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

Bone Neoplasms ; pathology ; Chondrosarcoma, Mesenchymal ; pathology ; Dura Mater ; pathology ; Humans ; Meninges ; pathology

PURPOSE: Leptomeningeal collateral, in moyamoya disease (MMD), appears as an ivy sign on fluid-attenuated inversion-recovery (FLAIR) images. There has been little investigation into the relationship between presentation of ivy signs and old brain lesions. We aimed to evaluate clinical significance of ivy signs and whether they correlate with old brain lesions and the severity of clinical symptoms in patients with MMD. MATERIALS AND METHODS: FLAIR images of 83 patients were reviewed. Each cerebral hemisphere was divided into 4 regions and each region was scored based on the prominence of the ivy sign. Total ivy score (TIS) was defined as the sum of the scores from the eight regions and dominant hemispheric ivy sign (DHI) was determined by comparing the ivy scores from each hemisphere. According to the degree of ischemic symptoms, patients were classified into four subgroups: 1) nonspecific symptoms without motor weakness, 2) single transient ischemic attack (TIA), 3) recurrent TIA, or 4) complete stroke. RESULTS: TIS was significantly different as follows: 4.86+/-2.55 in patients with nonspecific symptoms, 5.89+/-3.10 in patients with single TIA, 9.60+/-3.98 in patients with recurrent TIA and 8.37+/-3.39 in patients with complete stroke (p=0.003). TIS associated with old lesions was significantly higher than those not associated with old lesions (9.35+/-4.22 vs. 7.49+/-3.37, p=0.032). We found a significant correlation between DHI and motor symptoms (p=0.001). CONCLUSION: Because TIS has a strong tendency with severity of ischemic motor symptom and the presence of old lesions, the ivy sign may be useful in predicting severity of disease progression.
Adolescent ; Adult ; Aged ; Brain/metabolism/*pathology ; Cerebral Arteries/*pathology ; Child ; Child, Preschool ; Collateral Circulation ; Disease Progression ; Female ; Humans ; Magnetic Resonance Imaging/*methods ; Male ; Meninges/*pathology ; Middle Aged ; Moyamoya Disease/complications/*pathology ; Severity of Illness Index ; Stroke ; Young Adult

OBJECTIVE: To describe the anatomy,radiographic manifestation, and surgical treatment of pulsatile tinnitus due to sigmoid sinus diverticulum. METHOD: The retrospectively study of diagnosis,clinical and radiographic manifestations,and surgical treatment of 2 cases of pulsatile tinnitus caused by sigmoid sinus diverticulum was carried out. RESULT: Bony defect was found in the anterior sigmoid wall in both cases, and one of them was combined with invasion of soft tissues in mastoid cells. Both of them undertook a surgery to reconstruct sinus wall and received a satisfactory results. CONCLUSION Sigmoid sinus diverticulum is one of causes which lead to pulsatile tinnitus. It can be identified by imaging examination and can be cured by sinus wall reconstruction.
Adult ; Diverticulum ; complications ; Female ; Humans ; Male ; Meninges ; Retrospective Studies ; Tinnitus ; etiology ; pathology ; surgery

Adult ; Antigens, Neoplasm ; analysis ; Diagnosis, Differential ; Female ; Humans ; Immunohistochemistry ; Magnetic Resonance Imaging ; Melanoma ; cerebrospinal fluid ; metabolism ; pathology ; Melanoma-Specific Antigens ; Melanosis ; cerebrospinal fluid ; metabolism ; pathology ; Meningeal Neoplasms ; cerebrospinal fluid ; metabolism ; pathology ; Meninges ; chemistry ; pathology ; Neoplasm Proteins ; analysis ; S100 Proteins ; analysis

Angiomatosis ; complications ; pathology ; Brain ; blood supply ; Brain Diseases ; complications ; pathology ; Brain Neoplasms ; complications ; pathology ; Epilepsy ; etiology ; pathology ; surgery ; Ganglioglioma ; complications ; pathology ; Humans ; Malformations of Cortical Development ; classification ; complications ; pathology ; Meninges ; blood supply

Adolescent ; Adult ; Head and Neck Neoplasms ; diagnosis ; pathology ; Humans ; Magnetic Resonance Imaging ; Male ; Meninges ; pathology ; Sarcoma ; diagnosis ; pathology ; Scalp ; pathology ; Skin Neoplasms ; diagnosis ; pathology ; Skull ; pathology

Adult ; Antigens, CD ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Diagnosis, Differential ; Histiocytosis, Langerhans-Cell ; pathology ; Humans ; Immunohistochemistry ; Male ; Meningeal Neoplasms ; metabolism ; pathology ; surgery ; Meninges ; pathology ; Peroxidase ; metabolism ; Sarcoma, Myeloid ; metabolism ; pathology ; surgery

OBJECTIVETo evaluate the therapeutic effect of a new recombinant immunotoxin mMIP-1alpha-DT390 on experimental autoimmune encephalomyelitis (EAE).

METHODSEAE was induced in the low-sensitive strain C57BL/6 mice with intraperitoneal injection of myelin basic protein (MBP) to simulate the human disease multiple sclerosis, followed by intramuscular injection of cationic liposome carrying the plasmid DNA SRalpha-mMIP-1alpha-DT390 in the leg muscle to elicit resistance to EAE development. The mice were then examined daily for clinical signs of EAE by an observer blind to the treatment protocol. For immunohistochemistry the mice were anesthetized and perfused with sterile PBS and paraformaldehyde, and the cerebrum, cerebellum, medulla and spinal cord were removed for preparation of serial sections. The mononuclear cells (MNCs) from the EAE mouse spleens were prepared for three-color flow cytometry analysis of the surface markers with appropriate antibodies following the BD Pharmingen cytokine staining protocol.

RESULTSEAE model was successfully established by active MBP immunization in C57BL/6 mice. Administration of the immunotoxin mMIP-1alpha-DT390 significantly delayed the disease onset and lowered the mean clinical score for EAE as compared with the control mice. Immunohistochemistry demonstrated much less CCR5(+) infiltrating cells in the central nervous system in mMIP-1alpha-DT390-treated mice than in the control. The treatment also eliminated reactive T cells in the periphery blood without affecting the number of B cells.

CONCLUSIONThe immunotoxin mMIP-1alpha-DT390 can attenuate the disease activity of EAE in mice, suggesting its potential use in the treatment of other autoimmune disorders.

Animals ; Antigens, CD19 ; analysis ; B-Lymphocytes ; cytology ; metabolism ; CD3 Complex ; analysis ; Chemokine CCL3 ; genetics ; metabolism ; Diphtheria Toxin ; genetics ; metabolism ; Disease Models, Animal ; Encephalomyelitis, Autoimmune, Experimental ; drug therapy ; Female ; Flow Cytometry ; Immunoglobulin Fragments ; genetics ; metabolism ; Immunohistochemistry ; Immunologic Factors ; therapeutic use ; Immunotoxins ; therapeutic use ; Meninges ; chemistry ; pathology ; Mice ; Mice, Inbred C57BL ; Multiple Sclerosis ; drug therapy ; NIH 3T3 Cells ; Receptors, CCR5 ; analysis ; Recombinant Fusion Proteins ; genetics ; metabolism ; therapeutic use ; T-Lymphocytes ; cytology ; metabolism