Objective To elucidate the clinical features and treatment of hemangiopericytomas (HPCs) in different parts of the body, especially in CNS. Methods Clinical data of 41 cases with HPC which were treated at 301 Hospital from 1993 to 2004 were retrospectively analyzed with a review of relevant literature. Follow-up periods ranged from 4 to 150 months after surgery (mean 34 months). Results Among 41 patients of HPC, 21 tumors (51.2%) were found in CNS, and the rest 20 (48.8%) in other systems. Thirteen tumors were diagnosed as malignant HPC. Surgery for tumor removal was performed in all 41 patients. Among the 62 operations, total removal of tumor was achieved in 49 cases and subtotal removal in 13 cases. Recurrence in original site of the tumor occurred 21 times (33.9%) in 14 patients. Five patients (12.1%) presented one or more distant metastasis, and 5 patients died. HPCs in CNS and in other systems displayed similar clinical characteristics and pathological morphology. Conclusions HPC is a rare angioneoplasm that may arise in any part of the body. It is a great challenge to neurosurgeons as well as other surgeons to surgically manage the tumor. HPC has an unfavorable prognosis because its high rate of recurrence and metastasis. HPC in CNS and in other parts of the body should be recognized as one disease. At the same time, HPC in CNS has its distinct features.

Objective To evaluate MRI or CT appearance and clinicopathologic features of dysembryoplastic neuroepithelial tumor (DNT). Methods MRI or CT appearance and clinicopathologic features in 12 cases of histopathology confirmed DNT were retrospectively studied. Results There were 6 men and 6 women, and the age ranged from 12 to 68 years with the average age of 36.7 years. Most patients had partial seizures, but the neurological deficits were absent. All lesions were located in the supratentorial region and involved the cerebral cortex on MRI scan. Localization of lesions was predominantly in the frontal (n=4) and temporal (n=3) lobes. The maximum lesion diameter ranged in size from 2 cm to 5 cm, and the lesion showed round, lobular- or irregular-shaped. The white matter was involved in 2 cases, and 7 were with cystic change (58.3%). All lesions were hypointense on T1-weighted images and hyperintense on T2-weighted images as well as devoid of peritumoral edema or mass effect. MRI signal intensity of cystic lesions was homogeneous on T1-weighted images which was equal to or slightly higher than that of cerebrospinal fluid. CT scan of 6 cases disclosed moderately hypodense lesion, 2 of which were markedly hypodense cystic-lobular shaped, and foci of calcification was observed in 1 case. Contrast enhancement was absent in 4 cases and only 1 case showed slight enhancement unevenly. Histologically, the DNT were classified into three subtypes: simple form (n=4), complex form (n=6), and non-specific variant (n=2). All patients experienced lesion gross total or subtotal surgical removal, and had received no chemo- or radiotherapy postoperatively. Conclusion DNT is a benign lesion, and its characterization is better disclosed by MRI compared to CT scan.

Objective To understand pathological TDP-43 features in the central nervous systems of patients with clinically and autopsy confirmed motor neuron disease (MND).Methods The clinical and histopathological features of 4 cases with MND confirmed by autopsy were summarized; anti-ubiquitin (Ub) and anti-TDP-43 immunohistochemical staining were carried out on tissue of brains and spinal cords from 4 cases with MND and 3 control cases without history of neurological disorders.Results These 4 cases presented with typical clinical and histologic features of MND.Ub-positive inclusions were observed in brain and spinal cord from 3 cases with the Ub-positive inclusions of skein-round-and lewy body-like structures.Strong TDP-43 pathological staining in brain and spinal cord was identified in 2 cases with MND presented as neuronal and glial cytoplasmic inclusions with various shapes.The TDP-43 positive inclusions were widely distributed in the motor cortex of brain and the anterior horn of spinal cord.TDP-43 weak staining in the spinal cord tissue was observed in 1 case with MND.No Ub-and TDP-43 positive inclusions were found in 3 control cases.Conclusion There is widespread pathological TDP-43 expression in the central nervous system of MND.TDP-43 positive inclusions in MND have relatively high specificity.It is worth further study on their formation mechanism.

BACKGROUND: Synaptic density, a key index of structure and function of brain tissues, is related to cognitive function. Synaptic loss occurs during human brain aging and in Alzheimer disease (AD), inducing the changes of synaptic density.OBJECTIVE: To observe quantitative synaptic alterations in human brain and changes of synaptic density in different parts during normal aging so as to compare them with those of AD patients.DESIGN: Sampling survey.SETTING: Senile Neurological Department of General Hospital of Chinese PLA.PARTICIPANTS: Pathological data were selected from General Hospital of Chinese PLA from June 1996 to December 2002. Inclusion criteria: had no major nervous system diseases and neuropathological changes. Brain tissues of 28 corpses in normal aging group, 23 males and 5 females aged 23-100 years with an average of (65±22.8) years, were obtained at autopsy.All corpses were divided into three groups according to their age, namely,adult group (23-55 years old, n=9), senile group (64-72 years old, n=7),and ＞75 group (76-100 years old, n=12). Cerebral hippocampal samples of other six corpses diagnosed with AD were selected from clinic. The corpses included 5 men and 1 woman aged 76-94 years with an average of (83±7.7) years.METHODS: Response intensity of synaptophysin immunochemistry remained stable after 4-8 hours of death, so brains were obtained at autopsy after 8-72 hours of death and fixed with 4% formalin for at least 6 weeks.In normal aging group, tissues were taken from left superior frontal gyrus,striatal area of left occipital lobe, left putamen (striatum section, including head of caudate nucleus), and left hippocampus (from lateral geniculate body section to medial occipitotemporal gyrus). In AD cases, tissues were taken from left hippocampus of 4 corpses and right hippocampus of other 2. All sections were stained with hematoxylin eosin (HE), toluidine blue and synaptophysin immunostaining (rabbit anti-human synaptophysin polyclonal antibody from Beijing Zhongshan Biotechnology Co., Ltd.). Morphology and distribution of positive objects in synapse immunologic reaction were observed under the light microscope. Relation between absorbance in each region and age was determined with Pearson's coefficient. Differences among groups were analyzed with nonparametric test, and the differences in hippocampal CA3 area between ＞ 75 group and AD group were analyzed with the same test.MAIN OUTCOME MEASURES:① Absorbency of synaptophysin at various sites of normal aging group and correlation with age; ② absorbance value in CA3 area between AD patients and advanced aged normal subjects (＞75 years) was compared.RESULTS:All the 34 cerebral samples entered the final analysis.①Synaptophysin-positive granules of various size were scattered through neocortex, putamen and hippocampus, neuronal somata, neuroglia, vessels and white matter. Density was particularly strong over layers Ⅱ and Ⅲ in frontal lobe, and layer ⅣV in occipital lobe. ② Synaptophysin density was negatively correlated with age, which was -0.688 in frontal lobe, -0.592 in occipital lobe, -0.458 in putamen and -0.619 in hippocampal CA2 area,respectively (P = 0.000, 0.001, 0.014, and 0.000). ③ Significant difference in synaptic density in CA3 area was found between AD patients (0.031 3±0.003 0)and normal subjects over the age of 75 (0.040 7±0.005 3) (Z=-2.997, P=0.001)in nonparametric test.CONCLUSION:① Synaptic density was found to decrease in frontal lobe, occipital lobe, CA3 area of hippocampus and putamen with age; the changes had significant correlation with age.② Synaptic density of AD patients was lower than that of normal subjects, and their cognitive hypofunction was related to synaptic loss. ③ All tissues were obtained after 8-72 hours of death and fixed over 6 weeks, which to the greatest extent reduced the effects of tissue autolysis and formalin fixation on the results.

Purpose To investigate the clinicopathological characteristics,diagnosis and differential diagnosis of benign metastasizing leiomyoma (BML).Methods The clinicopathological data in 6 patients with BML were collected.All cases of BLM were investigated by HE and immunohistochemistry of EnVision method.Results All cases were female,with age of 33 -65 years,and had undergone myomectomy.5 cases had lung metastasis,including abdominal wall metastasis and spinal metastasis in each of the 1 cases,and another case had inguinal metastasis.Morphology showed that the tumor cells were spindle without obvious atypia,nuclear mitoses and necrosis,some cases were cellular.Immunohistochemical staining showed that the tumor cells were positive for SMA,SM-MHC,desmin,ER,PR,vimentin,while negative for S-100,CD117,CD34.Ki-67 label index were less than 5％.3 patients were alive with tumor and 3 patients were alive without tumor in the follow up of 18,28,40,31,36,80 months.Conclusion BML often occurs in female patients that undergone uterine myomectomy.The lung is the most common site of metastasis,often accompanied by other sites.The disease progresses slowly,and most patients have a longer survival time.

Objective To improve the diagnostic ability of leukoencephalopathy with cerebral calcifications and cysts (LCC),a rare central nervous system disease.Methods The clinical manifestations,neuroimages and neuropathological features of a 19-year-old male patient were analyzed.A total of 20 cases from 14 literatures were reviewed.Results The patient was admitted with right limb weakness,cognitive decline,headache and blurred eyesight.Head CT scan showed multiple calcifications,cysts formation and leukoencephalopathy.Brain MRI showed several cysts in bilateral hemisphere,basal ganglia,thalamus and paraventricular areas.A mural nodule was noted inside one of the cyst,which was enhanced on the contrasted MRI.The wall of the cysts was partially enhanced,but not with the fluid inside the cysts.The corresponding CT calcifications foci showed on T1 and T2 with either both hyperintensity or both hypointensity,which was also partial enhanced.Extensive leukoencephalopathy was formed around the cysts and the ventricles.But neither Cho nor NAA changed a lot on MRS.Amplitude diagram of SWI series exhibited multiple round small dark signals all over the affected areas with mixed signals showed in the phase diagram,which indicated both calcifications and microbleedings at the lesions.Neuropathological examinations found no tumor cells in the operated cyst,and showed angiomatous small blood cells were dominant in the cyst wall.Hyaline degenerations,microcalcifications and hemosiderin deposition were observed.No obvious demyelination was discovered,while gliosis,numerous Rosenthal fibers and fibrinoid vascular necrosis were found around the lesions.The clinical,neuroimaging and pathological features of this patient were in accordance with the cases reported in the literatures.Conclusions Neuroimaging is the most important method for the diagnosis of LCC.As small vessel lesions are probably closely related to the pathophysiology of LCC,SWI could be recommended to further reveal the etiology of LCC.

Objective To describe the MR manifestations of demyelinating pseudotumor of the central nervous system (CNS), and to discuss the pathologic features and MR diagnostic value and limit in this disease entity. Methods Seven pathologically proved and one clinically proved cases of demyelinating pseudotumor of CNS were studied with MR imaging, and the MR imaging features were retrospectively analyzed. Results MR imaging demonstrated localized mass without adjacent multiple accompanying lesions in all 8 cases. On T 1WI, the lesions showed homogenous low signal in 5 cases, inhomogenous low signal in 2 cases, and mixed high and low signal in 1 case. On T 2WI, the lesions presented as homogenous high signal in 5 cases and inhomogenous high signal in 3 cases. Of the 7 cases with Gd DTPA administration, marked enhancement was seen in each case. The enhancement pattern of vertical distribution to the lateral ventricle was demonstrated in left frontoparietal lobe in one case, and predominant dorsal white matter enhancement of the cervical spinal cord was revealed in another case. Follow up MR imaging showed no lesion recurrence, and gradual shrinkage of the lesion after steroid therapy was demonstrated in one case. Conclusion It is a difficult task to make the correct diagnosis of CNS demyelinating pseudotumor based on the clinical information and imaging findings, and this disease entity is often misdiagnosed as tumor by MR imaging. Thorough analysis of the clinical history and careful observation of MR manifestations (especially contrast enhanced MR findings) would be helpful in diagnosing the demyelinating pseudotumor and, in such circumstances, providing test steroid therapy, thus avoiding the devastating injury caused by surgery or radiation therapy.

Objective To improve differential diagnosis of tumefactive demyelinating lesions (TDL) and primary central nervous system lymphoma (PCNSL) by analyzing the clinicopathological features of the diseases.Methods The clinical features,neuroimaging findings and pathological characteristics of 4 patients with pathologically proven TDL and 9 patients with pathologically proven PCNSL were retrospectively analyzed.Computer tomography and magnetic resonance imaging were used for neuroimaging studies.The hematoxylin and eosin staining,Luxol Fast Blue staining and immunohistochemistry were used for pathological studies.Results (1) The features of lesions on brain imaging scan:CT in TDL patients showed low density.Enhanced MRI demonstrations were different in different courses:3 cases with ring enhancement,1 case with spotty strengthen;5 PCNSL cases showed hyperdensity in CT,1 case showed isodensity,and 3 cases low-density.MRI showed enhancement of uniform enhancement in PCNSL patients.(2) The features of lesions on pathology:the plaques of lesions in TDL patients were characterized by massive demyelination with relatively axonal preservation associated with prominent astrocytosis and profound infiltrates composed.Typical pathological features in PCNSL cases were that tumor cells around blood vessels showed the cuff-like arrangement.Due to use of hormones and other causes,pathological demonstrations of a part of PCNSL cases were atypical,which were easily confused with TDL.There were 4 cases with more than one biopsy for diagnosis.Conclusions (1) PCNSL with low or equal density in CT needs to be differentiated with TDL.(2) The pathological features of some cases of PCNSL after hormone therapy were similar to TDL.It is better not to use hormone before definite diagnosis.(3) The pathology of PCNSL may be related to the progression of the disease.Some of patients need to be re-biopsied.It is important to combine clinical imaging and pathology for diagnosis of the disease,and attention should be paid to followup.

ObjectiveTo investigate the clinic and pathologic features of one patient diagnosed with neurocutaneous melanosis ( NCM ) by biopsy.MethodsA 21-year-old woman presented with a 2-month history of tinnitus,headache,vomiting and 1-month history of impaired vision.At birth,a massive nevus covering most of the posterior abdomen had been noted as well as the presence of multiple smaller lesions all over the body.Magnetic resonance imaging demonstrated a posterior fossa cyst compatible with the Dandy-Walker syndrome and extensive leptomeningeal enhancement. Surgery was performed to cystectomy and to obtain pathologic specimens from the leptomeninges. Biopsy and immunohistochemical study was performed.ResultsAt surgery,diffuse black pigmentation of the leptomeninges and the cyst was found.Under microscope,the cyst and leptomeninges were composed with melanocytes with variable pigmentation.Those cells positive for HMB45,MelanA,S100 and vimentin.Ki-67 positive cells ＜ 1％.The pathologic diagnosis wasleptomeningeal diffusemelanocytosis. Thepatientdied 2months after thesurgery.ConclusionsNCM is characterized by a focal or diffuse proliferation of melanin-producing cells in both the skin and the leptomeninges.NCM could be compatible with the Dandy-Walker syndrome.Definite diagnosis relies upon the histological data obtained by mean of biopsy.

Objective: To study the clinicopathological features, differential diagnosis and prognosis of primary histiocytic sarcoma of central nervous system(CNS). Methods: Three cases of CNS histiocytic sarcoma were collected at Chinese People′s Liberation Army General Hospital from 2005 to 2018. Their clinicopathological characteristics were analyzed, and the related literature reviewed. Results: The three patients included two females and one male, aged 36, 44, 58 years (median 44 years). MRI showed heterogeneously enhancing lesions which were considered meningioma, high-grade glioma or metastatic carcinoma. Histopathologically there were moderately pleomorphic, mitotically active tumor cells with a loose arrangement, effacing the normal brain tissue. These cells possess abundant eosinophilic cytoplasm, highly atypical nuclei, predominant nucleoli, and hemophagocytosis; multinucleated or spindled forms were also seen, as was background reactive inflammation. The tumor cells were typically positive for CD68, CD163, vimentin and lysozyme, S-100 protein, two of three cases were positive for BRAF V600E,one of three cases was partly positive for CD45, CD45RO, CD4, CD34, and negative for GFAP, Olig-2, CK, EMA, SSTR2, CD99, CD117, MPO, CD1a, Langerin, CD21, CD23, CD35, CD15, CD30, CD38, and CD138. The index of Ki-67 was 30%-75%. Rich reticular fiber network was seen in all cases; BRAF V600E mutation was present in two cases. Conclusions CNS histiocytic sarcoma is a rare malignant tumor; histopathologic and immunohistochemical examination are necessary for the diagnosis and to exclude other primary CNS and hematolymphopoietic tumors. Primary CNS histiocytic sarcoma is treated by surgery, chemotherapy and radiation therapy, but the prognosis is poor. Complete resection combined with high dose focused radiotherapy can improve the prognosis.