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Table 3 Clinical course of patients who received cytotoxic treatment (CT) (n = 11)

From: Treatment and outcome of Ganglioneuroma and Ganglioneuroblastoma intermixed

Age at diagnosis Tumor localization Tumor volume Tumor stage Extent of surgery prior to chemotherapy Histology CT Response to CT Current status Remarks
11 – 18 yrs Abdominal 768 ml 3 Biopsy GN mature 2 x N5, 2 x N6, 5 x N7 SD SD Progression 18 months after end of maintenance therapy (N7)
5 – 11 yrs Abdominal 120 ml 3 Macroscopic residuals GN maturing 1 x N5, 1 x N6 SD VGPR Secondary surgery due to no response to chemotherapy
18 – 21 yrs Abdominal 72 ml 2a Macroscopic residuals GNBI 2 x N5, 2 x N6 SD VGPR Secondary surgery due to no response to chemotherapy
5 – 11 yrs Adrenal 44 ml 2a Macroscopic residuals GNBI 2 x N5, 2 x N6 SD CR Secondary surgery due to no response to chemotherapy
5 – 11 yrs Pelvic 102 ml 2a Macroscopic residuals GNBI 3 x N4, 1 x N5 PR LFU LFU after 18 months
18 mo – 5 yrs Adrenal 150 ml 3 Biopsy GNBI 2 x N5, 2 x N6 SD CR Secondary surgery due to no response to chemotherapy
18 mo – 5 yrs Cervical 87 ml 2a Biopsy GNBI 2 x N5, 2 x N6 SD VGPR Secondary surgery due to no response to chemotherapy
18 mo – 5 yrs Thoracic n.a. 3 Macroscopic residuals GNBI 4 x N5, 4 x N6, 4 x N7, 1 x N8, 1 x RA PR PR Progression of paraplegia 3 months after diagnosis
18 mo – 5 yrs Abdominal 240 ml 3 Biopsy GNBI 3 x N5, 3 x N6, 4 x N7, 9 x RA PR CR Secondary surgery due to poor response to chemotherapy
18 mo – 5 yrs Thoracic 39 ml 2a Biopsy GNBI 2 x N5, 2 x N6, 2 x N8, 1 x TE SD PD Progression 71 months after last cycle of chemo therapy
0 – 18 mo Adrenal 20 ml 1 Complete resection GNBI 2 x N5, 2 x N6 - Dead Death of chemotherapy-related pulmonary edema
  1. GNBI = ganglioneuroblastoma intermixed, GN = ganglioneuroma, n.a. = data not available, yrs = years, mo = months,
  2. CR = complete remission, VGPR = very good partial response, PR = partial response, SD = stable disease, PD = progressive disease, LFU = lost to follow-up,
  3. N5 = cisplatin/etopiside/vindesine, N6 = vincristine/dacarbacin/ifosfamide/doxorubicine, N7 = cyclophasphamide (oral), N8 = topotecan/cyclophasphamide/etoposide,
  4. TE = topotecan/etoposide