Potential prognostic determinants for FET::CREB fusion-positive intracranial mesenchymal tumor.

Intracranial mesenchymal tumor (IMT), FET::CREB fusion-positive is a provisional tumor type in the 2021 WHO classification of central nervous system tumors with limited information available. Herein, we describe five new IMT cases from four females and one male with three harboring an EWSR1::CREM fusion and two featuring an EWSR1::ATF1 fusion. Uniform manifold approximation and projection of DNA methylation array data placed two cases to the methylation class "IMT, subclass B", one to "meningioma-benign" and one to "meningioma-intermediate". A literature review identified 74 cases of IMTs (current five cases included) with a median age of 23 years (range 4-79 years) and a slight female predominance (female/male ratio = 1.55). Among the confirmed fusions, 25 (33.8%) featured an EWSR1::ATF1 fusion, 24 (32.4%) EWSR1::CREB1, 23 (31.1%) EWSR1::CREM, one (1.4%) FUS::CREM, and one (1.4%) EWSR1::CREB3L3. Among 66 patients with follow-up information available (median: 17 months; range: 1-158 months), 26 (39.4%) experienced progression/recurrences (median 10.5 months; range 0-120 months). Ultimately, three patients died of disease, all of whom underwent a subtotal resection for an EWSR1::ATF1 fusion-positive tumor. Outcome analysis revealed subtotal resection as an independent factor associated with a significantly shorter progression free survival (PFS; median: 12 months) compared with gross total resection (median: 60 months; p < 0.001). A younger age (< 14 years) was associated with a shorter PFS (median: 9 months) compared with an older age (median: 49 months; p < 0.05). Infratentorial location was associated with a shorter overall survival compared with supratentorial (p < 0.05). In addition, the EWSR1::ATF1 fusion appeared to be associated with a shorter overall survival compared with the other fusions (p < 0.05). In conclusion, IMT is a locally aggressive tumor with a high recurrence rate. Potential risk factors include subtotal resection, younger age, infratentorial location, and possibly EWSR1::ATF1 fusion. Larger case series are needed to better define prognostic determinants in these tumors.

Neuroendoscopic Evacuation Improves Outcomes Compared with External Ventricular Drainage in Patients with Spontaneous Intraventricular Hemorrhage: A Systematic Review with Meta-Analyses.

BACKGROUND: Spontaneous intraventricular hemorrhage (IVH) is a cause of significant morbidity and mortality. Treatment for resulting obstructive hydrocephalus has traditionally been via an external ventricular drain (EVD). We aimed to compare patient outcomes after neuroendoscopic surgery (NES) evacuation of IVH versus EVD management. METHODS: MEDLINE, Embase, and Cochrane Library databases were searched on October 8, 2022. Of the 252 records remaining after removal of duplicates, 12 met study inclusion criteria. After extraction of outcomes data, fixed-effect and random-effects models were used to establish odds ratios (ORs) with 95% confidence intervals (CIs) for intensive care unit length of stay, rate of permanent cerebrospinal fluid diversion, Glasgow Outcome Scale score, and mortality rate. RESULTS: The results of the pooled analysis showed that intensive care unit length of stay was shorter (OR -2.61 [95% CI -5.02, -0.19]; I2 = 97.76%; P = 0.034), permanent cerebrospinal fluid diversion was less likely (OR -0.79, 95% CI [-1.17, -0.41], I2 = 46.96%, P < 0.001), higher Glasgow Outcome Scale score was more likely (OR 0.48, 95% CI [0.04, 0.93], I2 = 60.12%, P = 0.032), and all-cause mortality was less likely (OR -1.11, 95% CI [-1.79, -0.44], I2 = 0%, P = 0.001) in the NES evacuation group compared with the EVD group. CONCLUSIONS: NES for evacuation of spontaneous IVH results in reduced intensive care unit length of stay, reduced permanent cerebrospinal fluid diversion rates, improved Glasgow Outcome Scale score, and reduced mortality when compared with EVD. More robust prospective, randomized studies are necessary to help inform the safety and utility of NES for IVH.

Presacral mature cystic teratoma associated with Currarino syndrome in an adolescent with androgen insensitivity: illustrative case.

BACKGROUND: Currarino syndrome is a rare disorder that classically presents with the triad of presacral mass, anorectal malformation, and spinal dysraphism. The presacral mass is typically benign, although malignant transformation is possible. Surgical treatment of the mass and exploration and repair of associated dysraphism are indicated for diagnosis and symptom relief. There are no previous reports of Currarino syndrome in an androgen-insensitive patient. OBSERVATIONS: A 17-year-old female patient presented with lack of menarche. Physical examination and laboratory investigation identified complete androgen insensitivity. Imaging analysis revealed a presacral mass lesion, and the patient was taken to surgery for resection of the mass and spinal cord untethering. Intraoperative ultrasound revealed a fibrous stalk connecting the thecal sac to the presacral mass, which was disconnected without the need for intrathecal exploration. The presacral mass was then resected, and pathological analysis revealed a mature cystic teratoma. Postoperatively, the patient recovered without neurological or gastrointestinal sequelae. LESSONS: Diagnosis of incomplete Currarino syndrome may be difficult but can be identified via work-up of other disorders, such as androgen insensitivity. Intraoperative ultrasound is useful for surgical decision making and may obviate the need for intrathecal exploration during repair of dysraphism in the setting of Currarino syndrome.

Decision-Making and Management in a Patient With Coexistent Colloid Cyst and Pituitary Macroadenoma: A Case Report.

The coexistence of separate and distinct primary intracranial tumors is rare. Specifically, there are no previous reports of a colloid cyst coexisting with a pituitary macroadenoma. We present the case of a 40-year-old male with a colloid cyst associated with mild enlargement of the right lateral ventricle and a coexistent pituitary macroadenoma with compression of the optic apparatus. An endoscopic endonasal transsphenoidal surgery (EETS) for resection of the pituitary mass was performed first due to the patient's complaints of acute visual changes. He then underwent a right frontal craniotomy for resection of the colloid cyst one month later. The patient recovered without residual deficits in vision, and he did not require ventricular shunting after removal of the colloid cyst. We aimed to discuss our decision-making process and the management of these coexistent lesions.

Spontaneous Spinal Epidural Hematoma Associated With Apixaban Therapy: A Report of two Cases.

Spontaneous spinal epidural hematoma (SSEH) is a rare clinical entity that can result in severe neurological deficit and warrants emergent neurosurgical evaluation and management. The exact etiology of this entity remains unknown, but certain risk factors exist, including the use of anticoagulant medications. There are few published reports of the association of SSEH with direct factor Xa inhibitors. We aimed to present 2 cases of SSEH in patients on chronic apixaban therapy. To the best of our knowledge, there is only 1 other report of SSEH in the setting of apixaban therapy. A comparison between the cases suggests the importance of rapid recognition and management of SSEH in order to achieve favorable neurological outcomes.

Review of psychiatric comorbidities and their associations with opioid use in elective lumbar spine surgery.

The opioid epidemic is an ongoing concern in the United States and efforts to ameliorate this crisis are underway on multiple fronts. Opiate use is an important consideration for patients undergoing lumbar spine surgery with concurrent psychiatric diagnoses and more information is needed regarding the factors involved in these patients. That information may help guide opioid prescribing practices for individual patients with certain psychiatric conditions that are undergoing these procedures. This study was done to identify psychiatric conditions that are associated with preoperative and postoperative opioid use in this cohort of veteran patients undergoing elective lumbar spine surgery.A 3 month preoperative and 3 month postoperative chart review was conducted on 25 patients per year who underwent elective lumbar spine surgery over a 16-year period at the Veterans Affairs Nebraska-Western Iowa Healthcare Center (n = 376 after exclusion criteria applied). The association between psychiatric comorbidities and use of opioids during the 90-day period after surgery was assessed using a linear model that adjusted for surgical type, opioid use prior to surgery, and other relevant comorbidities.Patients are more likely to use opioids preoperatively if they have major depression (P = .02), hepatitis C (P = .01), or musculoskeletal disorders (P = .04). PTSD (P = .02) and lumbar fusion surgery (P < .0001) are associated with increased postoperative use, after adjusting for preoperative use and other comorbidities.Certain psychiatric comorbidities are significantly correlated with opioid use for this cohort of lumbar spine surgery patients in the preoperative and postoperative periods. Awareness of an individual's psychiatric comorbidity burden may help guide opioid prescription use.

Knockdown of SCF(Skp2) function causes double-parked accumulation in the nucleus and DNA re-replication in Drosophila plasmatocytes.

In Drosophila, circulating hemocytes are derived from the cephalic mesoderm during the embryonic wave of hematopoiesis. These cells are contributed to the larva and persist through metamorphosis into the adult. To analyze this population of hemocytes, we considered data from a previously published RNAi screen in the hematopoietic niche, which suggested several members of the SCF complex play a role in lymph gland development. eater-Gal4;UAS-GFP flies were crossed to UAS-RNAi lines to knockdown the function of all known SCF complex members in a plasmatocyte-specific fashion, in order to identify which members are novel regulators of plasmatocytes. This specific SCF complex contains five core members: Lin-19-like, SkpA, Skp2, Roc1a and complex activator Nedd8. The complex was identified by its very distinctive large cell phenotype. Furthermore, these large cells stained for anti-P1, a plasmatocyte-specific antibody. It was also noted that the DNA in these cells appeared to be over-replicated. Gamma-tubulin and DAPI staining suggest the cells are undergoing re-replication as they had multiple centrioles and excessive DNA content. Further experimentation determined enlarged cells were BrdU-positive indicating they have progressed through S-phase. To determine how these cells become enlarged and undergo re-replication, cell cycle proteins were analyzed by immunofluorescence. This analysis identified three proteins that had altered subcellular localization in these enlarged cells: Cyclin E, Geminin and Double-parked. Previous research has shown that Double-parked must be degraded to exit S-phase, otherwise the DNA will undergo re-replication. When Double-parked was titrated from the nucleus by an excess of its inhibitor, geminin, the enlarged cells and aberrant protein localization phenotypes were partially rescued. The data in this report suggests that the SCF(Skp2) complex is necessary to ubiquitinate Double-parked during plasmatocyte cell division, ensuring proper cell cycle progression and the generation of a normal population of this essential blood cell type.