caa a22 4500 463200705 CHVBK 20180405153111.0 cr unu---uuuuu 170326e20071101xx s 000 0 eng 10.1007/s10014-007-0218-0 doi (NATIONALLICENCE)springer-10.1007/s10014-007-0218-0 An infant case of intracranial peripheral-type primitive neuroectodermal tumor with long-term survival [Elektronische Daten] [Kenichiro Asano, Jyun Kikuchi, Akira Munakata, Hiroki Ohkuma, Osami Kubo] Supratentorial primitive neuroectodermal tumors (S-PNET) that develop in children have recently been classified into two types: central-type PNET (C-PNET), which has been reported over the years, and peripheral-type PNET (P-PNET), which develops intracranially and was referred to as Ewing's sarcoma in the past. P-PNET is fundamentally a malignant tumor, but the patient reported here represents a case of long-term survival from onset without recurrence. At the age of 21 months, a male infant developed a cranial bone deformity and symptoms of high intracranial pressure. A CT scan revealed a cystic tumor attaching to the falx, and cyst drainage operation was immediately performed. The intracranial tumor was then resected. The tumor was an intradural extramedullary tumor, and it was totally excised with the falx attachment. The tumor was initially diagnosed as a neuroblastoma, and postoperative treatment consisted of administration of radiotherapy and chemotherapy using cyclophosphamide and vincristine. Twenty years have now passed without any recurrence. Recent repeated performance of histopathological analysis resulted in a diagnosis of P-PNET. In recent years, studies in molecular biology have demonstrated that P-PNET involves the EWS-FLI1 chimeric gene, and immunohistochemical staining has shown P-PNET to be MIC2 positive. P-PNET also differs from C-PNET with regard to prognosis, and for this reason it is believed that P-PNET and C-PNET should be considered separate entities. That is, in spite of the fact that P-PNET is a malignant tumor, patient survival can be comparatively long. Because P-PNET originates intracranially, it is fundamentally an intradural extramedullary tumor. For this reason, treatment should consist of surgical excision that is as complete as possible, followed by appropriate radiotherapy and chemotherapy. This approach can be expected to result in the patient's long-term survival. The Japan Society of Brain Tumor Pathology, 2007 Peripheral-type PNET nationallicence MIC2 nationallicence Long-term survival nationallicence Asano Kenichiro Department of Neurosurgery, Hirosaki University, School of Medicine, Zaifu-cho 5, Hirosaki, 036-8562, Aomori, Japan aut Kikuchi Jyun Department of Neurosurgery, Hirosaki University, School of Medicine, Zaifu-cho 5, Hirosaki, 036-8562, Aomori, Japan aut Munakata Akira Department of Neurosurgery, Hirosaki University, School of Medicine, Zaifu-cho 5, Hirosaki, 036-8562, Aomori, Japan aut Ohkuma Hiroki Department of Neurosurgery, Hirosaki University, School of Medicine, Zaifu-cho 5, Hirosaki, 036-8562, Aomori, Japan aut Kubo Osami Department of Neurosurgery, Tokyo Women's Medical University, Tokyo, Japan aut Brain Tumor Pathology Springer-Verlag 24/2(2007-11-01), 69-74 1433-7398 24:2<69 2007 24 10014 https://doi.org/10.1007/s10014-007-0218-0 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s10014-007-0218-0 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Asano Kenichiro Department of Neurosurgery, Hirosaki University, School of Medicine, Zaifu-cho 5, Hirosaki, 036-8562, Aomori, Japan aut NATIONALLICENCE 700 1- Kikuchi Jyun Department of Neurosurgery, Hirosaki University, School of Medicine, Zaifu-cho 5, Hirosaki, 036-8562, Aomori, Japan aut NATIONALLICENCE 700 1- Munakata Akira Department of Neurosurgery, Hirosaki University, School of Medicine, Zaifu-cho 5, Hirosaki, 036-8562, Aomori, Japan aut NATIONALLICENCE 700 1- Ohkuma Hiroki Department of Neurosurgery, Hirosaki University, School of Medicine, Zaifu-cho 5, Hirosaki, 036-8562, Aomori, Japan aut NATIONALLICENCE 700 1- Kubo Osami Department of Neurosurgery, Tokyo Women's Medical University, Tokyo, Japan aut NATIONALLICENCE 773 0- Brain Tumor Pathology Springer-Verlag 24/2(2007-11-01), 69-74 1433-7398 24:2<69 2007 24 10014 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer