Giorgio Perilongo1, Maria Luisa Garrè, Felice Giangaspero. 1. Paediatric Neuro-oncology Program, Division of Haematology-Oncology, Department of Paediatrics, University Hospital of Padova, Via Giustiniani 3, 35128 Padova, Italy. giorgio.perilongo@unipd.it
Abstract
DISCUSSION: The leptomeningeal dissemination (LMD) of low-grade gliomas (LGGs) is reported in clinical neuro-oncology practice more and more frequently. It is estimated that 5% of all childhood LGGs present LMD at diagnosis and 7-10% at the time of progression. LMD has been reported in association with almost all the known subtypes of LGGs. Furthermore, "unusual " LGGs can be encountered among slow-growing brain neoplasm capable of LMD, which cannot comfortably be included in the present WHO brain tumour classification. The biological, genetic and clinical characteristics that seem to favour LMD are far from being understood. Similarly, the clinical profile of those children with disseminated LGGs has not yet been firmly established. Young, non-neurofibromatosis type 1 boys with large hypothalamic-chiasmatic pilocytic astrocytomas seem to be at increased risk of LMD. The neuroradiological appearance of LMD in childhood LGGs is similar to that observed in malignant tumours, except for those cases characterised by multiple superficial non-enhancing cystic lesions, which seem to be almost exclusively associated with a type of slow-growing not yet fully identified brain stem or spinal tumour. No firm guidelines for the treatment of these diseases are yet available, mostly due to the rarity of this condition and the existing uncertainties regarding their natural clinical history. CONCLUSION: The evidence of LMD in children with LGGs does not seem to have a negative impact on patients' long-term outcome.
DISCUSSION: The leptomeningeal dissemination (LMD) of low-grade gliomas (LGGs) is reported in clinical neuro-oncology practice more and more frequently. It is estimated that 5% of all childhood LGGs present LMD at diagnosis and 7-10% at the time of progression. LMD has been reported in association with almost all the known subtypes of LGGs. Furthermore, "unusual " LGGs can be encountered among slow-growing brain neoplasm capable of LMD, which cannot comfortably be included in the present WHO brain tumour classification. The biological, genetic and clinical characteristics that seem to favour LMD are far from being understood. Similarly, the clinical profile of those children with disseminated LGGs has not yet been firmly established. Young, non-neurofibromatosis type 1boys with large hypothalamic-chiasmatic pilocytic astrocytomas seem to be at increased risk of LMD. The neuroradiological appearance of LMD in childhood LGGs is similar to that observed in malignant tumours, except for those cases characterised by multiple superficial non-enhancing cystic lesions, which seem to be almost exclusively associated with a type of slow-growing not yet fully identified brain stem or spinal tumour. No firm guidelines for the treatment of these diseases are yet available, mostly due to the rarity of this condition and the existing uncertainties regarding their natural clinical history. CONCLUSION: The evidence of LMD in children with LGGs does not seem to have a negative impact on patients' long-term outcome.
Authors: Guillaume Bergthold; Pratiti Bandopadhayay; Wenya Linda Bi; Lori Ramkissoon; Charles Stiles; Rosalind A Segal; Rameen Beroukhim; Keith L Ligon; Jacques Grill; Mark W Kieran Journal: Biochim Biophys Acta Date: 2014-02-28