Needless to say, pediatric cancer therapy, especially leukemia, had remarkably progressed and successful changed in last century. However, the chemo-radiation therapy of diffuse intrinsic pontine glioma (DIPG) has still been behind the success story of pediatric cancer therapy. Although, neurosurgical pioneers had tried a lot of new project to cure pediatric DPIG, nothing of new effective protocols have been accepted with satisfactory long term survival. So, we review the pediatric DIPG therapies of neurosurgical pioneers, reconsider temozolomide based protocol, discuss to need brain biopsy, and look for the new direction of pediatric DIPG in the near future.

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For neuropathological diagnosis of glial tumors, molecular diagnosis is also important as well as classical histological diagnosis. Key molecules for glial tumors include mutations in isocitrate dehydrogenase (IDH) 1and 2, 1p/19q loss of heterozygocity (LOH), p53 mutation, ATRX mutation, MGMT promoter methylation, Tert promoter methylation as well as Ki67. Immunohistochemistry is simple, robust and universal detection methods for these mokecules compared to genetic analysis. We applied automatedquantitative analysis for detection of Ki67 index, p53 mutation and MGMT promoter methylation using Gunma-LI that were developed for Ki67 detection of brain tumors. Automated count (trial method) and manual count as a golden standard) of 18 samples of glioma sections were compared. We found automated analysis with Gunma-LI is useful for quantitative detection of p53 mutation and MGMT promoter methylation as well as Ki67 index.This method might be applied for individual molecular analysis instead of genetic examination in multicenter clinical trial.

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Treatment of malignant gliomas is extremely difficult because of their invasive nature. Stem cells have been extensively investigated as vehicles for gene delivery to malignant gliomas because of their inherent tumor-tropic properties. Here we present current trends in stem cell-based gene therapy against malignant gliomas and discuss the possibilities and problems for translating experimental findings into clinic studies.

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Intracranial meningeal hemangiopericytoma (M-HPC) is a rare, highly cellular and vascularized mesenchymal tumor that is characterized by high tendency for recurrence and extraneural metastasis.Preoperative images such as CT, MRI, and angiography are necessary for the useful information of M-HPC, although M-HPC shares similar clinical manifestations with meningioma. In distinction from others, radical resection including the attaching dura or dural sinus following adjuvant postoperative radiotherapy remains the cornerstone of therapy in M-HPC. Recently, positive STAT6 immunostaining of the tumor showed that the tumor is different from meningioma, and is the same with the entity of solitary fibrous tumor (SFT). But, advanced molecular study about NAB2-STAT6 fusion gene support the concept that classic SFT and M-HPC are separate groups that share common features but correlate to different clinical outcome.

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Craniopharyngioma is a benign epithelial neoplasm of the sellar region presumably derived from Rathke pouch epithelium.Two clinicopathological types are distinguished, the adamantinomatous and the (squamous) papillary type. The former shows odontogenic epithelial differentiation and is characterized by CTNNB1 mutations resulting in nuclear expression of beta-catenin.The latter occurs exclusively in adults and shows BRAF V600E mutation. Treatment of craniopharyngioma, a deep-seated benign tumor, has been one of the biggest challenges confronting neurosurgeons. Extent of surgical resection is the most critical prognostic factor for recurrence-free and overall survival. Recently, endoscopic endonasal surgery has become more broadly indicated because of its many advantages including direct and wide exposure of the tumor and surrounding vital structures, and avoidance of brain retraction. Despite significant improvements in overall survival, many “cured” patients, particularly pediatric patients, suffer from morbidities including endocrinopathy, visual impairment, hypothalamic dysfunction (obesity), neurocognitive dysfunction, et al. Multi-disciplinary and life-long management is essential.

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We report a case of cystic lesion in the sella turcica to suprasellar region that was difficult to diagnose with pathological and histological examinations. A 67-year-old man presented chief complaint of visual acuity.Magnetic resonance imaging (MRI) revealed cystic lesion in the sella turcica to suprasellar region. The cystic lesion stretched the pituitary stalk. The cyst wall was partially resected under bifrontal craniotomy.As pathological finding, cyst wall had been covered with single layer of cubic epithelium and/or squamous epithelium, cilia were not recognized. In immunohistochemical study, cytokeratin AE1/AE3 and Cam5.2 were strongly positive in the epithelial cells of the cyst wall, however, s-100 positive cells in the epithelium were also present. Cells lining the cyst wall were suggested to possess neuro-epithelial component. Most cases of cystic lesion in the sella turcica to suprasellar region are Rathke’s cleft cyst, and histological diagnosis of the cyst can be easy. However, histological findings for Rathke’s cleft, colloid, epithelial, and ependymal cysts are similar, and some cases are difficult to diagnose. Rathke’s pouch is ectodermal in origin, and Rathke’s cleft cyst is what remains in the sella turcica during formation of the anterior and intermediate pituitary lobes in the embryonic period. It should be differentiated from endoderm-derived colloid, epithelial, and ependymal cysts. In the present case, in the benign cystic lesion in the sella turcica to suprasellar region, the parietal cells of the cystic lesion were cytokeratin positive in the histopathological findings. Thus, Rathke’s cleft cyst was suspected by imaging findings and intraoperative findings. However, the columnar epithelium and cilium were unclear. As S-100 positive cells were disseminated, neuroepithelial cells were also suspected. Thus, a definite diagnosis of Rathke’s cleft cyst could not be made. If a histologically definitive diagnosis could not be made, it is considered necessary to first diagnose the lesion as a benign cystic lesion, record the diagnosis as histologically presumed, and then attempt a comprehensive diagnosis in combination with imaging findings.

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