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Treatment and outcome of Ganglioneuroma and Ganglioneuroblastoma intermixed

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Treatment and outcome of Ganglioneuroma and Ganglioneuroblastoma intermixed. / Decarolis, Boris; Simon, Thorsten; Krug, Barbara; Leuschner, Ivo; Vokuhl, Christian; Kaatsch, Peter; von Schweinitz, Dietrich; Klingebiel, Thomas; Müller, Ingo; Schweigerer, Lothar; Berthold, Frank; Hero, Barbara.

In: BMC CANCER, Vol. 16, 27.07.2016, p. 542.

Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review

Harvard

Decarolis, B, Simon, T, Krug, B, Leuschner, I, Vokuhl, C, Kaatsch, P, von Schweinitz, D, Klingebiel, T, Müller, I, Schweigerer, L, Berthold, F & Hero, B 2016, 'Treatment and outcome of Ganglioneuroma and Ganglioneuroblastoma intermixed', BMC CANCER, vol. 16, pp. 542. https://doi.org/10.1186/s12885-016-2513-9

APA

Decarolis, B., Simon, T., Krug, B., Leuschner, I., Vokuhl, C., Kaatsch, P., von Schweinitz, D., Klingebiel, T., Müller, I., Schweigerer, L., Berthold, F., & Hero, B. (2016). Treatment and outcome of Ganglioneuroma and Ganglioneuroblastoma intermixed. BMC CANCER, 16, 542. https://doi.org/10.1186/s12885-016-2513-9

Vancouver

Decarolis B, Simon T, Krug B, Leuschner I, Vokuhl C, Kaatsch P et al. Treatment and outcome of Ganglioneuroma and Ganglioneuroblastoma intermixed. BMC CANCER. 2016 Jul 27;16:542. https://doi.org/10.1186/s12885-016-2513-9

Bibtex

@article{318b405820c040fea5a8039bb4e289c1,
title = "Treatment and outcome of Ganglioneuroma and Ganglioneuroblastoma intermixed",
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.TRIAL REGISTRATION: ClinicalTrials.gov identifiers - NB97 (NCT00017225; registered June 6, 2001); NB2004 (NCT00410631; registered December 11, 2006).",
keywords = "Journal Article",
author = "Boris Decarolis and Thorsten Simon and Barbara Krug and Ivo Leuschner and Christian Vokuhl and Peter Kaatsch and {von Schweinitz}, Dietrich and Thomas Klingebiel and Ingo M{\"u}ller and Lothar Schweigerer and Frank Berthold and Barbara Hero",
year = "2016",
month = jul,
day = "27",
doi = "10.1186/s12885-016-2513-9",
language = "English",
volume = "16",
pages = "542",
journal = "BMC CANCER",
issn = "1471-2407",
publisher = "BioMed Central Ltd.",

}

RIS

TY - JOUR

T1 - Treatment and outcome of Ganglioneuroma and Ganglioneuroblastoma intermixed

AU - Decarolis, Boris

AU - Simon, Thorsten

AU - Krug, Barbara

AU - Leuschner, Ivo

AU - Vokuhl, Christian

AU - Kaatsch, Peter

AU - von Schweinitz, Dietrich

AU - Klingebiel, Thomas

AU - Müller, Ingo

AU - Schweigerer, Lothar

AU - Berthold, Frank

AU - Hero, Barbara

PY - 2016/7/27

Y1 - 2016/7/27

N2 - 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.TRIAL REGISTRATION: ClinicalTrials.gov identifiers - NB97 (NCT00017225; registered June 6, 2001); NB2004 (NCT00410631; registered December 11, 2006).

AB - 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.TRIAL REGISTRATION: ClinicalTrials.gov identifiers - NB97 (NCT00017225; registered June 6, 2001); NB2004 (NCT00410631; registered December 11, 2006).

KW - Journal Article

U2 - 10.1186/s12885-016-2513-9

DO - 10.1186/s12885-016-2513-9

M3 - SCORING: Journal article

C2 - 27465021

VL - 16

SP - 542

JO - BMC CANCER

JF - BMC CANCER

SN - 1471-2407

ER -