Treatment and outcome of Ganglioneuroma and Ganglioneuroblastoma intermixed

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

doi:10.1186/s12885-016-2513-9

BMC Cancer

Table 2 Clinical course of patients with delayed surgery (> 3 months after diagnosis) (n = 22)

From: Treatment and outcome of Ganglioneuroma and Ganglioneuroblastoma intermixed

Age at diagnosis	Tumor localization	Tumor volume	Extent of primary surgery	Histology	Chemotherapy	Time from diagnosis to delayed surgery	Extent of secondary surgery	Current status	Reason for delayed surgery
18 mo – 5 yrs	Pelvic	179 ml	Macroscopic residuals	GN mature	None	32 mo	Complete resection	CR	Suspected Progression
11 – 18 yrs	Pelvic	680 ml	Macroscopic residuals	GN maturing	None	8 mo	Macroscopic residuals	PR	Progression before delayed surgery
11 – 18 yrs	Abdominal	n.a.	Biopsy	GN maturing	None	4 mo	Complete resection	CR	Planned surgery
5 – 11 yrs	Thoracic	n.a.	Macroscopic residuals	GN maturing	None	4 mo	Macroscopic residual	PR	Planned surgery
5 – 11 yrs	Thoracic	168 ml	Macroscopic residuals	GN maturing	None	6 mo	Macroscopic residuals	LFU	First surgery not in Germany
5 – 11 yrs	Abdominal	20 ml	Biopsy	GN maturing	None	9 mo	Near -complete resection	CR	Suspected Progression
5 – 11 yrs	Abdominal	120 ml	Macroscopic residuals	GN maturing	2 cycles	4 mo	Macroscopic residual	VGPR	No response to chemotherapy
5 – 11 yrs	Adrenal	56 ml	Macroscopic residuals	GN maturing	None	5 mo	Macroscopic residuals	VGPR	Planned surgery
18 mo – 5 yrs	Abdominal	90 ml	Biopsy	GN maturing	None	47 mo	Macroscopic residuals	VGPR	Progression before delayed surgery
18 mo – 5 yrs	Abdominal	282 ml	Biopsy	GN maturing	None	9 mo	Complete resection	CR	Suspected Progression
18 mo – 5 yrs	Thoracic	16 ml	Biopsy	GN maturing	None	6 mo	Macroscopic residual	VGPR	Suspected Progression
0 – 18 mo	Thoracic	26 ml	Biopsy	GN maturing	None	21 mo	Macroscopic residuals	PR	Suspected Progression
18 – 21 yrs	Abdominal	72 ml	Macroscopic residuals	GNBI	4 cycles	11 mo	Biopsy	VGPR	No response to chemotherapy
5 – 11 yrs	Thoracic	11 ml	Biopsy	GNBI	None	4 mo	Near -complete resection	CR	No regression during observation
5 – 11 yrs	Adrenal	44 ml	Macroscopic residuals	GNBI	4 cycles	5 mo	Near -complete resection	CR	No response to chemotherapy
5 – 11 yrs	Thoracic	42 ml	Biopsy	GNBI	None	11 mo	Complete resection	CR	Suspected Progression
18 mo – 5 yrs	Adrenal	150 ml	Biopsy	GNBI	4 cycles	6 mo	Complete resection	CR	No response to chemotherapy
18 mo – 5 yrs	Cervical	87 ml	Biopsy	GNBI	4 cycles	5 mo	Macroscopic residual	VGPR	No response to chemotherapy
18 mo – 5 yrs	Thoracic	n.a.	Macroscopic residuals	GNBI	9 cycles (+MT +RA)	17 mo	Macroscopic residual	PR	Clinical progression 3 months after diagnosis/No response to chemotherapy
18 mo – 5 yrs	Abdominal	240 ml	Biopsy	GNBI	6 cycles (+MT +RA)	7 mo	Near -complete resection	CR	No response to chemotherapy
18 mo – 5 yrs	Thoracic	39 ml	Biopsy	GNBI	7 cycles	4 mo 10 mo 81 mo	Macroscopic residual / Macroscopic residual / Biopsy	PD	No response to chemotherapy/ Progression before surgery
18 mo – 5 yrs	Pelvic	98 ml	Macroscopic residuals	GNBI	None	22 mo	Macroscopic residual	VGPR	Suspected Progression

GNBI = ganglioneuroblastoma intermixed, GN = ganglioneuroma, n.a. = data not available, yrs = years, mo = months, MT = maintenance therapy, RA = retinoic acid,
CR = complete remission, VGPR = very good partial response, PR = partial response, SD = stable disease, PD = progressive disease, LFU = lost to follow-up

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ISSN: 1471-2407

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