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Table 1 Familial tumors syndrome (Modified from reference 17)548of tumors.To educate people about such medical care, some companies in Europe and the U.S. offer advice on whether or not to take a genetic test by simply answering a few simple questions on their websites3). However, there are warnings against easy genetic testing, especially for minors who might be exposed to test results without having the experience or Neurofibromatosis type 1Neurofibromatosis type 2SchwannomatosisLi-FraumeniVon Hippel-LindauTurcot type 1 (brain tumor-polyposis syndrome 1, hereditary non-polyposis cancer syndrome [HNPCC], constitutional mismatch repair cancer syndrome or deficiency, Lynch syndrome)Turcot type 2 (brain tumor-polyposis Syndrome 2, familial adenomatous polyposis [FAP], Gardner syndrome)Gorlin syndrome (Nevoid basal cell carcinoma)Tuberous sclerosis complexMelanoma-astrocytoma (Familial atypical multiple mole Melanoma [FAMMM])Breast cancer (BRCA)Cowden syndrome (multiple hamartoma syndrome)DICER1 syndrome(pleuropulmonaryBlastoma familial tumor and dysplasia syndrome)Multiple endocrine neoplasia type 1(Werner syndrome)GeneNF1Inheritance50% AD; 50% de novoNF250% AD; 50% de novoSMARCB1TP53AD80% AD; 20% de novoWide range of brain tumorsVHL80% AD; 20% de novoMMR genesMLH1, PMS2,MSH2, MSH6,EPCAMAPC85% AD; 15% de novoMedulloblastoma(WNT-activated)PTCH1, SUFU75% AD; 25% de novoTSC1, TSC285% AD; 25%ADCDKN2ACDKN2BP14/ARFBRCA-1BRCA-2PTENAD50% AD; 50% de novoDysplastic gangliocytoma of DICER1ADMEN1ADunderstanding to prepare for possible future diseases. Genetic societies in Japan have issued the following guidelines, which should be understood by medical professionals before testing4).Genetic testing may not be performed in the following cases:・ If the patient requests a genetic test, but the physician in charge determines that the test is CNS tumors15-20% optic pathway glioma4% low-grade glioma(Brainstem glioma)1% glioblastoma50% intracranial meningioma20% spinal meningioma30% spinal ependymoma, gliomaMeningiomareported in 20-60%GlioblastomaMedulloblastomaChoroid plexus carcinoma80% hemangioblastoma(cerebellum/spinal cord/retina)3% glioblastoma(Hypermutated phenotype)5% medulloblastoma(SHH-activated)Meningioma10% subependymal giantCell astrocytomaAstrocytomaPleomorphic xanthoastrocytomaMeningiomaGliomacerebellum(Lhermitte-Duclos syndrome)PineoblastomaPituitary blastomaPituitary adenomaEpendymoma

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