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Journal of Neurosciences in Rural... 2023
PubMed: 37692830
DOI: 10.25259/JNRP-2022-2-32 -
Simultaneous Bilateral Ophthalmic Artery Chemosurgery for Bilateral Retinoblastoma (Tandem Therapy).PloS One 2016Report on the 7-year experience with bilateral ophthalmic artery chemosurgery (OAC-Tandem therapy) for bilateral retinoblastoma.
OBJECTIVE
Report on the 7-year experience with bilateral ophthalmic artery chemosurgery (OAC-Tandem therapy) for bilateral retinoblastoma.
DESIGN
Retrospective, single institution study.
SUBJECTS
120 eyes of 60 children with bilateral retinoblastoma treated since March 2008.
METHODS
Retrospective review of all children treated at Memorial Sloan Kettering with bilateral ophthalmic artery chemosurgery (Melphalan, Carboplatin, Topotecan, Methotrexate) delivered in the same initial session to both naïve and previously treated eyes.
MAIN OUTCOME MEASURES
Ocular survival, metastatic disease, patient survival from metastases, second cancers, systemic adverse effects, need for transfusion of blood products, electroretinogram before and after treatment.
RESULTS
116 eyes were salvaged (4 eyes were enucleated: 3 because of progressive disease, 1 family choice). Kaplan Meier ocular survival was 99.2% at one year, 96.9% at 2 and 3 years and 94.9% for years 4 through 7. There were no cases of metastatic disease or metastatic deaths with a mean follow-up of 3.01 years. Two children developed second cancers (both pineoblastoma) and one of them died. Transfusion of blood products was required in 3 cases (4 transfusions), 1.9%. Two children developed fever/neutropenia requiring hospitalization (0.95%). ERGs were improved in 21.6% and unchanged after treatment in 52.5% of cases (increase or decrease of less than 25μV).
CONCLUSIONS
Bilateral ophthalmic artery chemosurgery is a safe and effective technique for managing bilateral retinoblastoma-even when eyes are advanced bilaterally, and if both eyes have progressed after systemic chemotherapy. Ocular survival was excellent (94.9% at 8 years), there were no cases of of metastatic disease and no deaths from metastatic disease, but children remain at risk for second cancers. In 21.6% of cases ERG function improved. Despite using chemotherapy in both eyes in the same session, systemic toxicity was low.
Topics: Carboplatin; Child; Child, Preschool; Electroretinography; Female; Humans; Kaplan-Meier Estimate; Male; Melphalan; Ophthalmic Artery; Retinal Neoplasms; Retinoblastoma; Retrospective Studies; Topotecan
PubMed: 27258771
DOI: 10.1371/journal.pone.0156806 -
Cureus May 2023Pineal tumors are quite rare and are fairly aggressive tumors seen in young adults and children. These tumors arise from the pineal region or recess from various types...
Pineal tumors are quite rare and are fairly aggressive tumors seen in young adults and children. These tumors arise from the pineal region or recess from various types of cells in the gland and structures located in close propinquity to the gland. Pineal gland tumors have a heterogeneous spectrum that includes pineal parenchymal tumors (PPTs) and papillary tumors of the pineal region (PTPR). The PPTs are further subclassified into pineocytomas (Grade 1), PPTs of intermediate differentiation (grade 2 or 3), and pineoblastomas (grade 4) based on the World Health Organization (WHO) grades and histopathological features. We discuss the case of an 11-year-old male child who presented with complaints of headache for 15 days, vomiting for seven days, and diplopia for four days. On magnetic resonance imaging (MRI), a soft tissue density lesion was noticed in the posterior third ventricle region. Based on the location and the MRI findings, the differential diagnosis considered were a pineal lesion, a choroid plexus papilloma, or a meningioma. He underwent a right occipital ventriculoperitoneal shunt followed by total excision of the tumor, and the resected specimen was sent for histopathological examination. After pathologic examination, the diagnosis of pineoblastoma (grade 4) with features of a PPT of intermediate differentiation (grades 2-3) was revealed, and the same was confirmed on immunohistochemistry.
PubMed: 37273335
DOI: 10.7759/cureus.38495 -
AJNR. American Journal of Neuroradiology Nov 2019Supratentorial primitive neuroectodermal tumors and pineoblastomas have traditionally been grouped together for treatment purposes. Molecular profiling of these tumors...
MRI Features of Histologically Diagnosed Supratentorial Primitive Neuroectodermal Tumors and Pineoblastomas in Correlation with Molecular Diagnoses and Outcomes: A Report from the Children's Oncology Group ACNS0332 Trial.
BACKGROUND AND PURPOSE
Supratentorial primitive neuroectodermal tumors and pineoblastomas have traditionally been grouped together for treatment purposes. Molecular profiling of these tumors has revealed a number of distinct entities and has led to the term "CNS-primitive neuroectodermal tumors" being removed from the 2016 World Health Organization classification. The purpose of this study was to describe the MR imaging findings of histologically diagnosed primitive neuroectodermal tumors and pineoblastomas and correlate them with molecular diagnoses and outcomes.
MATERIALS AND METHODS
Histologically diagnosed primitive neuroectodermal tumors and pineoblastomas were enrolled in this Children's Oncology Group Phase III trial, and molecular classification was retrospectively completed using DNA methylation profiling. MR imaging features were systematically studied and correlated with molecular diagnoses and survival.
RESULTS
Of the 85 patients enrolled, 56 met the inclusion criteria, in whom 28 tumors were in pineal and 28 in nonpineal locations. Methylation profiling revealed a variety of diagnoses, including pineoblastomas ( = 27), high-grade gliomas ( = 17), embryonal tumors ( = 7), atypical teratoid/rhabdoid tumors ( = 3), and ependymomas ( = 2). Thus, 39% overall and 71% of nonpineal tumor diagnoses were discrepant with histopathology. Tumor location, size, margins, and edema were predictors of embryonal-versus-nonembryonal tumors. Larger size and ill-defined margins correlated with poor event-free survival, while metastatic disease by MR imaging did not.
CONCLUSIONS
In nonpineal locations, only a minority of histologically diagnosed primitive neuroectodermal tumors are embryonal tumors; therefore, high-grade glioma or ependymoma should be high on the radiographic differential. An understanding of molecularly defined tumor entities and their relative frequencies and locations will help the radiologist make more accurate predictions of the tumor types.
Topics: Adolescent; Brain Neoplasms; Child; Child, Preschool; Female; Glioma; Humans; Infant; Magnetic Resonance Imaging; Male; Neoplasms, Germ Cell and Embryonal; Neuroectodermal Tumors, Primitive; Pineal Gland; Pinealoma; Retrospective Studies; Rhabdoid Tumor; Supratentorial Neoplasms; Teratoma; Young Adult
PubMed: 31601576
DOI: 10.3174/ajnr.A6253 -
Journal of Korean Neurosurgical Society Oct 2015Pineal parenchymal tumors (PPTs) in adults are rare, and knowledge regarding their optimal management and treatment outcome is limited. Herein, we present the clinical...
OBJECTIVE
Pineal parenchymal tumors (PPTs) in adults are rare, and knowledge regarding their optimal management and treatment outcome is limited. Herein, we present the clinical results of our series of PPTs other than pineoblastomas managed by stereotactic radiosurgery (SRS) at upfront setting.
METHODS
Between 1997 and 2014, nine consecutive adult patients with the diagnosis of PPTs, either pineocytoma or pineal parenchymal tumor of intermediate differentiation, were treated with SRS. There were 6 men and 3 women. The median age was 39 years (range, 31-53 years). All of the patients presented with symptoms of hydrocephalus. Endoscopic third ventriculostomy and biopsy was done for initial management. After histologic diagnosis, patients were treated with Gamma Knife with the mean dose of 13.3 Gy (n=3) or fractionated Cyberknife with 32 Gy (n=6).
RESULTS
After a mean follow-up of 78.6 months (range, 14-223 months), all patients were alive and all of their tumors were locally controlled except for one instance of cerebrospinal fluid seeding metastasis. On magnetic resonance images, tumor size decreased in all patients, resulting in complete response in 3 patients and partial response in 6. One patient had experienced temporary memory impairment after SRS, which improved spontaneously.
CONCLUSION
SRS is effective and safe for PPTs in adults and can be considered as a useful alternative to surgical resection at upfront setting.
PubMed: 26587186
DOI: 10.3340/jkns.2015.58.4.334 -
Frontiers in Oncology 2021Pineal gland tumors are exceedingly rare and account for 0.4-1.0% of brain neoplasms. Their rarity has confounded a clear understanding of the prognostic factors and...
INTRODUCTION
Pineal gland tumors are exceedingly rare and account for 0.4-1.0% of brain neoplasms. Their rarity has confounded a clear understanding of the prognostic factors and standards of care for these neoplasms. In this study, we aimed to investigate the incidence, prognostic indicators, and survival trend of tumors emanating from the pineal gland.
METHODS
We accessed the Surveillance, Epidemiology, End Results (SEER) Program for pineal gland tumors from 1975-2016. A multivariate Cox regression model was used to investigate the impact of clinicopathological parameters on all-cause mortality. For survival trend analysis, we employed the Kaplan Meier curve and pairwise comparisons to examine the trend.
RESULTS
We found 1,792 and 310,003 pineal gland and brain neoplasms during 1975-2016 resulting in an incidence of 0.6%. In the multivariate Cox proportional hazards model, older age, male gender, non-germ cell tumor, and receipt of chemotherapy were significantly associated with poor survival ( < 0.001). The extent of resection and radiotherapy administration did not produce survival advantages. Our result also highlighted an increased survival of pineal gland tumors over the years.
CONCLUSION
Our study investigated the prognostic factors that influenced survival in patients with pineal gland tumors. Chemotherapy use adversely affected patient outcomes and should be considered carefully in specific circumstances to avoid its harmful effects. These findings provide important evidence to improve current standards of care for this rare group of tumors. The survival of pineal tumors has improved over time reflecting improvements in current practice.
PubMed: 34869031
DOI: 10.3389/fonc.2021.780173 -
JPMA. the Journal of the Pakistan... Feb 2022To analyse the size and morphology of the normal pineal gland in the paediatric age group using magnetic resonance imaging.
OBJECTIVE
To analyse the size and morphology of the normal pineal gland in the paediatric age group using magnetic resonance imaging.
METHODS
The retrospective study was conducted at the Aga Khan University Hospital, Karachi, and comprised data of patients aged <18 years who had magnetic resonance imaging done between 2007 and 2017. Pineal size was estimated measuring its largest anteroposterior and supero-inferior diameters on sagittal T2-weighted sequences on any slice with maximal diameter. The width of pineal gland was measured on axial or coronal T2 sequences. The volume, morphology and enhancement patterns of the gland were also assessed. Data was analysed using SPSS 21.
RESULTS
Of the 200 patients, 116(58%) were males and 84(42%) were females. The overall mean age was 7.8±5.5 years. The mean anteroposterior dimension was 4.85±1.46mm, height 3.31±0.95mm, width 4.16±1.19, and volume 39.54±38.12 mm3. Mean size of cyst was 2.77±1.4mm (range: 1.2-7.5mm). No significant differences in the pattern of enhancement were found in different age groups (p>0.05).
CONCLUSIONS
Comprehensive knowledge of the size of the typical pineal organ is useful for radiologists in the identification of pineal gland anomalies and the exclusion of neoplastic lesions.
Topics: Adolescent; Body Height; Child; Child, Preschool; Cysts; Female; Humans; Magnetic Resonance Imaging; Male; Pineal Gland; Retrospective Studies
PubMed: 35320171
DOI: 10.47391/JPMA.314 -
Frontiers in Oncology 2022Lu-DOTATATE, a radionuclide therapy that binds somatostatin type-2A receptors (SST2A), has demonstrated efficacy in neuroendocrine tumors and evidence of central nervous...
Immunohistochemical assessment and clinical, histopathologic, and molecular correlates of membranous somatostatin type-2A receptor expression in high-risk pediatric central nervous system tumors.
INTRODUCTION
Lu-DOTATATE, a radionuclide therapy that binds somatostatin type-2A receptors (SST2A), has demonstrated efficacy in neuroendocrine tumors and evidence of central nervous system (CNS) penetration, supporting potential expansion within pediatric neuro-oncology. Understanding the prevalence of SST2A expression across pediatric CNS tumors is essential to identify patients who may benefit from somatostatin receptor-targeted therapy and to further elucidate the oncogenic role of SST2A.
METHODS
SST2A immunohistochemistry (IHC) was performed on tumor specimens and interpreted by an experienced pathologist (blinded), utilizing semi-quantitative scoring of membranous expression within viable tumor. Immunoreactive cell percentage was visually scored as 0 (none), 1 (<10%), 2 (10-50%), 3 (51-80%), or 4 (>80%). Staining intensity was scored as 0 (none), 1 (weak), 2 (moderate), or 3 (strong). Combined scores for each specimen were calculated by multiplying percent immunoreactivity and staining intensity values (Range: 0-12).
RESULTS
A total of 120 tumor samples from 114 patients were analyzed. Significant differences in SST2A IHC scores were observed across histopathologic diagnoses, with consistently high scores in medulloblastoma (mean ± SD: 7.5 ± 3.6 [n=38]) and meningioma (5.7 ± 3.4 [n=15]), compared to minimal or absent expression in ATRT (0.3 ± 0.6 [n=3]), ETMR (1.0 ± 0 [n=3]), ependymoma (grades I-III; 0.2 ± 0.7 [n=27]), and high-grade glioma (grades III-IV; 0.4 ± 0.7 [n=23]). Pineoblastoma (3.8 ± 1.5 [n=4]) and other embryonal tumors (2.0 ± 4.0 [n=7]) exhibited intermediate, variable expression. Among medulloblastomas, SST2A IHC scores were higher in non-SHH (8.5 ± 3.1) than SHH (5.0 ± 3.3) molecular subgroups (p=0.033). In a subset of paired primary and recurrent specimens from four patients, SST2A IHC scores remained largely unchanged.
DISCUSSION
High membranous SST2A expression was demonstrated in medulloblastoma, meningioma, and some rarer embryonal tumors with potential diagnostic, biologic, and therapeutic implications. Somatostatin receptor-targeted therapy such as Lu-DOTATATE deserves further investigation in these highly SST2A-expressing pediatric CNS tumors.
PubMed: 36465400
DOI: 10.3389/fonc.2022.996489 -
Neurosurgical Focus: Video Jul 2021Pineal region tumors represent a formidable challenge to the neurosurgeon. Choosing the right approach is key to optimizing the extent of resection and minimizing...
Pineal region tumors represent a formidable challenge to the neurosurgeon. Choosing the right approach is key to optimizing the extent of resection and minimizing surgical morbidity. In this video, the authors show an interhemispheric transcallosal approach to a pineal region tumor in a 15-year-old boy. The advantage of this corridor over posterior approaches is that it provides a nice view of the tumor plane with the venous complex, especially while dissecting tumor from the anterior aspect of the internal cerebral veins on their vertical path. Thus, this approach represents a safe and effective alternative for selected pineal tumors. The video can be found here: https://stream.cadmore.media/r10.3171/2021.4.FOCVID2120.
PubMed: 36284903
DOI: 10.3171/2021.4.FOCVID2120 -
World Neurosurgery Nov 2019Deep-seated cerebral lesions have fascinated and frustrated countless surgical innovators since the dawn of the microneurosurgical era. To determine the optimal...
Deep-seated cerebral lesions have fascinated and frustrated countless surgical innovators since the dawn of the microneurosurgical era. To determine the optimal approach, the microneurosurgeon must take into account the characteristics and location of the pathological lesion as well as the operator's range of technical expertise. Increasingly, microneurosurgeons must select between multiple operative corridors that can provide access to the surgical target. Innovative trajectories have emerged for many indications that provide more flexible operative angles and superior exposure but result in longer working distances and more technically demanding maneuvers. In this article, we highlight 4 innovative surgical corridors and compare their strengths and weaknesses against those of more conventional approaches. Our goal is to use these examples to illustrate the following principles of microneurosurgical innovation: (1) discover more efficient and flexible exposures with superior working angles; (2) ensure maximal early protection of critical neurovascular structures; and (3) effectively handle target pathology with minimal disruption of normal tissues.
Topics: Craniotomy; Humans; Intracranial Arteriovenous Malformations; Medical Illustration; Meningeal Neoplasms; Meningioma; Microsurgery; Neurosurgical Procedures; Pinealoma; Skull Base Neoplasms
PubMed: 31284052
DOI: 10.1016/j.wneu.2019.06.210