BACKGROUND: Mixed gliomas, such as oligoastrocytomas (OA), anaplastic oligoastrocytomas, and glioblastomas (GBMs) with an oligodendroglial component (GBMO) are defined as tumors composed of a mixture of two distinct neoplastic cell types, astrocytic and oligodendroglial. Recently, mutations ATRX and TP53, and codeletion of 1p/19q are shown to be genetic hallmarks of astrocytic and oligodendroglial tumors, respectively. Subsequent molecular analyses of mixed gliomas preferred the reclassification to either oligodendroglioma or astrocytoma. This study was designed to apply genetically integrated diagnostic criteria to mixed gliomas and determine usefulness and prognostic value of new classification in Korean patients. METHODS: Fifty-eight cases of mixed OAs and GBMOs were retrieved from the pathology archives of Seoul National University Hospital from 2004 to 2015. Reclassification was performed according to genetic and immunohistochemical properties. Clinicopathological characteristics of each subgroup were evaluated. Overall survival was assessed and compared between subgroups. RESULTS: We could reclassify all mixed OAs and GBMOs into either astrocytic or oligodendroglial tumors. Notably, 29 GBMOs could be reclassified into 11 cases of GBM, IDH-mutant, 16 cases of GBM, IDH-wildtype, and two cases of anaplastic oligodendroglioma, IDH mutant. Overall survival was significantly different among these new groups (p<.001). Overall survival and progression-free survival were statistically better in gliomas with IDH mutation, ATRX mutation, no microscopic necrosis, and young patient age (cut off, 45 years old). CONCLUSIONS: Our results strongly suggest that a genetically integrated diagnosis of glioma better reflects prognosis than former morphology-based methods.
Astrocytoma ; Classification ; Diagnosis ; Disease-Free Survival ; Genetics ; Glioblastoma ; Glioma ; Humans ; Necrosis ; Oligodendroglioma ; Pathology ; Prognosis ; Seoul

Antigens, CD ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Astrocytoma ; metabolism ; pathology ; CD3 Complex ; metabolism ; Central Nervous System ; metabolism ; pathology ; Central Nervous System Neoplasms ; metabolism ; pathology ; Demyelinating Diseases ; diagnosis ; metabolism ; pathology ; Diagnosis, Differential ; Glial Fibrillary Acidic Protein ; metabolism ; Humans ; Lymphoma ; metabolism ; pathology ; Magnetic Resonance Imaging

Astrocytoma ; blood supply ; pathology ; Capillaries ; pathology ; Central Nervous System Neoplasms ; blood supply ; pathology ; Diagnosis, Differential ; Ependymoma ; blood supply ; pathology ; Glioblastoma ; blood supply ; pathology ; Hemangioblastoma ; blood supply ; pathology ; Humans ; Hyperplasia ; Microvessels ; pathology ; Neoplasms, Neuroepithelial ; blood supply ; pathology ; Oligodendroglioma ; blood supply ; pathology ; Paraganglioma ; blood supply ; pathology

Astrocytoma ; metabolism ; pathology ; surgery ; Brain Neoplasms ; metabolism ; pathology ; surgery ; Child, Preschool ; Diagnosis, Differential ; Ependymoma ; metabolism ; pathology ; Female ; Follow-Up Studies ; Glial Fibrillary Acidic Protein ; metabolism ; Humans ; Neoplasm Recurrence, Local ; Neoplasm Staging ; Neurofibroma, Plexiform ; metabolism ; pathology ; S100 Proteins ; metabolism ; Vimentin ; metabolism

OBJECTIVETo investigate the immunohistochemical expression of isocitrate dehydrogenase 1 gene (IDH1) R132H in glioma and its diagnostic utility.

METHODSImmunohistochemical study of IDH1R132H expression was performed on formalin-fixed paraffin-embedded tissue samples of 75 gliomas, including 33 cases of grade II, 20 cases of grade III and 22 cases of grade IV tumors. Six cases of pilocytic astrocytoma and 12 cases of gliosis were used as controls.

RESULTSNineteen in 33 cases of grade II (57.6%), 8 in 20 cases of grade III (40.0%), 6 in 22 cases of grade IV (27.3%) showed positive cytoplasmic staining of IDH1R132H. Scattered invasive glioma cells at the tumor periphery also expressed IDH1R132H. Gliomas involving the frontal lobe showed more strong IDH1R132H staining. In contrast, none of the pilocytic astrocytomas and gliosis showed IDH1R132H staining. Moreover, the rate of p53 immunopositivities were 42.4% (14/33) in grade II, 65.0% (13/20) in grade III and 77.3% (17/22) in grade IV gliomas. There were no statistic correlations between expression of IDH1R132H and p53.

CONCLUSIONIDH1R132H tends to express preferentially in low-grade gliomas, and it thus may serve as a valuable marker in distinguishing low grade gliomas from gliosis.

Adolescent ; Adult ; Aged ; Astrocytoma ; metabolism ; pathology ; Brain Neoplasms ; metabolism ; pathology ; Child ; Diagnosis, Differential ; Female ; Glioma ; metabolism ; pathology ; Gliosis ; metabolism ; pathology ; Humans ; Isocitrate Dehydrogenase ; genetics ; metabolism ; Male ; Middle Aged ; Mutation ; Tumor Suppressor Protein p53 ; metabolism ; Young Adult

Bone metastasis from a spinal cord astrocytoma has been reported only twice in the English medical literature. It is generally known that bone metastasis is found after the initial diagnosis with/without intervening surgery rather than being found at the time of the diagnosis of astrocytoma. The purpose of this article is to report for the first time a case of concurrent bone metastasis from a spinal cord astrocytoma at the time of diagnosing the spinal cord astrocytoma.
Astrocytoma/diagnosis/*secondary ; Humans ; *Lumbar Vertebrae ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Spinal Cord Neoplasms/diagnosis/*pathology ; Spinal Neoplasms/diagnosis/*secondary ; *Thoracic Vertebrae

OBJECTIVETo study the immunohistochemical expression and diagnostic significance of CD34 in brain tumors of patients with refractory epilepsy.

METHODSImmunohistochemical study for CD34 was performed on formalin-fixed paraffin-embedded tissue blocks of 54 cases of brain tumors occurring in patients with refractory epilepsy. The tumor types included ganglioglioma (GG, number = 21), dysembryoplastic neuroepithelial tumor (DNT, number = 8), tumors/lesions which had the transitional features that between glioneuronal hamartia and mixed neuronal-glial tumor (number = 21) and pleomorphic xanthoastrocytoma (PXA, number = 4). Cases of glioblastoma (number = 4) and oligoastrocytoma (number = 5) were used as controls.

RESULTSTwenty of the 21 cases of GG, 1 of the 8 cases of DNT, 16 of the 21 cases of tumors/lesions which had the transitional features and 3 of the 4 cases of PXA showed cytoplasmic and membranous positivity for CD34. The adjoining brain tissues in 9 of the 18 cases of GG, 6 of the 16 cases of tumors/lesions which had the transitional features and 1 of the 3 cases of PXA also expressed CD34. In contrast, only 1 case of glioblastoma showed membranous positivity for CD34.

CONCLUSIONSCD34 preferred to staining for GG and PXA. Which represent a valuable tool for distinguishing GG, PXA and DNT, oligoastrocytoma, glioblastoma.

Antigens, CD34 ; metabolism ; Astrocytoma ; complications ; metabolism ; pathology ; surgery ; Brain Neoplasms ; complications ; metabolism ; pathology ; surgery ; Diagnosis, Differential ; Epilepsy ; etiology ; Ganglioglioma ; complications ; metabolism ; pathology ; surgery ; Glioblastoma ; complications ; metabolism ; pathology ; Humans ; Neoplasms, Neuroepithelial ; complications ; metabolism ; pathology ; surgery

Astrocytoma ; metabolism ; pathology ; Biomarkers, Tumor ; metabolism ; Brain Neoplasms ; metabolism ; pathology ; Cell Proliferation ; Diagnosis, Differential ; Glioblastoma ; metabolism ; pathology ; Humans ; Ki-67 Antigen ; metabolism ; Melanoma ; metabolism ; pathology ; Meningeal Neoplasms ; metabolism ; pathology ; Meningioma ; metabolism ; pathology ; Nevus ; metabolism ; pathology ; Proliferating Cell Nuclear Antigen ; metabolism ; Skin Neoplasms ; metabolism ; pathology

We report a case of acute comitant esotropia in a child with a cerebellar tumor. A 3-year-old boy was referred for management of a 9 month history of acute acquired comitant esotropia. On first presentation, the patient's angle of esodeviation was 50 prism-diopters (PD) at distance and near fixation without any lateral incomitance. The cycloplegic refraction revealed +0.75 diopters in both eyes. Very mild bilateral papilledema was found on the fundus examination, but the neurological examination did not reveal any other pathological findings. Brain MRI showed a 5 cm mass located in the midline of the cerebellum as well as hydrocephalus. The mass was completely excised and histological examination confirmed the diagnosis of pilocytic astrocytoma. Despite neurosurgery, the patient's strabismus remained unresolved. One year after neurosurgery, both medial rectus muscles were surgically recessed by 6 mm, resulting in esotropia of 8PD at distant and near fixation without restoration of bifoveal fusion at follow-up 2 years after the eye muscle surgery. Therefore, acute onset comitant esotropia in a child can be the first sign of a cerebellar tumor, even without any other neurological signs and symptoms.
Acute Disease ; Astrocytoma/*complications/surgery ; Brain/pathology ; Cerebellar Neoplasms/*complications/diagnosis/surgery ; Child, Preschool ; Esotropia/*etiology/pathology/physiopathology/surgery ; Humans ; Magnetic Resonance Imaging ; Male ; Neurosurgical Procedures ; Oculomotor Muscles/surgery ; Refraction, Ocular ; Time Factors

OBJECTIVETo study clinicopathologic features, treatment and prognosis of pilocytic astrocytoma (PA).

METHODSHistopathological, ultrastructural, immunohistochemical (EnVision method) and clinical features of 68 cases of PA were studied by microscopic investigation with correlation of clinical follow-up information when available.

RESULTSThirty-five male patients and 33 female patients were studied. The patient's age ranged from 3 to 66 years (mean = 20.1 years). The mean time from symptom onset to surgery was 371 days (range, 3 days to 14 years). Cystic degeneration was noted in 41 cases (60.3%), and enhancement of the tumor was noted in 43 cases (87.8%). On postcontrast imaging examination there were 33 cases involving the cerebellum (48.5%). Total tumor excision was performed in 35 patients, subtotal tumor excision was performed in 31 patients, and the procedures of other 2 patients were not clear. Among 51 patients with follow-up information, 44 were alive, 7 had recurrent tumor, and 7 died. The post-operative survival ranged from 2 months to 124 months (mean survival = 48.1 months). Five years and ten years survival rates were 89%, respectively. Tumors with classic histopathology demonstrated biphasic pattern of growth, consisting of compact elongated bipolar astrocytes associated with rosenthal fibers, and less cellular areas of multipolar cells with granular bodies and microcyst. Some cases showed atypia of nuclei, and occasional mitoses. Involvement of subarachnoid space was seen in 17 cases. One case had anaplastic features. All cases showed diffuse positive staining for GFAP and low expression for Ki-67, except 1 anaplastic tumor with 10% Ki-67 indices. Tumors with subarachnoid space involvement showed positive reticular fiber staining and negative EMA staining.

CONCLUSIONSPA is a benign, WHO grade I tumor with favorable prognosis, and does not require radiotherapy after total resection. The tumor can be mistaken as higher-grade astrocytoma when involving the subarachnoid space, and with cytological atypia, leading to unnecessary radiotherapy after surgery. Recurrence rate is increased when only partial resection is achieved. The outcome for patients with brainstem tumor or anaplastic PA is poor.

Astrocytoma ; diagnosis ; genetics ; Brain Neoplasms ; diagnosis ; genetics ; Cell Nucleus ; pathology ; Female ; Glial Fibrillary Acidic Protein ; genetics ; Humans ; Male ; Prognosis ; Recurrence ; Treatment Outcome