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

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

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

OBJECTIVETo investigate the histogenetic origin of primary central nervous system diffuse large B-cell lymphoma (DLBCL) with respect to the stage of B-cell differentiation, and identification of the relevant prognostic markers.

METHODSImmunohistochemical staining (EnVision method) for CD10, bcl-6, MUM-1, CD138 and FOXP1 antigens was performed on 47 paraffin-embedded sections.

RESULTSCD10, bcl-6, MUM-1 and FOXP1 expression in the tumor cells were 6.4%, 53.2%, 91.5% and 93.6% respectively. There was no expression of CD138 in all the cases. Among the 47 patients, 43 cases (91.5%) showed an activated B-cell-like (ABC) phenotype: 21 (44.7%) were bcl-6+ and MUM-1+, suggesting an "activated germinal center (GC) B-cell-like" in origin; 22 (46.8%) were exclusively MUM-1+, suggesting an "activated non-GCB" in origin. No significant correlation of the classification and FOXP1 expression found on the outcome (P=0.279 and P=0.154).

CONCLUSIONSMost primary central nervous system DLBCL are shown belonging to the ABC subgroup, suggesting that primary central nervous system DLBCL is quite similar to a DLBCL subset, which is derived from late GC to early post-GC B cell. The classification and FOXP1 expression do not show prognostic value in primary central nervous system DLBCL.

Adolescent ; Adult ; Aged ; B-Lymphocytes ; pathology ; Biomarkers, Tumor ; analysis ; Central Nervous System ; Central Nervous System Neoplasms ; diagnosis ; Female ; Humans ; Lymphoma, B-Cell ; diagnosis ; metabolism ; Lymphoma, Large B-Cell, Diffuse ; diagnosis ; metabolism ; Male ; Middle Aged ; Prognosis ; Young Adult

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

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

The authors retrospectively analyzed the angiographic findings performed in 17 cases with central neurocytoma to document the angiographic characteristics and suggested the origin of tumor based on the angiographic findings. Their medical records and radiological investigations were reviewed and cerebral angiographies were thoroughly examined in arterial, capillary, and venous phases. In ten cases, marked or moderate tumor staining was found whereas the staining was either scanty or absent in seven cases. In the ten cases showing marked or moderate tumor staining, the feeding vessels were originated from the ipsilateral carotid and/or vertebro-basilar system. In the venous phase, the ipsilateral thalamostriate vein, tortuous and enlarged, was elevated, and the internal cerebral vein was depressed. Thus, the venous angle of the ipsilateral side was widened at an irregular contour. Based on the characteristic displacement and the increased size of the associated venous system, it is suggested that the central neurocytoma might originate from a neuronal cell mass of the subependymal zone located on the floor of the lateral ventricle around the foramen of Monro rather than from the septum pellucidum.
Adult ; Angiography/*methods ; Carotid Arteries/pathology ; Central Nervous System Neoplasms/*diagnosis/*pathology ; Cerebral Ventricle Neoplasms/*diagnosis/*pathology ; Female ; Human ; Magnetic Resonance Imaging ; Male ; Models, Anatomic ; Neurocytoma/*diagnosis/*pathology ; Neurons/metabolism ; Retrospective Studies

We report a very rare case of atypical granular cell tumor arising in the neurohypophysis of a 56-year-old woman. The tumor was seen on radiology to be lobulated, soft and diffusely enhanced, the same as pituitary macroadenoma, but it was anatomically localized within the posterior part of the hypophysis. We partially removed the tumor via the transsphenoidal approach. The pathology showed nuclear pleomorphism, spindling features, and lymphoplasmacytic infiltration. Ki-67 and S-100 protein were focally positive in tumor cells. Histological diagnosis confirmed an atypical granular cell tumor in the sellar region, which is a rare tumor that often has the clinical appearance of a pituitary adenoma. Ophthalmologic symptoms are the most common, followed by endocrinologic manifestations. Here we describe its symptoms and radiological and pathological features.
Central Nervous System ; Diagnosis ; Female ; Granular Cell Tumor* ; Humans ; Middle Aged ; Pathology ; Pituitary Gland ; Pituitary Gland, Posterior ; Pituitary Neoplasms ; S100 Proteins

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

BACKGROUND: Astrocytoma in spinal cord is rare, comprising only 1% of all primary central nervous system tumors. Malignant astrocytomas( grades III and IV) account for only 7.5% of intramedullary glioma occuring in all age. Dissemination from malignant astrocytoma in spinal cord to the cerebral subarachnoid space has been rarely reported. CASE DESCRIPTION: A 22-year-old male was brought for evaluation of a back pain and progressive left leg weakness over 40 days. MRI of the thoracolumbar spine showed intramedullary mass lesion(T11-L1). Concurrent cranial CT and CSF cytology showed no abnormal findings. The tumor was removed partially. At that time, pathologic diagnosis was low grade astrocytoma. He was given regional irradiation. Six months after surgery the patient was reevaluated because of seizure. CSF cytology revealed malignant cells. Brain MRI showed leptomeningeal carcinomatosis with hydrocephalus. A second pathology of the tumor revealed malignant astrocytoma. CONCLUSION: We report a arae case of spinal intramedullary malignant astrocytoma with intracranial seeding.
Astrocytoma* ; Back Pain ; Brain ; Central Nervous System Neoplasms ; Diagnosis ; Glioma ; Humans ; Hydrocephalus ; Leg ; Magnetic Resonance Imaging ; Male ; Meningeal Carcinomatosis ; Pathology ; Seizures ; Spinal Cord ; Spine ; Subarachnoid Space ; Young Adult