Treatment and outcome of Ganglioneuroma and Ganglioneuroblastoma intermixed.

Boris Decarolis,Lothar Schweigerer,Barbara Hero,Ingo Mueller,Thomas Klingebiel,Thorsten Simon,Christian Vokuhl,Ivo Leuschner,Barbara Krug,Peter Kaatsch,Frank Berthold,Dietrich Von Schweinitz

doi:10.1186/s12885-016-2513-9

Abstract

BackgroundGanglioneuroma (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.MethodsClinical 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.ResultsOf 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.ConclusionsGN/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 registrationClinicalTrials.gov identifiers - NB97 (NCT00017225; registered June 6, 2001); NB2004 (NCT00410631; registered December 11, 2006)Electronic supplementary materialThe online version of this article (doi:10.1186/s12885-016-2513-9) contains supplementary material, which is available to authorized users.

Highlights

Ganglioneuroma (GN) and ganglioneuroblastoma intermixed (GNBI) are mature variants of neuroblastic tumors (NT)
We focused on localized stages as the typical presentations of GN and GNBI as metastatic disease is extremely rare in mature NT
In conclusion, clinical features and behavior are very similar for GN and GNBI and outcome is excellent for both

Summary

Introduction

Ganglioneuroma (GN) and ganglioneuroblastoma intermixed (GNBI) are mature variants of neuroblastic tumors (NT). Neuroblastic tumors (NT) are the most common extracranial solid tumors in childhood [1] and include neuroblastoma, ganglioneuroblastoma (nodular or intermixed), and ganglioneuroma They arise from the neural crest and range from immature, undifferentiated to mature, differentiated tumors. According to the International Neuroblastoma Pathology Classification (INPC) [2], ganglioneuroblastoma intermixed (GNBI) and ganglioneuroma (GN) represent the mature end of this range [3]. In this system, GN maturing has been defined as a “link” between GN and GNBI. Case reports on GNBI are rarer [15, 16]

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