BT-4 The treatment history of long-term survivors of Optic Pathway Glioma in Kobe Children’s Hospital

Optic pathway glioma(OPG) is almost recognized in childhood and about 0.01–0.02% of whole brain tumor in Japan. Because of the rare tumor, there are few reports about results of its treatment. In 2021, Guideline of Optic pathway/hypothalamic glioma is indicated from The japan Society for Neuro-Oncology. We retrospectively study 9 cases history of OPG who have treated for more than 5 years from January 2005 to March 2021 in Kobe Children’s Hospital. Cases are 4 boys and 5 girls. Average age at diagnosis is 3.8 years old and average follow up term is 11 years 10 months. They are 4 Pilocytic astrocytoma, 3 Pilomyxoid astrocytoma, and 1 Fibrillary astrocytoma. Al l cases have survived. There are only 2 cases who could be controlled with single series of surgical treatment and chemotherapy. Most cases need several times of resection and chemotherapy and uncontrollable 5 cases required radiological treatment. 2 cases are still under treatment for over 10 years. For OPG, partial resection to control hydrocephalus is recommended and several trial of chemotherapy must be carried out. There exist a few cases who need continuous treatment for long term. The other side, a few cases are uncontrollable those need radiotherapy to manage tumor volume. Because the history of OPG would be long term, we should adjust the treatment plan to environment of patients.

A Simplified Overview of World Health Organization Classification Update of Central Nervous System Tumors 2016

ABSTRACTAfter 8 years, an update of central nervous system (CNS) tumors was published in 2016 after 2007. First time ever, molecular markers along with histology have been used in classification of any tumor. Major changes are seen in glioma and medulloblastoma groups. Few entities have been added such as diffuse midline glioma, H3 K27M-mutant, RELA fusion-positive ependymoma, embryonal tumor with multilayered rosettes, C19MC-altered, and hybrid nerve sheath tumors. Few variants and patterns that no longer have diagnostic and/or biological relevance and have been deleted such as glioblastoma cerebri, protoplasmic and fibrillary astrocytoma, and cellular ependymoma. Other changes include deletion of term “primitive neuroectodermal tumor,” addition of criterion of brain invasion in atypical meningioma, separation of melanotic schwannoma from other schwannoma, and combination of solitary fibrous tumors and hemangiopericytoma as one entity. There is also expansion of entities in nerve sheath tumors and hematopoietic/lymphoid tumors of the CNS. In this review article, we tried to review CNS tumors 2016 classification update in a simplified manner; comparing the differences between 2016 and 2007 CNS tumors classifications with brief description of important molecular markers and finally utility as well as challenges of this classification.

Bilateral Thalamic Glioma –A Rare Tumour

We report a case of 48-year old woman who presented with dementia, progressive mental decline, personality change, paresis of the right lower extremity, and gait ataxia. Magnetic resonance imaging (MRI) T1-weighted image revealed bilateral thalamic swelling with homogenous low signal intensity and mild contrast enhancement with gadolinium. The T2- weighted image showed high intensity lesions in the bilateral thalamus, septum pellucidum and fornix. A CT-guided stereotactic biopsy of the lesion revealed fibrillary astrocytoma grade III as per WHO classification. Bilateral thalamic gliomas are very rare tumours and when present may resemble other benign diseases having similar clinical presentations DOI: http://dx.doi.org/10.3126/jonmc.v3i1.10059 Journal of Nobel Medical College Vol.3(1) 2014; 71-73