Notes

Joint Deterioration in a Computer mouse Style of Pseudoachondroplasia: Emergeny room Strain, Irritation, and Obstruct associated with Autophagy.
le application of GASMAS technology in respiratory treatment and diagnostics.A 25-year-old female presented with a generalized tonic-clonic seizure. She had no previous history of seizures. A brain magnetic resonance imaging scan revealed a solitary enhancing mass in the right fronto-parietal cortex. During surgery, the mass was noted to be pure cortical with no connection to the ventricular lining. The tumor was completely resected. After surgery, the patient had no further seizures. The biopsy result showed a supratentorial ependymoma, which was C11orf95-RELA-fusionpositive.Intracranial neurenteric cyst at the anterior craniocervical junction is very rare, and its treatment and prognosis have not been established. We report a case of neurenteric cyst at the anterior craniocervical junction and review the relevant literature. A 16-year-old girl presented with a 2-month history of slowly progressive headache. MRI revealed a well-defined intradural extramedullary cyst in the anterior medulla and brain stem with C1 cord compression. We performed gross total resection of the cyst using a far-lateral transcondylar approach. Surgical resection is the treatment of choice for neurenteric cysts at anterior craniocervical junction, the far-lateral transcondylar approach might be the optimal surgical approach.We report a patient with severe neurological deterioration due to leptomeningeal metastases involving brain and spinal cord from anaplastic lymphoma kinase (ALK)-positive lung adenocarcinoma, managed rapidly and successfully with lorlatinib therapy. A 48-year-old male patient presented with acute mental deterioration, severe headache, and weakness of both legs. The patient's previous medical history included cerebral metastases from ALK-positive lung adenocarcinoma, which had been successfully managed via whole brain radiation therapy and gamma knife radiosurgery one year and three months before, respectively. Physical examination revealed neck stiffness and paraparesis with motor grade I. Gadolinium-enhanced brain MRI showed newly developed leptomeningeal enhancement along cerebellar folia, and whole spine MRI revealed similar leptomeningeal metastasis along the whole spinal axis. Lorlatinib was started orally with a dose of 100 mg/day. The patient showed rapid clinical improvement after one week. The patient was alert and the headache disappeared, while the paraparesis improved to normal ambulatory status. Two months of lorlatinib treatment resulted in almost complete disappearance of previous leptomeningeal enhancement of brain and spinal cord, and absence of newly developed metastatic lesions in the central nervous system, based on MRI results. The patient had been regularly followed with ongoing lorlatinib therapy for 5 months without any systemic complications or neurological abnormality. Conclusively, lorlatinib could be a rapid and effective treatment for patients with central nervous system leptomeningeal metastases arising from ALK-positive lung cancer.IgG4-related disease (IgG4-RD) is an immune-mediated inflammatory condition which is characterized by dense lymphoplasmacytic infiltrations with a predominance of IgG4 plasma cells in the affected tissue. Although pachymeninx and pituitary gland are the most common sites where IgG4-RD infiltrates, the associations with IgG4-RD and a true intracranial tumor have not been yet reported in literature. Herein, we report two cases with intracranial tumors associated with IgG4-RD; a 36-year-old male patient with a huge meningioma and another 54-year old woman with a pituitary macroadenoma. Pathological examination revealed their tumors were substantially infiltrated by IgG4 plasma cells indicating its possible relation with IgG4-RD.A 42-year-old man presented with a headache and right hemiparesis and was found to have a cystic mass with a calcified mural nodule in the left thalamus. Because the thalamus is surrounded by vital neurovascular structures, the surgical approach to thalamic lesions can be challenging. We decided to remove the mass for decompression and pathological diagnosis. The mass was removed through a contralateral interhemispheric transcallosal transchoroidal approach with less retraction and parenchymal injury than other approaches to avoid brain retraction and cortical injury. The pathological diagnosis was cavernous malformation. Temporary worsening of the preoperative hemiparesis was recovered over two months following surgery. Tolerable thalamic pain syndrome remained. Here, we report a rare case of thalamic cavernous malformation with a favorable outcome through a contralateral surgical approach.Pulmonary blastoma is a rare type of primary lung cancer that accounts for only 0.25%-0.5% of all lung malignancies. Pulmonary blastoma consists of three subgroups classic biphasic pulmonary blastoma (CBPB), pleuropulmonary blastoma, and well-differentiated fetal adenocarcinoma. Due to the rarity of the tumor, metastatic brain tumor from CBPB is extremely rare, and only 13 cases, including our case, have been reported. A 60-year-old woman who underwent left upper lobectomy of the lung because of pathologically diagnosed as CBPB 5 months ago, suddenly lost consciousness and presented with stupor mental status. The emergent CT scan showed a large, 51 mL, intracerebral hemorrhage on left parieto-occipital lobe with midline shifting. The patient underwent emergent craniotomy, and a hypervascular tumor was identified during the operation. Histopathologic examination reported metastatic pulmonary blastoma, CBPB. The patient has been in a vegetative state, but there has been no evidence of recurrence over a 6-month follow-up period. We report a rare case of brain metastasis from CBPB presenting with altered mentality due to massive tumor bleeding. This is the only reported case of brain metastasis from CBPB presenting with acute intracerebral hemorrhage.We present an extremely rare case of intracranial extraskeletal myxoid chondrosarcoma. A 36-year-old male presented with dizziness persisting for 2 weeks. MRI of the patient showed well-enhanced mass of fourth ventricle. The tumor was totally removed under telovelar approach. Pathology results confirmed an intracranial extraskeletal myxoid chondrosarcoma. Adjuvant radiotherapy was initiated one month after the surgery, and MRI followed 3 months after initial operation and showed no evidence of tumor recurrence.Pituicytoma is a rare solid benign tumor of the sellar and/or suprasellar region originating from the pituicytes of the neurohypophysis or infundibulum, which is not differentiated from a pituitary adenoma that is diagnosed mostly in the sellar and/or suprasellar region. In addition, cystic tumors are very rare and have not been reported due to their solid and hypervascular natures. Ipatasertib A 33-year-old man presented with a chronic headache which exacerbated recently. MRI was performed and revealed a cystic tumor in the sellar and suprasellar regions with a small parenchymal island in the cyst compressing the optic chiasm. The endoscopic endonasal transsphenoidal approach was used to remove the tumor. Immunohistochemical staining was positive for thyroid transcription factor 1, S-100 protein, and glial fibrillary acidic protein. The pituicytoma was diagnosed based on histologic findings. The authors review herein the literature on clinical presentation, diagnosis, surgical management, and outcome.
Cadherin-11, a cell-to-cell adhesion molecule, is associated with higher tumor grade and decreased patient survival. The purpose of this study was to investigate the clinical significance of cadherin-11 expression in the progression and prognosis of a newly diagnosed primary glioblastoma (GBL).

Between 2007 and 2016, 52 out of 178 patients diagnosed with a GBL and satisfied the following criteria 1) a new primary GBL, 2) gross-total resection, 3) immunohistochemically-available tissue, and 4) standardized adjuvant treatment.

In terms of staining intensity, the low-intensity cadherin-11 group showed longer progression-free survival (PFS) than the high-intensity cadherin-11 group (median PFS, 12.0 months [95% CI, 11.1-12.9] vs. median PFS, 6.0 months [95% CI, 3.7-8.3];
<0.001). The low-intensity cadherin-11 group revealed longer overall survival (OS) than the high-intensity cadherin-11 group (median OS, 20.0 months [95% CI, 11.8-16.6] vs. median OS, 15.0 months [95% CI, 11.8-18.2];
=0.003). The staining intensity of cadherin-11 was a statistically significant factor in PFS and OS in terms of univariate and multivariate analyses (univariate analysis
<0.001 and
=0.005; multivariate analysis
<0.001 and
=0.005).

Our clinical study demonstrates high cadherin-11 expression may be associated with poor PFS and OS for a newly diagnosed primary GBL.
Our clinical study demonstrates high cadherin-11 expression may be associated with poor PFS and OS for a newly diagnosed primary GBL.
Modified orbitozygomatic craniotomy is characterized by simplicity and wide exposure. The purpose of the present study was to describe a modified orbitozygomatic approach without resecting the zygomatic arch for large parasellar tumor surgeries.

Between April 2016 and December 2019, seven patients with parasellar tumor underwent surgiest with a modified orbitozygomatic approach. Surgical procedures, clinical outcomes, and complications were analyzed.

This study included 3 meningiomas, 2 pituitary adenomas, 1 chondrosarcoma, and 1 schwannoma. Modified orbitozygomatic craniotomy provides a wider surgical freedom in the opticocarotid and prechiasmatic cistern than frontotemporal craniotomy without orbitotomy, Total, subtotal, and partial resections were achieved for 3, 2, and 2 patients, respectively. Reasons for partial resections were tight adhesion to the carotid artery and encasing of the carotid artery. Permanent morbidities developed in one patient with 3rd nerve palsy and one patient with hemiparesis.

Modified orbitozygomatic approach can provide the shortest access to the interpeduncular cistern with a minimum brain retraction. Surgeons who experience surgical challenge during the conventional approach for parasellar tumor resection are recommended to learn the modified orbitozygomatic approach.
Modified orbitozygomatic approach can provide the shortest access to the interpeduncular cistern with a minimum brain retraction. Surgeons who experience surgical challenge during the conventional approach for parasellar tumor resection are recommended to learn the modified orbitozygomatic approach.
To assess the outcome after meningioma surgery and protontherapy (PT).

We processed the French Système National des Données de Santé database to retrieve appropriate cases of meningiomas operated and irradiated between 2008 and 2017. Survival methods were implemented.

One hundred ninety-three patients who received PT after meningioma surgery over a 10-year period were identified. Of the 193 patients, 75.6% were female. Median age at surgery was 50 years (interquartile range [IQR] 41-62). The median number of PT fractions was 31 (IQR 30-39) given over a median duration of 52 days (IQR 44-69). Fourteen patients (7.3%) also received photon radiotherapy and six patients (3.1%) stereotactic radiosurgery. Median follow-up was 4.4 years (IQR 3.86-4.71). Five-year progression-free survival (PFS) rate was 69% (95% confidence interval [CI] 62.1-76.6). For benign, atypical, and malignant meningioma, 5-year PFS rates were 71.5% (95% CI 64.4-79.4), 55.6% (95% CI 32.5-95), and 35.6% (95% CI 12.8-98.9), respectively (
<0.
Homepage: https://www.selleckchem.com/products/gdc-0068.html