Rare Cranial Nerve Schwannomas: A Retrospective Review of Nontrigeminal, Nonvestibular Cranial Nerve Schwannomas

Introduction: Intracranial schwannomas arising from non-trigeminal and non-vestibular sources are extremely rare constituting <0.8% of all schwannomas. In this article, we have analyzed our experience in the management of these rare tumors over a 10-year period. Material and Methods: There were a total of 16 cases, with 11 of them undergoing microsurgical resection and 5 undergoing stereotactic radiosurgery (SRS). Results: There were no fresh neurological deficit in any of these patients and two patients underwent postoperative SRS for residual tumor. One patient died due to postoperative septicemia. Conclusion: Knowledge of these lesions along with their clinicoradiological profile is essential to maintain a high index of suspicion and understand the nuances of treatment

Supratentorial Pure Cortical Ependymoma: An Unusual Lesion Causing Focal Motor Aware Seizure

Ependymomas usually arise from the ependymal lining cells of the ventricular system and central canal of the spinal cord. Supratentorial ependymoma is a rare entity with the variable clinical course. In a small number of cases, ependymoma arises from supratentorial parenchyma. Only a few cases are reported in the literature. We report a case of 3-year-old girl with left frontal mass. Total removal of the mass lesion was performed without any neurological deficit. Pathological examination of the excised tumor was consistent with anaplastic ependymoma. We have discussed management strategy of this rare entity

Comparative evaluation of cerebral gliomas using rCBV measurements during sequential acquisition of T1-perfusion and T2*-perfusion MRI.

ObjectiveTo assess the inter-technique agreement of relative cerebral blood volume (rCBV) measurements obtained using T1- and T2*-perfusion MRI on 3T scanner in glioma patients.MethodsA total of 49 adult patients with gliomas underwent both on T1- and T2*-perfusion in the same scanning session, and rCBV maps were estimated using both methods. For the quantitative analysis; Two independent observers recorded the rCBV values from the tumor as well as contralateral brain tissue from both T1- and T2*-perfusion. Inter-observer and inter-technique rCBV measurement agreement were determined by using 95% Bland-Altman limits of agreement and intra-class correlation coefficient (ICC) statistics.ResultsQualitative analysis of the conventional and perfusion images showed that 16/49 (32.65%) tumors showed high susceptibility, and in these patients T2*-perfusion maps were suboptimal. Bland-Altman plots revealed an agreement between two independent observers recorded rCBV values for both T1- and T2*-perfusion. The ICC demonstrated strong agreement between rCBV values recorded by two observers for both T2* (ICC = 0.96, p = 0.040) and T1 (ICC = 0.97, p = 0.026) perfusion and similarly, good agreement was noted between rCBV estimated using two methods (ICC = 0.74, PConclusionsIn the current study, T1- and T2*-perfusion showed similar diagnostic performance for discrimination of grade III and grade IV gliomas; however, T1-perfusion was found to be better for the evaluation of tumors with intratumoral hemorrhage, postoperative recurrent tumors, and lesions near skull base. We conclude that T1-perfusion MRI with a single dose of contrast could be used as an alternative to T2*-perfusion to overcome the issues associated with this technique in brain tumors for reliable perfusion quantification

Immunotherapy Resistance in Glioblastoma

Glioblastoma is the most common malignant primary brain tumor in adults. Despite treatment consisting of surgical resection followed by radiotherapy and adjuvant chemotherapy, survival remains poor at a rate of 26.5% at 2&nbsp;years. Recent successes in using immunotherapies to treat a number of solid and hematologic cancers have led to a growing interest in harnessing the immune system to target glioblastoma. Several studies have examined the efficacy of various immunotherapies, including checkpoint inhibitors, vaccines, adoptive transfer of lymphocytes, and oncolytic virotherapy in both pre-clinical and clinical settings. However, these therapies have yielded mixed results at best when applied to glioblastoma. While the initial failures of immunotherapy were thought to reflect the immunoprivileged environment of the brain, more recent studies have revealed immune escape mechanisms created by the tumor itself and adaptive resistance acquired in response to therapy. Several of these resistance mechanisms hijack key signaling pathways within the immune system to create a protumoral microenvironment. In this review, we discuss immunotherapies that have been trialed in glioblastoma, mechanisms of tumor resistance, and strategies to sensitize these tumors to immunotherapies. Insights gained from the studies summarized here may help pave the way for novel therapies to overcome barriers that have thus far limited the success of immunotherapy in glioblastoma

Plurihormonal PIT-1-Positive Pituitary Adenomas: A Systematic Review and Single-Center Series.

OBJECTIVE: The 2017 World Health Organization classification of pituitary adenomas identified the plurihormonal PIT-1-positive (PP1) adenoma as a distinct subtype. The reported data suggest that PP1 adenomas encompass the former class of silent subtype 3 (SS3) adenomas and might have an aggressive phenotype. In the present study, we summarized the current clinical data on PP1 and SS3 adenomas and compared the reported data with the data from a single institutional cohort. METHODS: Medline and Google Scholar were searched from 1990 to 2020 for clinical series of PP1 and SS3 adenomas in accordance with the PRISMA (preferred reporting items for systematic reviews and meta-analyses) guidelines. Studies were included if they had reported pituitary pathology as PP1 or SS3 adenomas and had reported the clinical outcomes after surgical intervention. To better define the PP1 phenotype compared with non-PP1 adenomas, we also reviewed the adenomas treated surgically at our institution from 2012 to&nbsp;2019. RESULTS: Of all the tumors reported in the studies as PP1 or SS3, 99% were macroadenomas and 18% were giant adenomas (&gt;4 cm). Of the reported patients, 31.8% had received radiotherapy, and 22.9% had undergone multiple surgeries for their pituitary tumor. In our single-center experience, 20 patients had an adenoma that met the criteria for a PP1 adenoma. Compared with the 1146 non-PP1 tumors, the PP1 tumors did not show statistically significant differences in the extent of resection, size, number of previous surgeries, future reoperations, rate of radiotherapy, p53 staining, or MIB-1 labeling index. CONCLUSIONS: The findings from the present large, single-center study comparing PP1 and non-PP1 adenomas do not suggest that PP1 tumors are more aggressive. Further work is warranted to identify the pathologic subtypes of pituitary adenomas that are consistently more clinically aggressive

Clinical characteristics and outcomes of null-cell versus silent gonadotroph adenomas in a series of 1166 pituitary adenomas from a single institution

Objective: Nonfunctioning pituitary adenomas present without biochemical or clinical signs of hormone excess and are the second most common type of pituitary adenomas. The 2017 WHO classification scheme of pituitary adenomas differentiates null-cell adenomas (NCAs) and silent gonadotroph adenomas (SGAs). The present study sought to highlight the differences in patient characteristics and clinical outcomes between NCAs and SGAs. Methods: The records of 1166 patients who underwent transsphenoidal surgery for pituitary adenoma between 2012 and 2019 at a single institution were retrospectively reviewed. Patient demographics and clinical outcomes were collected. Results: Of the overall pituitary adenoma cohort, 12.8% (n = 149) were SGAs and 9.2% (n = 107) NCAs. NCAs were significantly more common in female patients than SGAs (61.7% vs 26.8%, p \u3c 0.001). There were no differences in patient demographics, initial tumor size, or perioperative and short-term clinical outcomes. There was no significant difference in the amount of follow-up between patients with NCAs and those with SGAs (33.8 months vs 29.1 months, p = 0.237). Patients with NCAs had significantly higher recurrence (p = 0.021), adjuvant radiation therapy usage (p = 0.002), and postoperative diabetes insipidus (p = 0.028). NCA pathology was independently associated with tumor recurrence (HR 3.64, 95% CI 1.07–12.30; p = 0.038), as were cavernous sinus invasion (HR 3.97, 95% CI 1.04–15.14; p = 0.043) and anteroposterior dimension of the tumor (HR 2.23, 95% CI 1.09–4.59; p = 0.030). Conclusions: This study supports the definition of NCAs and SGAs as separate subgroups of nonfunctioning pituitary adenomas, and it highlights significant differences in long-term clinical outcomes, including tumor recurrence and the associated need for adjuvant radiation therapy, as well as postoperative diabetes insipidus. The authors also provide insight into independent risk factors for these outcomes in the adenoma population studied, providing clinicians with additional predictors of patient outcomes. Follow-up studies will hopefully uncover mechanisms of biological aggressiveness in NCAs and associated molecular targets

Clinical Characteristics and Outcomes of Null-cell Versus Silent Gonadotroph Adenomas in a Series of 1166 Pituitary Adenomas from a Single Institution

Objective: Nonfunctioning pituitary adenomas present without biochemical or clinical signs of hormone excess and are the second most common type of pituitary adenomas. The 2017 WHO classification scheme of pituitary adenomas differentiates null-cell adenomas (NCAs) and silent gonadotroph adenomas (SGAs). The present study sought to highlight the differences in patient characteristics and clinical outcomes between NCAs and SGAs. Methods: The records of 1166 patients who underwent transsphenoidal surgery for pituitary adenoma between 2012 and 2019 at a single institution were retrospectively reviewed. Patient demographics and clinical outcomes were collected. Results: Of the overall pituitary adenoma cohort, 12.8% (n = 149) were SGAs and 9.2% (n = 107) NCAs. NCAs were significantly more common in female patients than SGAs (61.7% vs 26.8%, p \u3c 0.001). There were no differences in patient demographics, initial tumor size, or perioperative and short-term clinical outcomes. There was no significant difference in the amount of follow-up between patients with NCAs and those with SGAs (33.8 months vs 29.1 months, p = 0.237). Patients with NCAs had significantly higher recurrence (p = 0.021), adjuvant radiation therapy usage (p = 0.002), and postoperative diabetes insipidus (p = 0.028). NCA pathology was independently associated with tumor recurrence (HR 3.64, 95% CI 1.07–12.30; p = 0.038), as were cavernous sinus invasion (HR 3.97, 95% CI 1.04–15.14; p = 0.043) and anteroposterior dimension of the tumor (HR 2.23, 95% CI 1.09–4.59; p = 0.030). Conclusions: This study supports the definition of NCAs and SGAs as separate subgroups of nonfunctioning pituitary adenomas, and it highlights significant differences in long-term clinical outcomes, including tumor recurrence and the associated need for adjuvant radiation therapy, as well as postoperative diabetes insipidus. The authors also provide insight into independent risk factors for these outcomes in the adenoma population studied, providing clinicians with additional predictors of patient outcomes. Follow-up studies will hopefully uncover mechanisms of biological aggressiveness in NCAs and associated molecular targets