Objective: To investigate the clinicopathological features, diagnosis and differential diagnosis of ectopic meningothelial hamartoma (EMH). Methods: Three cases of EMH diagnosed in the First Affiliated Hospital of Nanjing Medical University from January 2014 to December 2020 were enrolled. All cases were evaluated by clinical and imaging features, HE and immunohistochemical staining, and the relevant literature was reviewed. Results: There were one male and two female patients, aged 2, 67 and 19 years, respectively. Clinically, they presented as skin masses in the head and face region (two cases) and sacro-coccygeal region (one case). Grossly, the lesions ranged in size from 1.6 cm to 8.9 cm. Microscopically, the lesions were ill-defined, and located in the dermis and subcutis, and showed pseudovascular channels lined by monolayer of cuboidal to flattened epithelium with mild atypia, with variable cystic cavity formation. There was prominent interstitial fibrosis. Concentric, lamellated, onion skin-like arrangement with short spindle or ovoid cells and psammoma bodies were noted. Immunohistochemically, these cells were strongly positive for SSTR2, EMA, vimentin and progesterone receptor. Ki-67 positive index was low, approximately 1%. Conclusions: EMH is uncommon. Definitive diagnosis relies on histopathologic examination. The importance in recognizing the lesions is to differentiate from other more aggressive tumors.
Choristoma/pathology* ; Diagnosis, Differential ; Female ; Hamartoma/pathology* ; Humans ; Male ; Meninges ; Skin Diseases/pathology*

To study the clinicopathologic characteristics,immunohistochemical features,differential diagnosis,and prognosis of solitary fibrous tumours(SFT)/hemangiopericytomas(HPC)in the maters(meninx). Methods A series of 7 cases previously diagnosed as SFT/HPC at the Department of Pathology,Peking Union Medical College Hospital,during the period from 2008 to 2018 were analyzed for clinical data,histopathology,and immunohistochemical findings.The patients were followed up and the relevant literatures were reviewed. Results These seven patients included two males and 5 females aged 22 to 77 years(mean,49 years).Headache was the most common symptom.The magnetic resonance imaging of SFT/HPC showed irregularly contoured masses and dural tail sign was observed at the periphery of the lesion in 4 cases.The major axis of the tumor ranged from 1.8 cm to 10 cm(mean,4 cm).The tumors were located in the mater in 6 cases and in the spinal meninx in 1 case.The tumors were surgically removed in all cases.Under light microscope,the tumors were formed by long round,oval or spindle cells,with rich branching vascular pattern and varying quantity of collagenous fibers bands in both sparse areas and dense areas.According the WHO classification,2 cases were in WHO grade Ⅰ,2 cases in WHO grade Ⅱ,and 3 cases in WHO grade Ⅲ.Immunohistochemistry of the paraffin-embedded tissues in all cases showed positive immunoreativity for CD34 and vimentin in all seven cases,along with positive signal transducer and activator of transcription 6 in 4 cases,negative epithelial membrane antigen and S-100 in 7 cases,and negative progestational hormone and somatostatin receptor 2 in 6 cases.The Ki-67 index ranged from 1% to 15%.Five patients with follow-up data(including 1 current case)were alive,while 2 patients were lost to follow-up. Conclusions The SFT/HPC are rare in the maters(meninx)and is clinically difficult to be differentiated from other meningioma.The combination of CD34 and signal transducer and activator of transcription 6 helps to diagnose this disease.
Adult ; Aged ; Antigens, CD34 ; metabolism ; Diagnosis, Differential ; Female ; Hemangiopericytoma ; diagnosis ; pathology ; Humans ; Immunohistochemistry ; Male ; Meninges ; pathology ; Middle Aged ; Prognosis ; STAT6 Transcription Factor ; metabolism ; Solitary Fibrous Tumors ; diagnosis ; pathology ; Young Adult

Steroids are commonly used in patients with eosinophilic meningitis caused by A. cantonensis infections. The mechanism steroids act on eosinophilic meningitis remains unclear. In this mouse experiments, expressions of 14-3-3 isoform β and γ proteins significantly increased in the CSF 2–3 weeks after the infection, but not increasedin the dexamethasone-treated group. Expression of 14-3-3 β, γ, ɛ, and θ isoforms increased in brain meninges over the 3-week period after infection and decreased due to dexamethasone treatment. In conclusion, administration of dexamethasone in mice with eosinophilic meningitis decreased expressions of 14-3-3 isoform proteins in the CSF and in brain meninges.
Angiostrongylus cantonensis ; Angiostrongylus ; Animals ; Brain ; Dexamethasone ; Eosinophils ; Humans ; Meninges ; Meningitis ; Mice ; Protein Isoforms ; Steroids

The anatomy and clinical significance of the sinuvertebral nerve is a topic of considerable interest among anatomists and clinicians, particularly its role in discogenic pain. It has required decades of research to appreciate its role, but not until recently could these studies be compiled to establish a more complete description of its clinical significance. The sinuvertebral nerve is a recurrent nerve that originates from the ventral ramus, re-entering the spinal canal via the intervertebral foramina to innervate multiple meningeal and non-meningeal structures. Its complex anatomy and relationship to discogenic pain have warranted great interest among clinical anatomists owing to its sympathetic contribution to the lumbar spine. Knowledge of the nerve has been used to design a variety of diagnostic and treatment procedures for chronic discogenic pain. This paper reviews the anatomy and clinical aspects of the sinuvertebral nerve.
Anatomists ; Humans ; Meninges ; Spinal Canal ; Spine

Variations of the dural folds and the dural venous sinuses are infrequently reported in the existing medical literature. Such variations in the posterior cranial fossa may pose difficulties in various analytical and surgical procedures of this region. We present a rare concurrent variation of the falx cerebelli and tentorium cerebelli that was detected during routine dissection of an adult male cadaver. While removing the brain, a partial duplication of tentorium cerebelli was observed below the left half of the tentorium cerebelli and above the left cerebellar hemisphere. This fold did not have any dural venous sinus in it. Further, a complete duplication of falx cerebelli with a single occipital venous sinus within its attached border was also observed. We present the review of literature and discuss the comparative anatomy of this case.
Adult ; Anatomy, Comparative ; Brain ; Cadaver ; Cranial Fossa, Posterior ; Dura Mater ; Humans ; Male ; Meninges ; Spinal Cord

Posterior reversible encephalopathy syndrome (PRES) is a clinical-neuroradiological entity characterized by headache, vomiting, mental change, and seizures as well as images suggesting leptomeningeal edema involving in most cases posterior regions of the brain, as proven by magnetic resonance imaging. PRES occurred in association with severe infection, autoimmune disease, post-chemotherapy, and preeclampsia-eclampsia. Meningitis is an inflammation of the meninges. The most common causes of meningitis are viral and bacterial infections. While PRES is usually reversible, the early recognition and treatment of this syndrome is important to prevent permanent neurological disorders. The treatment is elimination of causal factors. We report a rare case of PRES after meningitis during pregnancy being treated with medical and rehabilitation treatment.
Autoimmune Diseases ; Bacterial Infections ; Brain ; Edema ; Headache ; Inflammation ; Magnetic Resonance Imaging ; Meninges ; Meningitis* ; Nervous System Diseases ; Posterior Leukoencephalopathy Syndrome* ; Pregnancy* ; Rehabilitation ; Seizures ; Vomiting

Bestrophin-1 (Best1) is a calcium-activated anion channel identified from retinal pigment epithelium where human mutations are associated with Best's macular degeneration. Best1 is known to be expressed in a variety of tissues including the brain, and is thought to be involved in many physiological processes. This review focuses on the current state of knowledge on aspects of expression and function of Best1 in the brain. Best1 protein is observed in cortical and hippocampal astrocytes, in cerebellar Bergmann glia and lamellar astrocytes, in thalamic reticular neurons, in meninges and in the epithelial cells of the choroid plexus. The most prominent feature of Best1 is its significant permeability to glutamate and GABA in addition to chloride ions because glutamate and GABA are important transmitters in the brain. Under physiological conditions, both Best1-mediated glutamate release and tonic GABA release from astrocytes modulate neuronal excitability, synaptic transmission and synaptic plasticity. Under pathological conditions such as neuroinflammation and neurodegeneration, reactive astrocytes phenotypically switch from GABA-negative to GABA-producing and redistribute Best1 from the perisynaptic microdomains to the soma and processes to tonically release GABA via Best1. This implicates that tonic GABA release from reactive astrocyte via redistributed Best1 is a common phenomenon that occur in various pathological conditions with astrogliosis such as traumatic brain injury, neuroinflammation, neurodegeneration, and hypoxic and ischemic insults. These properties of Best1, including the permeation and release of glutamate and GABA and its redistribution in reactive astrocytes, promise us exciting discoveries of novel brain functions to be uncovered in the future.
Astrocytes ; Brain Injuries ; Brain* ; Carisoprodol ; Choroid Plexus ; Epithelial Cells ; gamma-Aminobutyric Acid ; Glutamic Acid ; Humans ; Ions ; Macular Degeneration ; Meninges ; Neuroglia ; Neuronal Plasticity ; Neurons ; Permeability ; Physiological Processes ; Retinal Pigment Epithelium ; Synaptic Transmission

Vogt-Koyanagi-Harada disease is a rare multisystemic granulomatous autoimmune disorder affecting pigmented tissues such as the choroid, meninges, inner ear, and the skin. Neurologic symptoms are usually mild. Clinical manifestations include generalized muscle weakness, headache, meningismus, vertigo, decreased visual acuity, hearing loss and mental changes ranging from mild confusion to psychosis, hemiparesis, dysarthria, and aphasia. Seizures are very rare. We describe a case of ¹⁸F-fluorodeoxyglucose (F-18 FDG) positron emission tomography (PET) and software-fused PET-magnetic resonance imaging (MRI) in Vogt-Koyanagi-Harada disease with seizure.
Aphasia ; Choroid ; Dysarthria ; Ear, Inner ; Headache ; Hearing Loss ; Magnetic Resonance Imaging ; Meninges ; Meningism ; Muscle Weakness ; Neurologic Manifestations ; Paresis ; Positron-Emission Tomography ; Psychotic Disorders ; Seizures ; Skin ; Uveomeningoencephalitic Syndrome ; Vertigo ; Visual Acuity

Primary sarcomas of the central nervous system are rare. These tumors is rapid growth often produces mass effect on the brain. Diagnosis is rendered pathologically after resection. Surgical resection is the mainstay treatment and need the adjuvant therapy. We report a 44-year-old female with a meningeal sarcoma of frontal meninges. She complained headache for 2 months and palpable forehead mass for 3 weeks. Brain MRI demonstrated a soft tissue mass sized as 5.3×3.7×3.1 cm with well-defined osteolysis on the midline of the frontal bone. The mass attached to anterior falx without infiltration into the brain parenchyme. The tumor had extracranial and extraaxial extension with bone destruction. The tumor was totally removed with craniectomy and she had an adjuvant radiotherapy. However, an isolated subcutaneous metastasis developed at the both preauricular area of the scalp, originating from the scar which was remained the first surgery. After complete removal of this metastasis, she had an adjuvant radiotherapy in other hospital. However, she expired after six months after first surgery. We believe that the occurrence of tumor seeding at the site of incision in the scalp is related to using the fluid for irrigation after tumor resection and the same surgical instruments for the removal of the brain tumor.
Adult ; Brain ; Brain Neoplasms ; Central Nervous System ; Cicatrix ; Diagnosis ; Female ; Forehead ; Frontal Bone ; Headache ; Humans ; Magnetic Resonance Imaging ; Meningeal Neoplasms ; Meninges ; Neoplasm Metastasis ; Osteolysis ; Radiotherapy, Adjuvant ; Sarcoma* ; Scalp* ; Surgical Instruments

PURPOSE: Radiotherapy (RT) is considered a mainstay of treatment in parameningeal rhabdomyosarcoma (PM-RMS). We aim to determine the treatment outcomes and prognostic factors for PM-RMS patients who treated with RT. In addition, we tried to evaluate the adequate dose and timing of RT. MATERIALS AND METHODS: Twenty-two patients with PM-RMS from 1995 to 2013 were evaluated. Seven patients had intracranial extension (ICE) and 17 patients had skull base bony erosion (SBBE). Five patients showed distant metastases at the time of diagnosis. All patients underwent chemotherapy and RT. The median radiation dose was 50.4 Gy (range, 40.0 to 56.0 Gy). RESULTS: The median follow-up was 28.7 months. Twelve patients (54.5%) experienced failure after treatment; 4 local, 2 regional, and 6 distant failures. The 5-year local control (LC) and overall survival (OS) were 77.7% and 38.5%, respectively. The 5-year OS rate was 50.8% for patients without distant metastases and 0% for patients with metastases (p < 0.001). Radiation dose (<50 Gy vs. ≥50 Gy) did not compromise the LC (p = 0.645). However, LC was affected by ICE (p = 0.031). Delayed administration (>22 weeks) of RT was related to a higher rate of local failure (40.0%). CONCLUSION: RT resulted in a higher rate of local control in PM-RMS. However, it was not extended to survival outcome. A more effective treatment for PM-RMS is warranted.
Diagnosis ; Drug Therapy ; Follow-Up Studies ; Humans ; Ice ; Meninges ; Neoplasm Metastasis ; Radiotherapy* ; Rhabdomyosarcoma* ; Skull Base