Dysembryoplastic neuroepithelial tumors in two children with neurofibromatosis type 1 |
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Authors: | Arielle Lellouch-Tubiana Marie Bourgeois Michel Vekemans Olivier Robain |
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Affiliation: | (1) Laboratoire d'Histologie-Embryologie-Cytogénétique, Hôpital Necker-Enfants Malades, Paris, France;(2) Service de Neurologie Pédiatrique, Hôpital Necker-Enfants Malades, Paris, France;(3) Laboratoire de Neuropathologie, Inserm U29, Hôpital St Vincent-de-Paul, Paris, France;(4) Laboratoire de Neuropathologie, Hôpital Necker-Enfants Malades, 149, rue de Sèvres, F-75015 Paris, France |
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Abstract: | Dysembryoplastic neuroepithelial tumors (DNT) occur mainly in children and are always clinically associated with intractable complex partial seizures. In the first report, which included 39 cases, the patients had no neurological deficit and no stigmata of phacomatosis. In contrast, we observed a DNT in 2 children with a neurofibromatosis type 1. The first patient developed intractable complex partial seizures at age 9 years and was operated at the age of 13 years. Neuroimaging study showed multifocal involvement with three separated lesions in the frontal, parietal and temporal lobes. The second patient was a 16-year-old boy with 5-year history of severe and refractory epilepsy. Magnetic resonance imaging identified a right temporal lesion and the patient underwent a right temporal lobectomy. This unusual association of two cases of DNT with neurofibromatosis type 1 raises the question of whether this association is specific or fortuitous. |
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Keywords: | Epilepsy Brain tumor Dysembryoplastic neuroepithelial tumor Hamartoma Neurofibromatosis |
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