Table 3.
Clinical course of patients who received cytotoxic treatment (CT) (n = 11)
| 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 |
GNBI = ganglioneuroblastoma intermixed, GN = ganglioneuroma, n.a. = data not available, yrs = years, mo = months,
CR = complete remission, VGPR = very good partial response, PR = partial response, SD = stable disease, PD = progressive disease, LFU = lost to follow-up,
N5 = cisplatin/etopiside/vindesine, N6 = vincristine/dacarbacin/ifosfamide/doxorubicine, N7 = cyclophasphamide (oral), N8 = topotecan/cyclophasphamide/etoposide,
TE = topotecan/etoposide