UNSPECIFIED UNSPECIFIED von Schweinitz, Dietrich UNSPECIFIED UNSPECIFIED UNSPECIFIED Klingebiel, Thomas UNSPECIFIED UNSPECIFIED UNSPECIFIED Mueller, Ingo UNSPECIFIED UNSPECIFIED UNSPECIFIED Schweigerer, Lothar UNSPECIFIED UNSPECIFIED UNSPECIFIED Berthold, Frank UNSPECIFIED UNSPECIFIED UNSPECIFIED Hero, Barbara UNSPECIFIED UNSPECIFIED UNSPECIFIED
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:	Keywords Language NEUROBLASTIC TUMORS; RETROPERITONEAL GANGLIONEUROMA; MALIGNANT-TRANSFORMATION; RISK NEUROBLASTOMA; MATURATION; RECOMMENDATIONS; CLASSIFICATION; TERMINOLOGY; REGRESSION; CHILDHOOD Multiple languages Oncology Multiple languages
Refereed:	Yes
URI:	http://kups.ub.uni-koeln.de/id/eprint/26951