Clinicopathological characteristics of ganglioneuroma and ganglioneuroblastoma: a report from the CCG and COG

Abstract

Background: The International Neuroblastoma Pathology Classification (INPC) was the first to clearly define prognostic subgroups in ganglioneuroma (GN) and ganglioneuroblastoma (GNB).

Procedure: Histopathology and tumor resectability of 552 GN/GNB cases from the Children's Cancer Group (CCG) and Children's Oncology Group (COG) neuroblastoma studies were reviewed. The results were analyzed along with clinical information and biological data of the cases.

Results: According to the INPC, 300 tumors were classified into the Favorable Histology (FH) group and 252 were into the Unfavorable Histology (UH) group. Tumors in the FH group included 43 ganglioneuroma-maturing (GN-M), 198 ganglioneuroblastoma-intermixed (GNB-I), and 59 ganglioneuroblastoma-nodular, favorable subset (GNB-N-FS), and were often (91%) resected completely by single or multiple surgical procedures. Patients with the FH tumors had an excellent prognosis with no tumor-related deaths. The UH group included ganglioneuroblastoma-nodular, unfavorable subset (GNB-N-US) tumors. Patients with the UH tumors had a high incidence (53%) of distant metastasis at the time of diagnosis, and their prognosis significantly depended on clinical stage (5-year EFS: 80.1% for non-stage 4 patients; 16.7% for stage 4 patients): Complete primary tumor resection was not beneficial to those GNB-N-US patients, regardless of whether metastasis was present or not. MYCN amplification was detected in four tumors in the FH group and six tumors in the UH group. The majority (160/191, 84%) of GN-M and GNB-I tumors had a diploid pattern determined by flow cytometry.

Conclusions: Stringent application of the INPC along with clinical staging was critical for prognostic evaluation of the patients with this group of tumors.

Figure 1

Kaplan-Meier survival curves for GNB-N-US (Ganglioneuroblastoma- Nodular, Unfavorable Subset) patients with non-stage 4 (stage 1, 2, or 3) disease. No significant difference in both expected event-free (A) and overall (B) survival rates was seen between patients with complete primary tumor resection (solid line) and with incomplete primary resection (interrupted line), p=0.2227 and 0.7176, respectively.

Figure 2

Kaplan-Meier survival curves for GNB-N-US (Ganglioneuroblastoma, Nodular Unfavorable Subset) patients with stage 4 disease. No significant difference in both event-free (A) and overall (B) survival rates was seen between patients with complete primary tumor resection (solid line) and incomplete primary resection (interrupted line), p=0.1068 and 0.5797, respectively.