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The histone H3K27M-mutant diffuse midline glioma is often seen in children and has a very poor prognosis regardless of its histological grade. Although it can occur in adults, few studies on adult cases have been reported. We examined adult midline glioma cases for their histological grade, presence of H3K27M mutation, and expression of related factors-enhancer of zeste homolog 2 (EZH2), H3K27me3, p16, and methylthioadenosine phosphorylase. These tumor characteristics were also evaluated for their prognostic value in adult midline glioma. High histological grade, H3K27M-mutant, high EZH2 expression, and high H3K27me3 expression was detected in 12/23 (53%), 11/23 (48%), 9/23 (39%), and 12/23 (52%) cases, respectively. Histological grade and prognosis were significantly correlated (P

A 74-year-old woman presented with central nervous system (CNS) lymphomatoid granulomatosis (LYG) that spontaneously regressed and then regrew shortly thereafter. Initial magnetic resonance imaging studies showed a well demarcated, round, enhanced lesion with perifocal edema in the left temporal lobe. The enhanced lesion and perifocal edema had drastically regressed without treatment at follow-up examination. Two months later, the lesion reappeared and was larger, so was completely removed via left fronto-temporal craniotomy. The histological diagnosis was CNS LYG. CNS LYG should be considered in the differential diagnosis of spontaneously regressing brain tumors.

A 23-year-old man was admitted with a rare case of radiation-induced astrocytoma manifesting as 3-month history of unstable gait. He had received 50 Gy of irradiation therapy for a germ cell tumor in the right basal ganglia 13 years earlier. Magnetic resonance (MR) imaging on admission showed a non-enhanced mass lesion in the right cerebellar hemisphere with expansion to the vermis. The histological diagnosis of the stereotaxic biopsy specimen was grade II astrocytoma. Two months later, he developed drowsiness, and MR imaging demonstrated that the tumor had enlarged and was enhanced after gadolinium injection. The clinical diagnosis was high-grade glioma resulting from malignant transformation. The tumor had compressed the mesencephalic aqueduct, leading to obstructive hydrocephalus. Endoscopic third ventriculostomy was performed to improve the cerebrospinal fluid circulation. He underwent chemotherapy with temozolomide postoperatively, but died 8 months after the initial diagnosis of astrocytoma. The clinical course of radiation-induced astrocytoma is not benign. The potential for malignant transformation necessitates careful postoperative follow up for patients with this tumor.

A 13-year-old girl presented with an exceedingly rare case of primary yolk sac tumor located within the lateral ventricle, manifesting as headache, nausea, and diplopia. Magnetic resonance imaging revealed a 4-cm-diameter solid enhanced mass within the left inferior horn of the lateral ventricle. The tumor was removed subtotally via left middle temporal corticotomy. The histological and immunohistochemical diagnosis was pure yolk sac tumor. The serum alpha-fetoprotein (AFP) level was elevated at 1957.2 ng/ml and the serum beta-human chorionic gonadotropin level was 4 mIU/ml after surgery. The patient underwent radiotherapy (whole brain, 30 Gy; tumor bed, 21 Gy; whole spinal axis, 30 Gy) and chemotherapy (ifosfamide, cisplatin, etoposide). After three treatment cycles, the serum AFP level had decreased to 4.5 ng/ml. However, the tumor recurred with cerebrospinal fluid dissemination after nine cycles of chemotherapy. She died 18 months after surgery. The possibility of germ cell tumor should be considered in pediatric patients with brain tumors occurring outside the pineal or suprasellar region.

A 51-year-old female presented with a rare case of choroid plexus papilloma originating in the sella turcica manifesting as headaches that was not readily distinguishable preoperatively from pituitary adenoma. Head magnetic resonance imaging revealed a tumor extending from the sella turcica to the suprasellar cistern. The tumor was removed via an endonasal transsphenoidal approach. Histological examination indicated a papillary structure covered with a layer of columnar epithelial cells that resembled normal choroid plexus. These findings, together with immunohistochemistry, led to a diagnosis of choroid plexus papilloma.

An 18-year-old male presented with a chondroma in the petrous apex manifesting as major complaints of headaches and sensorineural hearing disturbance on the right. Head computed tomography and magnetic resonance imaging revealed a neoplastic lesion in the right petrous apex, which had bulged to compress the internal auditory meatus. Surgical removal improved his hearing with normalization of the postoperative air conduction threshold by reduction of the pressure of the internal auditory meatus, suggesting that the function of the cochlea was retained and the tumor was compressing the cochleal nerve at the level of the internal auditory meatus. Histological examination showed the tumor consisted of hyaline cartilage tissue without cellular atypia and the diagnosis was chondroma.

A 74-year-old woman presented with central nervous system (CNS) lymphomatoid granulomatosis (LYG) that spontaneously regressed and then regrew shortly thereafter. Initial magnetic resonance imaging studies showed a well demarcated, round, enhanced lesion with perifocal edema in the left temporal lobe. The enhanced lesion and perifocal edema had drastically regressed without treatment at follow-up examination. Two months later, the lesion reappeared and was larger, so was completely removed via left fronto-temporal craniotomy. The histological diagnosis was CNS LYG. CNS LYG should be considered in the differential diagnosis of spontaneously regressing brain tumors.

A 23-year-old man was admitted with a rare case of radiation-induced astrocytoma manifesting as 3-month history of unstable gait. He had received 50 Gy of irradiation therapy for a germ cell tumor in the right basal ganglia 13 years earlier. Magnetic resonance (MR) imaging on admission showed a non-enhanced mass lesion in the right cerebellar hemisphere with expansion to the vermis. The histological diagnosis of the stereotaxic biopsy specimen was grade II astrocytoma. Two months later, he developed drowsiness, and MR imaging demonstrated that the tumor had enlarged and was enhanced after gadolinium injection. The clinical diagnosis was high-grade glioma resulting from malignant transformation. The tumor had compressed the mesencephalic aqueduct, leading to obstructive hydrocephalus. Endoscopic third ventriculostomy was performed to improve the cerebrospinal fluid circulation. He underwent chemotherapy with temozolomide postoperatively, but died 8 months after the initial diagnosis of astrocytoma. The clinical course of radiation-induced astrocytoma is not benign. The potential for malignant transformation necessitates careful postoperative follow up for patients with this tumor.

An 18-year-old male presented with a chondroma in the petrous apex manifesting as major complaints of headaches and sensorineural hearing disturbance on the right. Head computed tomography and magnetic resonance imaging revealed a neoplastic lesion in the right petrous apex, which had bulged to compress the internal auditory meatus. Surgical removal improved his hearing with normalization of the postoperative air conduction threshold by reduction of the pressure of the internal auditory meatus, suggesting that the function of the cochlea was retained and the tumor was compressing the cochleal nerve at the level of the internal auditory meatus. Histological examination showed the tumor consisted of hyaline cartilage tissue without cellular atypia and the diagnosis was chondroma.

A 51-year-old female presented with a rare case of choroid plexus papilloma originating in the sella turcica manifesting as headaches that was not readily distinguishable preoperatively from pituitary adenoma. Head magnetic resonance imaging revealed a tumor extending from the sella turcica to the suprasellar cistern. The tumor was removed via an endonasal transsphenoidal approach. Histological examination indicated a papillary structure covered with a layer of columnar epithelial cells that resembled normal choroid plexus. These findings, together with immunohistochemistry, led to a diagnosis of choroid plexus papilloma.

A 13-year-old girl presented with an exceedingly rare case of primary yolk sac tumor located within the lateral ventricle, manifesting as headache, nausea, and diplopia. Magnetic resonance imaging revealed a 4-cm-diameter solid enhanced mass within the left inferior horn of the lateral ventricle. The tumor was removed subtotally via left middle temporal corticotomy. The histological and immunohistochemical diagnosis was pure yolk sac tumor. The serum alpha-fetoprotein (AFP) level was elevated at 1957.2 ng/ml and the serum beta-human chorionic gonadotropin level was 4 mIU/ml after surgery. The patient underwent radiotherapy (whole brain, 30 Gy; tumor bed, 21 Gy; whole spinal axis, 30 Gy) and chemotherapy (ifosfamide, cisplatin, etoposide). After three treatment cycles, the serum AFP level had decreased to 4.5 ng/ml. However, the tumor recurred with cerebrospinal fluid dissemination after nine cycles of chemotherapy. She died 18 months after surgery. The possibility of germ cell tumor should be considered in pediatric patients with brain tumors occurring outside the pineal or suprasellar region.