Validation and Evaluation of 5 Scoring Systems for Predicting Metastatic Risk in Pheochromocytoma and Paraganglioma.

Currently, 5 scoring systems have been proposed in the literature for predicting metastatic risk in pheochromocytoma and paraganglioma (PPGL): Pheochromocytoma of the Adrenal Gland Scaled Score (PASS), Grading System for Adrenal Pheochromocytoma and Paraganglioma (GAPP), Composite Pheochromocytoma/paraganglioma Prognostic Score (COPPS), Age, Size, Extra-adrenal location, Secretion type (ASES) score, and Size, Genetic, Age, and PASS (SGAP) model. To validate and evaluate these 5 scoring systems, we conducted a retrospective review of cases diagnosed as PPGL at the Department of Pathology, West China Hospital of Sichuan University, between January 2012 and December 2019. A total of 185 PPGL cases were included, comprising 35 cases with metastasis and 150 cases remained metastasis-free for over 8 years after surgery. The criteria of the 5 scoring systems were used for scoring and risk classification. The predictive performance of the 5 scoring systems was validated, compared, and evaluated using concordance index (C-index) and decision curve analysis (DCA). The C-indices for PASS, GAPP, and SGAP were 0.600, 0.547, and 0.547, respectively, indicating low discriminative ability. In contrast, COPPS and ASES had C-indices of 0.740 and 0.706, respectively, indicating better discriminative performance. DCA also showed that the predictive capability of COPPS was superior to that of ASES, with both outperformed PASS, while PASS had better predictive ability than GAPP and SGAP. Our analysis indicated that pathology-based scoring systems cannot accurately predict metastatic risk of PPGL. Establishing a precise prediction system requires integrating clinical, pathologic, and molecular information, using a scientific methodology for predictive factor selection and weight assessment.

Posterior Inferior Cerebellar Artery Aneurysm Shaped Like a "Calabash Gourd" and Mimicking a Tumor.

Posterior inferior cerebellar artery aneurysms are likely to be fusiform, yet they hardly enlarge to mimic a tumor in the posterior fossa on radiology. They constitute about 3%-4% of all cerebral aneurysms. A 65-year-old woman presented with tremor in her right upper limb for 1 year and intermittent dizziness for 8 months. Interestingly, magnetic resonance imaging revealed 2 unanimously enhanced masses like mother and daughter located in the right cerebellum hemisphere. The lesion was resected via surgery, and histopathology established the diagnosis of an aneurysm. Her tremor and dizziness subsided 3 months after the surgery, and at her 2-year follow-up she was well with no further neurologic deficits.

Lhermitte-Duclos disease with excessive calcification in a septuagenarian: A case report.

RATIONALE: Lhermitte-Duclos disease (LDD), or dysplastic cerebellar gangliocytoma (DCG), is a rare tumor originating from the cerebellar cortex. LDD is a benign neuroglial tumor with uncertain prognosis. Over 200 cases have been reported in the literature mostly in the form of case reports. Thus, we present a spectacular case of LDD with excessive calcification in a female septuagenarian. PATIENT CONCERNS: A 72-year-old female presented with progressive dizziness for 8 months and suffered a head and sacrococcygeal region injury 20 days prior to her admission in our neurosurgery department. DIAGNOSIS: Computed tomography scan showed a right nonspecific cerebellar mass with striated calcification. Magnetic resonance imaging revealed a right "tiger-striped" alteration of the cerebellar cortex. H&E staining revealed a low grade glial neural tumor which was consistent with the diagnosis of LDD or DCG. INTERVENTION: The lesion was total resected. OUTCOMES: The patient recovered well and the cerebellar dysfunctional symptoms subsided 3 months after the operation and 2 years follow-up revealed no recurrence of the lesion and no neurological deficits. LESION: We postulate that the calcification of LDD is age-related and the pathogenesis of disease often observed in young adulthood.

Localized Congenital Cutis Verticis Gyrate with Skull Erosion.

Localized congenital cutis verticis gyrate (CVG) is rare and potentially risks skull involvement. A 23-year-old woman presented with a congenital scalp mass in the occipital region. Local thickening of her left occipital scalp with ridges and furrows was observed on examination. Head computed tomography scan showed a lytic area underneath the same area of the occipital calvarium. The mass was surgically removed due to the skull erosion and cosmetic reasons. Pathologic evaluation established CVG. Surgical excision is best for localized congenital CVG with skull erosion due to cosmetic reasons. Surgical excision was rewarding to the patient it allowed her to style her hair.

Giant Brain Arteriovenous Malformation Managed by Staged Embolizations and Surgical Resection with Satisfactory Outcome.

Spetzler-Martin grade V (>6 cm) arteriovenous malformations (AVMs) are traditionally considered inoperable. A 35-year-old man presented with repeated seizures for 7 years, and computed tomography arteriography and magnetic resonance imaging revealed left deep hemispheric AVM. A combination of embolization and surgical resection successfully achieved a cure of the patient. Well-equipped neurosurgery facilities can best manage selective Spetzler-Martin grade V AVMs with no neurologic deficits contrary to their traditionally inoperable concept. Successful surgery offers the patient a better quality of life.

Rapid disappearance of acute subdural hematoma due to abrogated hyper-fibrinolytic activity by tranexamic acid: Case report.

RATIONALE: Acute subdural hematoma (ASDH) occurs after tearing of bridging veins within the dura resulting in the accumulation of blood between the arachnoid and dura layers within 72 hours after traumatic head injury. Also, antigen fibrin D-dimer (DD) is the principal enzymatic degradation product of cross-linked fibrin by plasmin. We observed that early tranexamic acid (TXA) treatment resolved hyper-fibrinolysis and rapid disappearance ASDH. PATIENTS CONCERNS: A 48-year-old female presented with unconsciousness for 2 hours after head trauma. Her Glasgow Coma Scale score was >8 points. DIAGNOSIS: Computed tomography scan established ASDH with midline shift and brainstem compression and surgery was scheduled. Also, laboratory results indicated high DD spike of 34,820 µg/L and a reduction in plasma fibrinogen 1 hour after the injury. INTERVENTION: She was treated with intravenous TXA immediately after admission. OUTCOMES: Her DD spike decreased remarkably in 48 hours with associated rapid disappearance of ASDH thereby averting surgical intervention. She recovered fully with no long-term complications. LESSONS: Historically, hyper-fibrinolysis is associated with poor outcome in head trauma. However, early initiation of TXA which is noninvasive treatment modality for ASDH could avert surgery and reduce cost, anesthesia, and other complications associated with surgery.

Targeting protein kinases for the treatment of Alzheimer's disease: Recent progress and future perspectives.

Alzheimer's disease (AD) is a serious neurodegenerative disease characterized by memory impairment, mental retardation, impaired motor balance, loss of self-care and even death. Among the complex and diverse pathological changes in AD, protein kinases are deeply involved in abnormal phosphorylation of Tau proteins to form intracellular neuronal fiber tangles, neuronal loss, extracellular ß-amyloid (Aß) deposits to form amyloid plaques, and synaptic disturbances. As a disease of the elderly, the growing geriatric population is directly driving the market demand for AD therapeutics, and protein kinases are potential targets for the future fight against AD. This perspective provides an in-depth look at the role of the major protein kinases (GSK-3ß, CDK5, p38 MAPK, ERK1/2, and JNK3) in the pathogenesis of AD. At the same time, the development of different protein kinase inhibitors and the current state of clinical advancement are also outlined.

Spatial transcriptomics reveals niche-specific enrichment and vulnerabilities of radial glial stem-like cells in malignant gliomas.

Diffuse midline glioma-H3K27M mutant (DMG) and glioblastoma (GBM) are the most lethal brain tumors that primarily occur in pediatric and adult patients, respectively. Both tumors exhibit significant heterogeneity, shaped by distinct genetic/epigenetic drivers, transcriptional programs including RNA splicing, and microenvironmental cues in glioma niches. However, the spatial organization of cellular states and niche-specific regulatory programs remain to be investigated. Here, we perform a spatial profiling of DMG and GBM combining short- and long-read spatial transcriptomics, and single-cell transcriptomic datasets. We identify clinically relevant transcriptional programs, RNA isoform diversity, and multi-cellular ecosystems across different glioma niches. We find that while the tumor core enriches for oligodendrocyte precursor-like cells, radial glial stem-like (RG-like) cells are enriched in the neuron-rich invasive niche in both DMG and GBM. Further, we identify niche-specific regulatory programs for RG-like cells, and functionally confirm that FAM20C mediates invasive growth of RG-like cells in a neuron-rich microenvironment in a human neural stem cell derived orthotopic DMG model. Together, our results provide a blueprint for understanding the spatial architecture and niche-specific vulnerabilities of DMG and GBM.

Surgical Treatment of Pineal Region Tumors: An 18 year-Experience at a Single Institution.

BACKGROUND: The pineal tumor was once considered as a restricted area for surgery. Such cases are rare, with many different opinions on surgical treatment. This study aimed to review our experience of tumor treatment in the pineal region and explore the optimal treatment strategy. METHODS: The clinical data of 72 patients with pineal tumors from January 1997 to May 2015 (18 years) were retrospectively analyzed. Preoperative preparation, pathology type, tumor resection rate, surgical approach, and follow-up outcomes were used as the indicators to evaluate the treatment efficacy. RESULTS: The Krause approach was used in 46 cases, the Poppen approach in 10 cases, and the transcallosal-lateral ventricle-choroid fissure approach in 16 cases. The postoperative pathological results were as follows: 24 cases of germinoma, 11 of teratoma, 15 of glioma, 6 of meningioma, 11 of Pineocytoma, 2 of cholesteatoma, 2 of cavernous hemangioma, and 1 of choriocarcinoma. Further, the study included 64 cases of total surgical resections, 8 of subtotal resections, and 2 deaths. The follow-up period was from 7 months to 10 years. Further, 51 (70.8%) patients were followed up. The multivariate regression model showed that the surgical method and the pathological type contributed significantly to predicting outcomes. CONCLUSIONS: The type of pathology, extent of excision, and surgical approach had a significant impact on the prognosis of patients. The transcallosal-lateral ventricle-choroid fissure approach for large and medium-sized pineal tumors near the posterior part of the third ventricle had good efficacy.

Does preoperative gamma knife treatment affect the result of microresection of vestibular schwannoma?

PURPOSE: We aimed to explore the influence of preoperative gamma knife treatment on the clinical effect of microsurgical resection of vestibular schwannoma. METHODS: The data of patients who underwent vestibular schwannoma resection in our hospital between November 2010 and December 2019 were retrospectively collected. According to the data collected retrospectively and the inclusion and exclusion criteria, we selected these patients and divided them into Group A (with preoperative gamma knife treatment) and Group B (without preoperative gamma knife treatment). The pre/postoperative clinical manifestations, neurological function grade, postoperative complications, tumor recurrence and increase were collected and compared between the two groups. RESULTS: There were 40 and 823 patients enrolled in Groups A and B, respectively. There were no significant differences in the general condition, tumor size and side, or neurological performance of the patients in those two groups before the operation. At the last follow-up, the number of patients with poor facial nerve function was 15 (39.5%) in Group A and 170 (20.7%) in Group B (P = 0.021 < 0.05). In Group A and Group B, disequilibrium occurred in 14 (36.8%) patients and 124 (15.1%) patients, respectively, after the operation (P = 0.012 < 0.05). Seven (17.5%) patients had pneumonia in Group A, and 21 (2.6%) patients had pneumonia in Group B (P = 0.04 < 0.05) after the operation. CONCLUSION: When a patient with vestibular schwannoma undergoes microsurgical surgery, the preoperative history with gamma knife treatment may make recovery from postoperative facial paralysis difficult for the patients, making them more prone to suffer from postoperative disequilibrium and postoperative pneumonia.

The impact of residual hematoma after evacuation on the outcomes of patients with ruptured intracranial aneurysms with intracerebral hematoma: A longitudinal single-center observational study.

Intracerebral hematoma (ICH) as a result of ruptured of intracranial aneurysms often arises in patients with subarachnoid hemorrhage. Few studies focused on risk factors for ICH and not the impact of residual hematoma after evacuation on the outcomes of the patients. Therefore, 2 questions need to be answered: does residual hematoma after evacuation have impact on the outcome of patients who present with ICH as a result of ruptured intracranial aneurysms? Is radical pursuit of the hematoma necessary? The study was a single-center longitudinal observational type. Data of 2044 consecutive patients with subarachnoid hemorrhage from January 2009 to December 2019 were reviewed. ICHs were established and the locations of aneurysms as well as hematoma volumes were measured by computed tomographic scan before aneurysm occlusion. Only patients who received aneurysm clipping were included. Patients were stratified into hematoma evacuation without residuals versus residual hematoma after evaluation groups, and outcome was assessed according to the modified Rankin Scale (mRS) at 6 months. Out of the 1365 patients who received clipping, 476 patients presented in poor grade, whereas 889 patients' good grade. Our mRS scores revealed that patients who attained hematoma evacuation without residuals in the good-grade category attained better functional outcome than those with residual hematoma after evacuation. Contrarily, our mRS scores did not establish any significant difference in outcome between the poor-grade patients with hematoma evacuation without residuals and patients with residual hematoma after evacuation. Furthermore, our logistic regression model showed that advance age, poor Hunt-Hess grade, and vascular injury due to surgery were contributing factors for poor outcome of patients with ICH. Our data suggested that aggressive hematoma evacuation may not benefit the poor-grade patients. Majority of poor outcomes were due to surgical complications which were vascular related as a result of excessive pursuit of ICH.

Hemangioblastoma masquerading as a ring enhancing lesion in the cerebellum: A case report.

RATIONALE: Hemangioblastomas (HGBMs) are very rare, and the cerebellum is usually the most common site of occurrence. HGBMs with ring-enhanced walls are often misdiagnosed as metastases, abscesses, glioblastomas, tuberculomas, and demyelinating diseases. Thus, we present a rare case of HGBM masquerading as a ring-enhancing lesion in the cerebellum. PATIENT CONCERNS: We present a 33-year-old female who was admitted to our department because of headaches, unstable walking, and visual loss in both eyes. Cranial nerve examination revealed deficits in cranial nerve II. DIAGNOSIS: Magnetic resonance imaging revealed 2 cystic lesions in the cerebellum, with irregular ring-enhanced cyst walls composed of smaller nodular parts. Immunohistochemical staining of resected specimens established HGBM. INTERVENTIONS: The lesions were completely resected using a right retrosigmoid approach. OUTCOMES: Two years of follow-up revealed no recurrence of her symptoms or tumor. She is currently well and performs her daily duties. LESSONS: HGBMs with enhanced cysts are often misdiagnosed by radiology because of their ring-enhanced nature. Computed tomography angiography may be the best modality for differentiating cerebellar HGBM from other ring-enhancing lesions. Surgery is the gold standard of treatment for these lesions.

A petroclival glioma mimicking trigeminal schwannoma: A case report.

RATIONALE: Glioma in the petroclival region is very rare. Also, very few cases of primary gliomas have been reported to have radiographic as well as intraoperative features of extra-axial lesions resulting in diagnostic dilemma in the literature. We present a rare case of petroclival glioma mimicking trigeminal schwannoma in a young female. PATIENT CONCERNS: We present a 21-years old female with a 3-month history of pain in the right eye with no visual impairment. Cranial nerves examination revealed mild deficits in the trigeminal nerve, facial nerve, auditory nerve, oculomotor as well as the trochlear nerve. DIAGNOSES: Magnetic resonance imaging detected an extra-axial mass with mixed signal intensities in the right petroclivus area. Immunohistochemical established glioma with world health organization (WHO) grade II. INTERVENTIONS: The lesion was resected via 2 successive operations in 6 months interval. The patient was further treated with radiotherapy and post-radiotherapy temozolamide. OUTCOMES: Two years follow-up revealed no recurrence of the lesions and she is well. Nevertheless, he is still being followed diligently to uncover any recurrence. LESSONS: The extra-axial nature as well as petroclival location of the glioma makes our case very unique and very rare. The imaging characteristics were very extraordinary for a glioma which resulted in diagnostic dilemma. Thus, the definitive diagnosis was based on the histopathological evaluation of the excised tumor.

Radiation induced glioma in a sexagenarian: A case report.

INTRODUCTION: Radiation induced gliomas often occurs after radiation therapy for other brain tumors. Medulloblastoma often occurs in children and its associated radiation-induced glioblastoma multiforme's (GBM) after radiotherapy often has a long latency period. Our case is very unique because the medulloblastoma was detected at an advance age and the latency period of radiation-induced GBM was relatively shorter. PATIENTS CONCERNS: A 64-year-old male was first admitted at our hospital in March 2018 with dizziness, vomiting, and blurred vision. DIAGNOSIS: Magnetic resonance imaging of brain revealed a lesion with local mixed density and mass enhancement in left cerebellar region. Histopathology established medulloblastoma (World Health Organization) grade 4 and a classic histological subtype after surgery. INTERVENTION: Surgical resection followed by radiation therapy were the initial therapeutic modalities. OUTCOMES: In April 2019, the patient was readmitted with dizziness and blurred vision. Magnetic resonance imaging showed the left cerebellar hemisphere bulky enhancement lesion. Again, a multimodal therapy comprising surgical resection, radiation therapy as well as chemotherapy was adapted after histopathology established GBM. LESION: Radiotherapy for medulloblastoma patients at advance ages is a critical predisposing factor for the development of radiation-induced GBM in a very short period of time. We suggest that, radiotherapy as adjuvant therapy for medulloblastoma patients at advance ages should be chosen with extreme caution.

Foramen magnum meningioma with excessive calcification and no dura tail sign: A case report.

RATIONALE: Foramen magnum meningiomas are very rare lesions. They frequently originate from the arachnoid cells at the dura matter of the craniocervical junction. Foramen magnum meningiomas are challenging for neurosurgeons because of the complex anatomy of foramen magnum. We present a rare case of FMM with excessive calcification and without the dura tail sign which made the lesion mimic a teratoma. PATIENTS CONCERNS: A 63 years old woman presented with progressive numbness and hyperesthesia of the shoulders and upper limbs for 2 and half years. She also experienced occasional headaches and dizziness with no nausea, vomiting or fever. DIAGNOSES: Computed tomography scan, and magnetic resonance imaging revealed a calcified mass at occipital cistern. The lesion did not show the usual "dura tail sign" which made it mimic a teratoma on magnetic resonance imaging. Histopathology established meningioma. INTERVENTION: The tumor was completely resected via suboccipital approach. OUTCOMES: Two years follow-up revealed no recurrence of the lesion and no neurological deficits. LESSONS: We advocate the use of electromyographic and auditory brainstem responses to monitor the inferior cranial nerves because the tumor often adheres to these nerves.

Risk factors for hydrocephalus following fourth ventricle tumor surgery: A retrospective analysis of 121 patients.

BACKGROUND AND AIM: Most patients who present with a fourth ventricle tumor have concurrent hydrocephalus, and some demonstrate persistent hydrocephalus after tumor resection. There is still no consensus on the management of hydrocephalus in patients with fourth ventricle tumor after surgery. The purpose of this study was to identify the factors that predispose to postoperative hydrocephalus and the need for a postoperative cerebrospinal fluid (CSF) diversion procedure. MATERIALS AND METHODS: We performed a retrospective analysis of patients who underwent surgery of the fourth ventricle tumor between January 2013 and December 2018 at the Department of Neurosurgery in West China Hospital of Sichuan University. The characteristics of patients and the tumor location, tumor size, tumor histology, and preventive external ventricular drainage (EVD) that were potentially correlated with CSF circulation were evaluated in univariate and multivariate analysis. RESULTS: A total of 121 patients were enrolled in our study; 16 (12.9%) patients underwent postoperative CSF drainage. Univariate analysis revealed that superior extension (p = 0.004), preoperative hydrocephalus (p<0.001), and subtotal resection (p<0.001) were significantly associated with postoperative hydrocephalus. Multivariate analysis revealed that superior extension (p = 0.013; OR = 44.761; 95% CI 2.235-896.310) and subtotal resection (p = 0.005; OR = 0.087; 95% CI 0.016-0.473) were independent risk factors for postoperative hydrocephalus after resection of fourth ventricle tumor. CONCLUSION: Superior tumor extension (into the aqueduct) and failed total resection of tumor were identified as independent risk factors for postoperative hydrocephalus in patients with fourth ventricle tumor.

Outcomes of patients undergoing craniotomy and decompressive craniectomy for severe traumatic brain injury with brain herniation: A retrospective study.

The treatment of severe traumatic brain injury (TBI) with brain herniation is challenging because outcomes are often associated with high mortality and morbidity. Our aim was to identity factors contributing to decompressive craniectomy (DC) and evaluate treatment outcomes in patients with severe TBI with brain herniation.In this retrospective study, we analyzed medical records of severe TBI with brain herniation from May 2009 to December 2013. We reviewed their demographic data, mechanism of injury, Glasgow Coma Scale (GCS) score, pupil status, computed tomography findings, surgical treatment methods, time interval between brain herniation and surgery, as well as outcomes. GCS and pupil status are clinical parameters for detecting increase intracranial pressure while brain parenchyma bulged above the inner plate of the skull during operation indicated brain swelling as well as increased intracranial pressure on which basis the decision to perform DC or craniotomy was determined intraoperatively.One hundred ninety-four patients were included in the study. We performed DC in 143 of the patients while 51 of them we performed craniotomy. There were no statistically significant differences in the age, gender, or injury mechanism between the 2 groups. GCS, pupillary dilation, midline shift, hematoma type and timing of surgery were associated with DC. Nevertheless, logistic regression analysis revealed that hematoma type and timing of surgery were significantly associated with favorable DC outcomes (P < .001 and P = .023). Subdural hematoma and timing of surgery >1 hour were both identified as risk factors for DC. Six months after TBI, 34.0% of patients exhibited favorable outcomes. Overall mortality rate was 30.4%. Age, GCS, pupil dilation, hematoma type, and timing of surgery were all associated with patient outcomes. Further logistic regression analysis revealed that, lower GCS, bilateral pupil dilation, timing of surgery >1 hour, and advanced age were independent risk factors for poor outcomes (P = .001, P = .037, P = .028, and P = .001, respectively).Our study revealed that, DC is not mandatory for all TBI patients with brain herniation. Nevertheless, DC decreases mortality rate in severe TBI patients with brain herniation. Subdural hematoma and timing of surgery >1 hour are key indicators for DC. Lower GCS, bilateral pupil dilation, delayed timing of surgery and advance age are indicators of poor outcomes.