Primary histiocytic sarcoma of central nervous system: a clinicopathological study of three cases / 中华病理学杂志

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.

Implement of multimodal navigation-based virtual reality in the needle biopsy of intracranial eloquent lesions / 中华外科杂志

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.

Clinicopathological study of 6 cases of benign metastasizing leiomyoma / 临床与实验病理学杂志

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.

Histopathological and immunohistochemical study of spinal cord tissues in neurodegenerative diseases / 中华病理学杂志

<p><b>OBJECTIVE</b>To investigate histopathology and proteinopathy in the spinal cord of patients with common neurodegenerative diseases.</p><p><b>METHODS</b>Spinal 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.</p><p><b>RESULTS</b>Examination 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.</p><p><b>CONCLUSION</b>Each 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.</p>

The pathological TDP-43 protein expression in the central nervous system of motor neuron disease / 中华内科杂志

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.

Clinicopathological analysis of misdiagnosed tumefactive demyelinating lesions and primary central nervous system lymphoma / 中华神经科杂志

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.

Meningioangiomatosis: a clinicopathological study of five cases / 中华病理学杂志

<p><b>OBJECTIVE</b>To investigate the clinicopathologic characteristics of meningioangiomatosis (MA).</p><p><b>METHODS</b>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.</p><p><b>RESULTS</b>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.</p><p><b>CONCLUSIONS</b>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.</p>

Leukoencephalopathy with cerebral calcification and cysts: a case report and review of literature / 中华内科杂志

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.

Neurocutaneous melanosis with the Dandy-Walker malformation: one case report / 中华神经科杂志

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.

Quantitative synaptic alterations in human brain during normal aging and in patients with Alzheimer disease / 中国组织工程研究

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.

Evaluating non-invasive medical imaging for diagnosis of carotid artery stenosis with ischemic cerebrovascular disease / 中华医学杂志(英文版)

<p><b>OBJECTIVES</b>To assess the value of non-invasive medical imaging for diagnosis of carotid artery stenosis and to study the relationship between carotid stenosis and brain infarction.</p><p><b>METHODS</b>Thirty-one patients with a total of 62 carotid arteries were studied using Doppler ultrasound (DUS) and magnetic resonance angiography (MRA). Eleven of the 31 patients were studied using CT angiography (CTA). CT and MRI of the brain were also done in all patients. The imaging results in 5 patients were compared with those of digital subtraction angiography (DSA). Eight patients with severe stenosis received carotid endarterectomy. The comparisons between the imaging results and pathological data were conducted in 2 patients.</p><p><b>RESULTS</b>Of the 62 carotid arteries, mild stenosis was seen in 11, moderate in 14, severe in 21, obstructed in 4 and normal in 12. In 25 patients with severe stenosis or occlusion of carotid arteries, there were a total of 35 focal or multifocal infarcts on the ipsilateral cerebral hemisphere, and 15 infarcts on the contrary side. Compared with the results of the operations, DUS correctly diagnosed 6 stenoses, while MRA identified 7 correctly and CTA 8. Agreement on location of stenosis as performed by endarterectomy, DUS, MRA and CTA occurred in all patients. Histologically, areas of calcification and fibrousness were related to high densities on CTA, strong echoes on DUS, and low signal intensities on MRA. Relatively large amounts of necrotic material and foam cells filled with lipolytic materials on the intimal surface of arteries were observed during pathologically, corresponding to low and iso-densities on CTA, low echoes on DUS, and inhomogeneous signal intensities on MRA.</p><p><b>CONCLUSIONS</b>A strong link exists between carotid stenosis and brain infarction. The combined use of DUS, MRA and CTA can improve diagnostic accuracy for the assessment of carotid artery stenosis, as well as assist in ascertaining the nature of the plaque.</p>

MRI of demyelinating pseudotumor of the central nervous system / 中华放射学杂志

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.

Neuroradiologic and clinicopathologic features of dysembryoplastic neuroepithelial tumor / 中华放射学杂志

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.

Hemangiopericytoma and its manifestations in central nervous system(CNS) / 解放军医学杂志

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.