caa a22 4500 475784472 CHVBK 20180406123654.0 cr unu---uuuuu 170329e20000801xx s 000 0 eng 10.1007/s004310000531 doi (NATIONALLICENCE)springer-10.1007/s004310000531 When do children with optic pathway tumours need treatment? An oncological perspective in 106 patients treated in a single centre [Elektronische Daten] [Jacques Grill, Véronique Laithier, Diana Rodriguez, Marie-Anne Raquin, Alain Pierre-Kahn, Chantal Kalifa] Progression patterns of optic pathway tumours (OPT) need to be precisely defined for treatment planning. In patients with neurofibromatosis type 1 (NF1), this disease is usually indolent and the available literature rarely reports progression after the age of 6 years. In patients without NF1, the disease course seems to be less favourable. We reviewed the clinical and radiological files of 106 children referred to our institution for the treatment of a symptomatic OPT since 1980. NF1 was present in 51 of them. Progression patterns in children with NF1 differed markedly from those in the other patients. A total of 83 children had tumour extension beyond the chiasm (Dodge type III). Children with NF1 had progressive tumours later during follow-up (47% after the age of 6 years), had more often proptosis and infiltrating tumours but less frequently nystagmus or increased intracranial pressure. 32 children were not treated at diagnosis because they had only mild symptoms related to the OPT. In these patients, progression occurred more often in children without than with NF1 (12/12 versus 12/20 respectively, P=0.04). A high number of patients needed treatment for progression or severe symptoms after 6 years of age. Of the patients, 33% needed treatment for progression or severe symptoms after 6 years of age. Conclusion Progression patterns of optic pathway tumours in children with neurofibromatosis type 1 differ markedly from those in other patients. This study emphasises the need for prolonged follow-up of children with optic pathway tumours, especially in neurofibromatosis type 1. Springer-Verlag Berlin Heidelberg, 2000 Key words Optic pathway glioma nationallicence Brain neoplasm nationallicence Neurofibromatosis nationallicence Grill Jacques Département de Pédiatrie, Institut Gustave Roussy, 39, rue Camille Desmoulins, 94805 Villejuif, cedex, France e-mail: jgrill@igr.fr Tel.: +33-1-42114188; Fax: +33-1-42115275, FR aut Laithier Véronique Département de Pédiatrie, Institut Gustave Roussy, 39, rue Camille Desmoulins, 94805 Villejuif, cedex, France e-mail: jgrill@igr.fr Tel.: +33-1-42114188; Fax: +33-1-42115275, FR aut Rodriguez Diana Consultation multidisciplinaire des Neurofibromatoses, Département de Neuropédiatrie, Hôpital Saint-Vincent de Paul, Paris, France, VC aut Raquin Marie-Anne Département de Pédiatrie, Institut Gustave Roussy, 39, rue Camille Desmoulins, 94805 Villejuif, cedex, France e-mail: jgrill@igr.fr Tel.: +33-1-42114188; Fax: +33-1-42115275, FR aut Pierre-Kahn Alain Département de Neurochirurgie Pédiatrique, Hôpital Necker-Enfants Malades, Paris, France, FR aut Kalifa Chantal Département de Pédiatrie, Institut Gustave Roussy, 39, rue Camille Desmoulins, 94805 Villejuif, cedex, France e-mail: jgrill@igr.fr Tel.: +33-1-42114188; Fax: +33-1-42115275, FR aut https://doi.org/10.1007/s004310000531 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s004310000531 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Grill Jacques Département de Pédiatrie, Institut Gustave Roussy, 39, rue Camille Desmoulins, 94805 Villejuif, cedex, France e-mail: jgrill@igr.fr Tel.: +33-1-42114188; Fax: +33-1-42115275, FR aut NATIONALLICENCE 700 1- Laithier Véronique Département de Pédiatrie, Institut Gustave Roussy, 39, rue Camille Desmoulins, 94805 Villejuif, cedex, France e-mail: jgrill@igr.fr Tel.: +33-1-42114188; Fax: +33-1-42115275, FR aut NATIONALLICENCE 700 1- Rodriguez Diana Consultation multidisciplinaire des Neurofibromatoses, Département de Neuropédiatrie, Hôpital Saint-Vincent de Paul, Paris, France, VC aut NATIONALLICENCE 700 1- Raquin Marie-Anne Département de Pédiatrie, Institut Gustave Roussy, 39, rue Camille Desmoulins, 94805 Villejuif, cedex, France e-mail: jgrill@igr.fr Tel.: +33-1-42114188; Fax: +33-1-42115275, FR aut NATIONALLICENCE 700 1- Pierre-Kahn Alain Département de Neurochirurgie Pédiatrique, Hôpital Necker-Enfants Malades, Paris, France, FR aut NATIONALLICENCE 700 1- Kalifa Chantal Département de Pédiatrie, Institut Gustave Roussy, 39, rue Camille Desmoulins, 94805 Villejuif, cedex, France e-mail: jgrill@igr.fr Tel.: +33-1-42114188; Fax: +33-1-42115275, FR aut Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer