Asunto(s)
Displasia Ectodérmica/genética , Enfermedades Genéticas Ligadas al Cromosoma X/genética , Quinasa I-kappa B/genética , Enfermedades de Inmunodeficiencia Primaria/genética , Candidiasis/genética , Infecciones por Citomegalovirus/genética , Humanos , Lactante , Masculino , Mutación Missense , Fenotipo , Neumonía por Pneumocystis/genética , RecurrenciaRESUMEN
There is little scientific evidence regarding the safety of GHRT in LGG, where GH deficiency is common. PURPOSE: to compare the recurrence rate in children with midline LGG, depending on whether or not they have received GHRT, in order to assess its impact on the risk of tumor recurrence. METHODS: This bicentric retrospective study included 124 patients under the age of 18 who were diagnosed with a midline low-grade glial tumor between 1998 and 2016. We also reviewed literature on this subject. The main outcome measure was tumor relapse, demonstrated by brain MRI. RESULTS: There were 17 patients in the GH-supplemented group (14%) and 107 patients in the non-supplemented group (86%). Relapse occurred in 65 patients (45.5%); 7 patients died (4.9%); no deaths occurred in patients receiving GHRT. Two patients developed a second tumor (1.4%), none of which had received GHRT. Relapse concerned 36.4% of patients without GHRT and 52.9% of patients with GHRT. The difference was not statistically significant between the two groups (p = 0.3). CONCLUSION: GHRT does not lead to a statistically significant increase in risk of relapse for pediatric midline low-grade pediatric glioma in our cohort. Although these results appear reassuring, future natural history or prospective studies should be done to ascertain these findings. Nevertheless, these reassuring data regarding GHRT are in agreement with the data in the current literature.