Objective:To analyze the clinicopathological features of chordoid glioma.Methods:A total of 12 cases of chordoid gliomas from 2009 to 2020 in Xuanwu Hospital of Capital Medical University and General Hospital of Chinese People′s Liberation Army were retrospectively analyzed. The clinical and imaging characteristics, pathologic and molecular characteristics were analyzed, and the relevant literature was reviewed.Results:All 12 patients (4 males and 8 females) aged from 25 to 67 years (mean 39 years) and mainly had a history of headache or/and vision loss. MRI showed that the lesions located in the third ventricle, and they showed abnormal enhancement. Pathologically, these 12 cases displayed the morphologic characteristics of chordoid gliomas, including papillary structures in two cases. Immunohistochemically, GFAP and vimentin were expressed in all 12 cases (12/12). TTF1 was also expressed in all cases (10/10). CD34 and CKpan were seen in 11 cases (11/12). EMA with dot-and/or-ring like positivity was seen in 9 cases (9/10). Tissues were available in nine chordoid gliomas for Sanger sequencing to detect PRKCA and IDH gene mutation, and eight cases (8/9) showed PRKCA gene D463H mutation. None of these cases showed IDH1 R132 and IDH2 R172 mutation. All 12 patients underwent surgery, and four were lost to follow up. The remaining eight patients were progression or recurrence free at last follow-up in January 2021.Conclusions:Chordoid gliomas have relatively distinguishing clinical and histopathological features. PRKCA gene mutation in chordoid gliomas can be considered as a biomarker for the diagnosis and differential diagnosis of chordoid gliomas, and may provide a direction for future targeted therapy.

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.

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.

Objective: To investigate the clinical value of multimodal navigation-based virtual reality (MNVR) in the needle biopsy of intracranial eloquent lesions. Methods: From January 2016 to January 2017, 20 patients with intracranial deep-seated lesions involving eloquent brain areas underwent MNVR-aided needle biopsy at Department of Neurosurgery, People′s Liberation Army General Hospital. Preoperatively, MNVR was used to propose and revise the biopsy planning. Intraoperatively, navigation helped trajectory avoid the eloquent structures. Intraoperative MRI (iMRI) was performed to prove the biopsy accuracy and detect the intraoperative complications. Perioperative neurological status, iMRI findings, intraoprative complications, surgical outcome and pathological diagnosis were recorded. Wilcoxon rank-sum test was conducted to compare the preoperative and postoperative neurological scores. Results: MNVR helped revised 45%(9/20) initial biopsy trajectories, which would probably injury the nearby eloquent structures. Navigation helped biopsy trajectories spare the eloquent structures during the operation. No statistical difference was found between postoperative and preoperative neurological status, despite all the lesions were adjacent to eloquent areas. Additionally, 20 patients totally received 21 iMRI scanning. iMRI helped revise incorrect biopsy site in one case and detected intraoperative hemorrhage in another case, both of cases were treated immediately and effectively. No MNVR related adverse events and complications occurred. Conclusions MNVR-aided needle biopsy of intracranial eloquent lesions is a safe, novel and efficient biopsy modality. This technique is helpful to reduce the incidence of surgery related neurological deficits.

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.

OBJECTIVETo investigate the clinicopathologic characteristics of meningioangiomatosis (MA).

METHODSFive cases of MA were evaluated morphologically by HE and immunohistochemistry on formalin-fixed paraffin-embedded tissue. Clinical information was also obtained. The literature was reviewed. The clinical pathology and biological behavior of MA were discussed.

RESULTSFive cases of MA were reported, arising in three males and two females, with an age range of 16 to 26 years at diagnosis. All five subjects had intractable seizure disorders, and the duration of illness ranged from 8 months to 18 years. The lesions were resected from the frontal lobe in four patients, and from the temporal lobe in one. All the lesions were confined to the cortex, firm in consistency, without capsules and had poor blood supply. There was focal involvement of the overlying leptomeninges. Microscopically, they showed characteristic features of MA, such as proliferating microvessels with perivascular cuffs of spindle-cell within the cortex. Some had numerous calcifications, others showed acidophilic granular bodies. The cells were positive for EMA and vimentin by immunohistochemistry, and for reticulin by histochemical staining.

CONCLUSIONSMA is a rare, benign hamartomatous lesion of the central nervous system. It usually presents as plaque-like or nodular mass in the cerebral cortex and the overlying leptomeninges, consisting of meningovascular proliferation and leptomeningeal calcification. In some cases the lesion may show perivascular proliferation of elongated spindle-shaped cells. MA usually affects children and young adults, and is located in the frontal or temporal lobes with variable involvement of the overlying leptomeninges. Clinically, most of sporadic cases have a long history of intractable seizures despite multiantiepileptic drugs. MA has also been reported to coexist with arteriovenous malformations,meningiomas and other tumorous lesions.

OBJECTIVETo investigate histopathology and proteinopathy in the spinal cord of patients with common neurodegenerative diseases.

METHODSSpinal cord tissues from clinically and neuropathologically confirmed neruodegnerative diseases were enrolled in this study, including 3 cases of multiple system strophy, 4 cases of amyotrophic lateral sclerosis, 5 cases of Alzheimer's disease (AD, included 2 cases of AD combined with Parkinson's disease), 2 cases of progressive supranuclear palsy, 1 case of dementia with lewy body and 1 case of corticobasal degeneration from 1955 to 2013 at Chinese People's Liberation Army General Hospital. Four normal control cases were also included. Routine HE and Gallyas-Braak staining, and immunohistochemical stainings for anti-PHF tau (AT8), anti-α-synuclein, anti-TDP-43 and anti-ubiquitin were performed.

RESULTSExamination of the spinal cord in 3 cases with multiple system strophy revealed severe neuron loss in the intermediolateral nucleus of thoracic segment and Onuf's nucleus of the sacral segment, along with moderate neuron loss in the anterior horn of the cervical segment and mild myelin pallor in the anterior funiculus and anterolateral funiculus in the cervical and thoracic segments. Large amount of argentophilic, ubiquitin and synuclein positive oligodendroglial cytoplasmic inclusions were found widely distributed in the anterior horn and the anterior funiculus and anterolateral funiculus of the full spinal cord. Severe neuron loss and several morphological changes with gliosis in the anterior horn and severe loss of myelin in the anterior funiculus and anterolateral funiculus of the full spinal cord were observed in 4 cases of amyotrophic lateral sclerosis, 2 of which were found with Bunina bodies in neurons of the anterior horn. Three amyotrophic lateral sclerosis cases had ubiquitin-positive neuronal inclusions and TDP-43 positive neuronal and glial inclusions in the anterior horn at cervical and lumbar segments. A few argentophilic, tau positive neurofibrillary tangles (NFTs) and neuropil threads in the anterior horn at cervical and lumbar segments were found in 4 AD cases. Examination of spinal cord in 2 cases with Parkinson's disease combined with AD and 1 case with dementia with lewy body revealed severe neuron loss in the intermediolateral nucleus of thoracic segment, and a few synuclein positive lewy bodies and neuritis were also observed. There was mild neuron loss in the anterior horn at cervical and lumbar segments, along with some argentophilic, tau positive globous NFTs and many argentophilic, tau positive neutrophil threads were observed in 2 progressive supranuclear palsy cases and 1 corticobasal degeneration case.

CONCLUSIONEach common neurodegenerative diseases of the spinal cord including multiple system strophy, amyotrophic lateral sclerosis and Parkinson's disease has its own specific histopathology and proteinopathy characteristics.

Alzheimer Disease ; pathology ; Amyotrophic Lateral Sclerosis ; pathology ; DNA-Binding Proteins ; metabolism ; Humans ; Immunohistochemistry ; Inclusion Bodies ; pathology ; Neurodegenerative Diseases ; pathology ; Neurofibrillary Tangles ; pathology ; Neurons ; pathology ; Parkinson Disease ; pathology ; Spinal Cord ; pathology ; Ubiquitin ; metabolism ; alpha-Synuclein ; metabolism

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.

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.

Objective To investigate the clinicopathologic characteristics of meningioangiomatosis ( MA).Methods Five cases of MA were evaluated morphologically by HE and immunohistochemistry on formalin-fixed paraffin-embedded tissue.Clinical information was also obtained.The literature was reviewed.The clinical pathology and biological behavior of MA were discussed.Results Five cases of MA were reported, arising in three males and two females, with an age range of 16 to 26 years at diagnosis.All five subjects had intractable seizure disorders, and the duration of illness ranged from 8 months to 18 years.The lesions were resected from the frontal lobe in four patients, and from the temporal lobe in one.All the lesions were confined to the cortex, firm in consistency, without capsules and had poor blood supply.There was focal involvement of the overlying leptomeninges.Microscopically, they showed characteristic features of MA, such as proliferating microvessels with perivascular cuffs of spindle-cell within the cortex.Some had numerous calcifications, others showed acidophilic granular bodies.The cells were positive for EMA and vimentin by immunohistochemistry, and for reticulin by histochemical staining.Conclusions MA is a rare, benign hamartomatous lesion of the central nervous system.It usually presents as plaque-like or nodular mass in the cerebral cortex and the overlying leptomeninges, consisting of meningovascular proliferation and leptomeningeal calcification.In some cases the lesion may show perivascular proliferation of elongated spindle-shaped cells.MA usually affects children and young adults, and is located in the frontal or temporal lobes with variable involvement of the overlying leptomeninges.Clinically, most of sporadic cases have a long history of intractable seizures despite multiantiepileptic drugs.MA has also been reported to coexist with arteriovenous malformations,meningiomas and other tumorous lesions.