Objective To analyze the disease spectrum and clinicopathological characteristics of central nervous system(CNS)diseases diagnosed based on pathological findings in Tibet. Methods We collected the data of all the cases with CNS lesions in Tibet Autonomous Region People's Hospital from January 2013 to December 2020.The clinicopathological features were analyzed via light microscopy,immunohistochemical staining,and special staining. Results A total of 383 CNS cases confirmed by pathological diagnosis were enrolled in this study,with a male-to-female ratio of 188∶195 and an average age of(40.03±17.39)years(0-74 years).Among them,127(33.2%)cases had non-neoplastic diseases,with a male-to-female ratio of 82∶45 and an average age of(31.99±19.29)years;256(66.8%)cases had neoplastic diseases,with a male-to-female ratio of 106∶150 and an average age of(44.01±14.87)years.The main non-neoplastic diseases were nervous system infectious diseases,cerebral vascular diseases,meningocele,cerebral cyst,and brain trauma.Among the infectious diseases,brain abscess,granulomatous inflammation,cysticercosis,and hydatidosis were common.The main neoplastic diseases included meningioma,pituitary adenoma,neuroepithelial tumor,schwannoma,metastatic tumor,and hemangioblastoma.The meningioma cases consisted of 95.4%(103/108)cases of grade Ⅰ,3.7%(4/108)cases of grade Ⅱ,and only 1(1/108,0.9%)case of grade Ⅲ.Among the neuroepithelial tumor cases,the top three were glioblastoma,grade Ⅲ diffuse glioma,and ependymoma. Conclusions There are diverse CNS diseases confirmed by pathological diagnosis in Tibet,among which non-neoplastic diseases account for 1/3 of all the cases.Infectious and vascular diseases are the most common non-neoplastic diseases in Tibet,and tuberculosis and parasitic infections are relatively common.The types and proportion of brain tumors in Tibet are different from those in other regions of China,and meningioma is the most common in Tibet,with higher proportion than neuroepithelial tumor.
Adolescent ; Adult ; Aged ; Brain Neoplasms/diagnosis* ; Central Nervous System Diseases/pathology* ; Child ; Child, Preschool ; Ependymoma ; Female ; Humans ; Infant ; Infant, Newborn ; Male ; Meningeal Neoplasms ; Middle Aged ; Retrospective Studies ; Tibet/epidemiology* ; Young Adult

Objective: To investigate immunohistochemical patterns of CXorf67 and H3K27me3 proteins in central nervous system germ cell tumors (GCTs) and to assess their values in both diagnosis and differential diagnosis. Methods: A total of 370 cases of central nervous system GCTs were collected from 2013 to 2020 at Huashan Hospital of Fudan University, Shanghai, China. The expression of CXorf67, H3K27me3 and commonly-used GCT markers including OCT4, PLAP, CD117, D2-40, and CD30 by immunohistochemistry (EnVision method) was examined in different subtypes of central nervous system GCTs. The sensitivity and specificity of each marker were compared by contingency table and area under receiver operating characteristic (ROC) curve. Results: Of the 370 cases there were 282 males and 88 females with a mean age of 19 years and a median age of 17 years (range, 2-57 years). Among the GCTs with germinoma, the proportions of male patients and the patients with GCT located in sellar region were both higher than those of GCTs without germinoma (P<0.05), respectively. CXorf67 was present in the nuclei of germinoma and normal germ cells, but not in other subtypes of GCT. H3K27me3 was negative in germinoma, but positive in the nuclei of surrounding normal cells and GCTs other than germinoma. In the 283 GCTs with germinoma components, the expression rate of CXorf67 was 90.5% (256/283), but no cases were positive for H3K27me3. There was also an inverse correlation between them (r²=-0.831, P<0.01). The expression rates of PLAP, OCT4, CD117 and D2-40 were 81.2% (231/283), 89.4% (253/283), 73.9% (209/283) and 88.3% (250/283), respectively. In 63 mixed GCTs with germinoma components, the expression rate of CXorf67 was 84.1% (53/63), while all cases were negative for H3K27me3. The expression rates of PLAP, OCT4, CD117 and D2-40 were 79.4% (50/63), 79.4% (50/63), 66.7% (42/63) and 87.3% (55/63), respectively. The 6 markers with largest area under ROC curve in ranking order were H3K27me3, CXorf67, D2-40, OCT4, PLAP and CD117 (P<0.05). Conclusions: CXorf67 and H3K27me3 have high sensitivity and high specificity in diagnosing germinoma. There is a significant inverse correlation between them. Therefore, they can both be used as new specific immunohistochemical markers for the diagnosis of GCTs.
Adolescent ; Adult ; Brain Neoplasms/pathology* ; Central Nervous System/pathology* ; Central Nervous System Neoplasms/metabolism* ; Child ; Child, Preschool ; China ; Female ; Germinoma/pathology* ; Histones ; Humans ; Male ; Middle Aged ; Neoplasms, Germ Cell and Embryonal/diagnosis* ; Oncogene Proteins ; Transcription Factors/metabolism* ; Young Adult

Adult ; Central Nervous System Neoplasms ; diagnosis ; Ciliary Body ; pathology ; Humans ; Male

Ataxia ; Carbamazepine ; analogs & derivatives ; therapeutic use ; Carcinoma ; Central Nervous System ; pathology ; Diagnosis, Differential ; Female ; Gait ; Humans ; Magnetic Resonance Imaging ; Middle Aged ; Nasopharyngeal Neoplasms ; complications ; radiotherapy ; Nervous System Diseases ; complications ; diagnosis ; Radiotherapy ; adverse effects ; Seizures ; complications ; diagnosis ; Siderosis

OBJECTIVETo investigate the characteristics, treatment and outcome of patients with primary central nervous system lymphoma （PCNSL）.

METHODSA total of 37 patients with PCNSL treated in Peking Union Medical College Hospital from June 1999 to June 2012 were enrolled into this retrospective study. The clinical characteristics, results of treatment and prognostic factors were analyzed.

RESULTSThe median age of 37 patients with PCNSL at diagnosis was 57 years（range 17 to 78 years） with a male to female ratio of 2.7:1. The symptoms or signs of elevated intracranial pressure and cognitive dysfunction were the most common initial manifestations. The median time period between onset of symptoms and diagnosis was 1.5 months. The majority of lesions were located in the cerebral hemisphere. At a median follow-up of 50 months, the median overall survival for all treated patients was 36.0 months （95% CI 21.7-50.3 months）, with a progression-free survival of 18.0 months（95% CI 9.1-26.9 months）. The 3-year cumulative survival rate was 46.9%. Compared to chemotherapy alone, combined-modality regimens which did not improve outcome were associated with a greater risk of neurotoxicity.

CONCLUSIONThe prognosis of PCNSL was still poor, and the optimal treatment strategy for these patients should be explored in the future clinical trials.

Adolescent ; Adult ; Aged ; Beijing ; Central Nervous System Neoplasms ; diagnosis ; pathology ; Disease-Free Survival ; Female ; Humans ; Lymphoma, Non-Hodgkin ; Male ; Middle Aged ; Prognosis ; Retrospective Studies ; Survival Rate ; Young Adult

No abstract available.
Antineoplastic Agents/*therapeutic use ; Benzamides/*therapeutic use ; Central Nervous System Neoplasms/*diagnosis ; Fusion Proteins, bcr-abl/genetics ; Humans ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive/*drug therapy ; Leukocytes/metabolism/pathology ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; Piperazines/*therapeutic use ; Pyrimidines/*therapeutic use

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

Central nervous system (CNS) is one of places in which direct infiltration and involvement or relapse occur in adult lymphocytic leukemia. The main mechanism of CNS infiltration in leukemia is associated with blood brain barrier (BBB), however the ALL CNS infiltration is difficulty early predicted and evaluated.For this reason, the studies on accurately evaluating the ALL CNS infiltration have important significance for early diagnosis and adjustment of therapeutic regimen, performance of individualised treatment and improvement of ALL patient's prognosis. In this article, the pathway of ALL CNS infiltration and its molecular mechanism, the evaluation methods for BBB function are reviewed.
Blood-Brain Barrier ; Central Nervous System Neoplasms ; diagnosis ; pathology ; Early Diagnosis ; Humans ; Neoplasm Invasiveness ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; diagnosis ; pathology

INTRODUCTIONWe reviewed the clinical features, brain and spinal cord magnetic resonance (MR) imaging findings and associated abnormalities in six patients with spinal cavernous malformations (CMs).

METHODSLesions were defined on gradient-recalled echo (GRE) images but measured on T2-weighted images performed on 1.5- and 3-tesla clinical scanners.

RESULTSFour patients had associated multiple cranial CMs and one patient had multiple spinal CMs. All spinal CMs were predominantly hypointense on GRE images, and most were predominantly hyperintense and surrounded by hypointense edge on T2-weighted images. Other associations included asymptomatic vertebral body and splenic haemangiomas.

CONCLUSIONWe conclude that intramedullary spinal CMs typically have 'mulberry' or 'popcorn' appearances similar to those of cranial CM. The presence of associated haemangioma or familial cranial CM syndrome on MR imaging may suggest the correct diagnosis without requiring invasive investigations.

Adult ; Aged ; Brain Neoplasms ; pathology ; Central Nervous System Vascular Malformations ; pathology ; Child, Preschool ; Diagnosis, Differential ; Female ; Hemangioma, Cavernous, Central Nervous System ; pathology ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Neoplastic Syndromes, Hereditary ; pathology ; Retrospective Studies ; Spinal Cord Diseases ; pathology ; Spinal Cord Neoplasms ; pathology

Aged ; Brain Neoplasms ; diagnosis ; pathology ; Central Nervous System Neoplasms ; diagnosis ; pathology ; Fatal Outcome ; Female ; Humans ; Lymphoma ; diagnosis ; pathology ; Meningeal Neoplasms ; diagnosis ; secondary