title: Primary leptomeningeal anaplastic oligodendroglioma with a 1p36-19q13 deletion: report of a unique case successfully treated with Temozolomide.
abstract: Primary leptomeningeal oligodendroglioma occurs very rarely and in only one patient a deletion of chromosome 1p has been reported. We describe a 60-year-old man with a prior history of an epileptic seizure three years earlier, who was referred because of depression and a rapid evolving cognitive impairment. Brain MRI showed a diffuse right parieto-occipital subarachnoid enhancing lesion without intra-axial extension. The diagnosis of an anaplastic oligodendroglioma (WHO grade 3) was made on pathological examination. Molecular analysis using the FISH technique revealed a combined deletion of chromosomes 1p36 and 19q13. A rapid progression of the lesion was shown on MRI with leptomeningeal spinal metastases. The patient was treated with Temozolomide (TMZ) 150 mg/m(2) for 5 days every 4 weeks and showed a marked clinical recovery. Serial MRI disclosed a near complete regression of the lesions with no residual enhancement left after 6 cycles of chemotherapy. At progression following 8 cycles of TMZ the patient underwent craniospinal radiotherapy with complete response of his disease. To our knowledge this is the first report of a patient with a primary leptomeningeal anaplastic oligodendroglioma with diffuse spinal seeding bearing a 1p36/19q13 deletion. Our patient achieved a durable clinical and radiological remission following TMZ treatment. Molecular analysis with determination of chromosome 1p/19q deletions should be performed in all cases of leptomeningeal gliomas to select those patients who might benefit from TMZ chemotherapy.
authors.affiliation: Dept of Neurology, Universitair Ziekenhuis Brussel, 1090 Brussels, Belgium. alex.michotte@uzbrussel.be
authors.forename: A
authors.initials: A
authors.lastname: Michotte
authors.forename: C
authors.initials: C
authors.lastname: Chaskis
authors.forename: J
authors.initials: J
authors.lastname: Sadones
authors.forename: P In't
authors.initials: PI
authors.lastname: Veld
authors.forename: B
authors.initials: B
authors.lastname: Neyns
mesh_terms: D018906:Antineoplastic Agents, Alkylating / Q000008:administration & dosage; D001099:Arachnoid / Q000473:pathology; D001921:Brain / Q000473:pathology; D002878:Chromosomes, Human, Pair 1 / Q000235:genetics; D002888:Chromosomes, Human, Pair 19 / Q000235:genetics; D004252:DNA Mutational Analysis; D003606:Dacarbazine / Q000008:administration & dosage / Q000031:analogs & derivatives*; D017353:Gene Deletion; D020022:Genetic Predisposition to Disease / Q000235:genetics; D005838:Genotype; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D055756:Meningeal Carcinomatosis / Q000188:drug therapy / Q000235:genetics / Q000473:pathology; D008577:Meningeal Neoplasms / Q000188:drug therapy* / Q000235:genetics* / Q000473:pathology; D008875:Middle Aged; D009154:Mutation / Q000235:genetics*; D009362:Neoplasm Metastasis / Q000188:drug therapy / Q000235:genetics / Q000473:pathology; D009837:Oligodendroglioma / Q000188:drug therapy* / Q000235:genetics* / Q000473:pathology; D010841:Pia Mater / Q000473:pathology; D011878:Radiotherapy / Q000379:methods; D013116:Spinal Cord / Q000473:pathology; D013346:Subarachnoid Space / Q000473:pathology; D000077204:Temozolomide; D016896:Treatment Outcome
chemical_list: D018906:Antineoplastic Agents, Alkylating; D003606:Dacarbazine; D000077204:Temozolomide