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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