Aim of the study To evaluate the effect of primary site resection on outcomes of infants with Stage 4S Neuroblastoma (4S-NB). Methods Multicentric data of 249 4S-NB patients (average age at diagnosis 3 months, range 0-11) were retrospectively reviewed. The adrenal gland was the most frequent primary site involved (n=157; 63%), the liver the most frequent metastatic one (n=203; 82%). Massive hepatomegaly was evident only in 8 patients (3%). MYC-N amplification was detected in 14 of 184 (8%) tested tumours. Regarding to surgical extent, patients were grouped in: (A) radical resection at diagnosis (n=78; 31%); (B) radical resection delayed after a previous course of observation or neo adjuvant chemotherapy (n=24, 10%); (C) partial resection (n=18; 7%); (D) biopsy (n=129; 52%). Overall survival (OS) and event free survival (EFS) were analysed (average follow up period 7 years, range 0-30). Main results The entire populations’ OS and EFS were 83% and 75% respectively, with no relation with MYC-N amplification. A total of 42 (17%) patients died: 37 of disease progression or relapse, 5 of therapy-related toxicity (mean 561 days, range 0–4003). The extent of surgical resection significantly conditions OS and EFS (p<0,001; p<0.0001): they were higher in case of complete tumour resection at diagnosis rather than in case of partial resection (p<0,05; p<0,001) or biopsy alone (p<0,001; p<0,0001). Conclusions Since complete primary tumour resection at diagnosis is associated with better survival outcomes, it may be recommended to prevent possible tumor growth and further dissemination. Indeed, risks and rewards should be investigated individually.

ROLE OF SURGERY IN NEUROBLASTOMA 4S : A CRITICAL UPDATE

PARABOSCHI, IRENE;MATTIOLI, GIROLAMO;LEONELLI, LORENZO;MASSIRIO, PAOLO;PIO, LUCA;MARTUCCIELLO, GIUSEPPE
2017-01-01

Abstract

Aim of the study To evaluate the effect of primary site resection on outcomes of infants with Stage 4S Neuroblastoma (4S-NB). Methods Multicentric data of 249 4S-NB patients (average age at diagnosis 3 months, range 0-11) were retrospectively reviewed. The adrenal gland was the most frequent primary site involved (n=157; 63%), the liver the most frequent metastatic one (n=203; 82%). Massive hepatomegaly was evident only in 8 patients (3%). MYC-N amplification was detected in 14 of 184 (8%) tested tumours. Regarding to surgical extent, patients were grouped in: (A) radical resection at diagnosis (n=78; 31%); (B) radical resection delayed after a previous course of observation or neo adjuvant chemotherapy (n=24, 10%); (C) partial resection (n=18; 7%); (D) biopsy (n=129; 52%). Overall survival (OS) and event free survival (EFS) were analysed (average follow up period 7 years, range 0-30). Main results The entire populations’ OS and EFS were 83% and 75% respectively, with no relation with MYC-N amplification. A total of 42 (17%) patients died: 37 of disease progression or relapse, 5 of therapy-related toxicity (mean 561 days, range 0–4003). The extent of surgical resection significantly conditions OS and EFS (p<0,001; p<0.0001): they were higher in case of complete tumour resection at diagnosis rather than in case of partial resection (p<0,05; p<0,001) or biopsy alone (p<0,001; p<0,0001). Conclusions Since complete primary tumour resection at diagnosis is associated with better survival outcomes, it may be recommended to prevent possible tumor growth and further dissemination. Indeed, risks and rewards should be investigated individually.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11567/871795
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