-
Frontiers in Surgery 2021An endoscope-assisted technique was recently introduced to microsurgery (MS) and may compensate for the disadvantages of MS for deep-seated lesions. This study was...
An endoscope-assisted technique was recently introduced to microsurgery (MS) and may compensate for the disadvantages of MS for deep-seated lesions. This study was performed to identify the effectiveness and safety of endoscopic-assisted microsurgery (EAM) and share our experience of EAM for pediatric cases with pineal region tumors. We retrospectively analyzed the clinical data of consecutive pediatric cases with pineal region tumors treated by EAM or MS from January 2016 to June 2020. These data included the patient population, clinical manifestations, preoperative examination findings, surgical approach, pathological results, and clinical outcomes. The clinical outcomes were analyzed in the EAM group and MS group with a focus on the gross total resection (GTR) rate, postoperative hydrocephalus remission rate, and Karnofsky performance score (KPS). Studies on the surgical management of children with pineal region tumors in the last decade were reviewed. Eighteen children successfully underwent tumor resection via MS ( = 8) or EAM ( = 10). The children's mean age was 11.4 ± 4.7 years, and the male to female ratio was 7:2. Seventeen patients (94.4%) complicated preoperative hydrocephalus, and 16 (88.9%) presented headache with nausea and/or vomiting. The pathological examination revealed germ cell tumors in 11 (61.1%) patients, neuroepithelial tumors in 4 (22.2%) patients, and a pineoblastoma, arachnoid cyst, and atypical teratoid rhabdoid tumor in 1 (5.6%) patient each. GTR was more commonly achieved in the EAM than MS group (80.0 vs. 50.0%, respectively), and the postoperative hydrocephalus remission rate was higher in the EAM than MS group (87.5 vs. 50.0%, respectively). At a mean follow-up time of 23.6 ± 11.5 weeks, the mean improvement of the KPS 6 months postoperatively was greater in the EAM than MS group (24.0 ± 9.7 vs. 17.5 ± 7.1 points, respectively). EAM combines endoscopic and microsurgical techniques and can be safely and effectively performed to achieve GTR of pineal region tumors in pediatric patients. In children with pineal region tumors who have obstructive hydrocephalus, EAM could improves hydrocephalus remission rates by checking and clearing the midbrain aqueduct under visualization.
PubMed: 34414209
DOI: 10.3389/fsurg.2021.641196 -
Neurosurgical Focus: Video Jul 2021The authors present a pediatric case of a pineoblastoma treated with gross-total removal through an occipital interhemispheric transtentorial approach (OITA). The child...
The authors present a pediatric case of a pineoblastoma treated with gross-total removal through an occipital interhemispheric transtentorial approach (OITA). The child presented with acute hydrocephalus that was treated by endoscopic third ventriculostomy (ETV) and tumor biopsy through a single burr hole. Histology revealed a pineoblastoma. Microsurgical total removal was performed 3 months after neoadjuvant chemotherapy. OITA was chosen on the basis of the tumor's location below the Herophilus-Galen line of sight. In this video, the authors show the positioning, the operating devices, the approach, and the microsurgical dissection, indicating all the neurovascular structures encountered. The video can be found here: https://stream.cadmore.media/r10.3171/2021.4.FOCVID2122.
PubMed: 36284905
DOI: 10.3171/2021.4.FOCVID2122 -
Journal of Neurosurgery. Case Lessons Apr 2022Pineoblastomas are a rare and aggressive pediatric neuroectodermal tumor subtype. Because of their rarity, pineoblastomas are still poorly understood, and there is...
BACKGROUND
Pineoblastomas are a rare and aggressive pediatric neuroectodermal tumor subtype. Because of their rarity, pineoblastomas are still poorly understood, and there is little research delineating their molecular development and underlying genetic phenotype. Recent multiomic studies in pineoblastomas and pineal parenchymal tumors identified four clinically and biologically relevant consensus groups driven by signaling/processing pathways; however, molecular level alterations leading to these pathway changes are yet to be discovered, hence the importance of individually profiling every case of this rare tumor type.
OBSERVATIONS
The authors present the comprehensive somatic genomic profiling of a patient with pineoblastoma presenting with the loss of protein polybromo-1 (PBRM1) as a candidate genomic driver. Loss of PBRM1, a tumor suppressor, has been reported as a driver event in various cancer types, including renal cell carcinoma, bladder carcinoma, and meningiomas with papillary features.
LESSONS
This is the first report presenting biallelic loss of PBRM1 as a candidate molecular driver in relation to pineoblastoma.
PubMed: 36303510
DOI: 10.3171/CASE2213 -
Digital Journal of Ophthalmology : DJO Jun 2021
Topics: Adult; Humans; Magnetic Resonance Imaging; Male; Neoplasms, Germ Cell and Embryonal; Nystagmus, Pathologic; Pinealoma; Pituitary Neoplasms; Radiotherapy Dosage; Radiotherapy, Conformal; Visual Fields
PubMed: 34512208
DOI: 10.5693/djo.02.2020.12.002 -
Journal of Neuro-oncology Mar 2024Central nervous system (CNS) tumours account for around 25% of childhood neoplasms. With multi-modal therapy, 5-year survival is at around 75% in the UK. Conventional... (Review)
Review
BACKGROUND
Central nervous system (CNS) tumours account for around 25% of childhood neoplasms. With multi-modal therapy, 5-year survival is at around 75% in the UK. Conventional photon radiotherapy has made significant contributions to survival, but can be associated with long-term side effects. Proton beam radiotherapy (PBT) reduces the volume of irradiated tissue outside the tumour target volume which may potentially reduce toxicity. Our aim was to assess the effectiveness and safety of PBT and make recommendations for future research for this evolving treatment.
METHODS
A systematic review assessing the effects of PBT for treating CNS tumours in children/young adults was undertaken using methods recommended by Cochrane and reported using PRISMA guidelines. Any study design was included where clinical and toxicity outcomes were reported. Searches were to May 2021, with a narrative synthesis employed.
RESULTS
Thirty-one case series studies involving 1731 patients from 10 PBT centres were included. Eleven studies involved children with medulloblastoma / primitive neuroectodermal tumours (n = 712), five ependymoma (n = 398), four atypical teratoid/rhabdoid tumour (n = 72), six craniopharyngioma (n = 272), three low-grade gliomas (n = 233), one germ cell tumours (n = 22) and one pineoblastoma (n = 22). Clinical outcomes were the most frequently reported with overall survival values ranging from 100 to 28% depending on the tumour type. Endocrine outcomes were the most frequently reported toxicity outcomes with quality of life the least reported.
CONCLUSIONS
This review highlights areas of uncertainty in this research area. A well-defined, well-funded research agenda is needed to best maximise the potential of PBT.
SYSTEMATIC REVIEW REGISTRATION
PROSPERO-CRD42016036802.
Topics: Child; Humans; Young Adult; Proton Therapy; Quality of Life; Central Nervous System Neoplasms; Central Nervous System; Pituitary Neoplasms; Cerebellar Neoplasms
PubMed: 38294638
DOI: 10.1007/s11060-023-04510-4 -
Radiation Oncology (London, England) Sep 2014Standard treatment strategies for embryonal central nervous system (CNS) tumors have not yet been established. We treated these tumors using an original chemoradiation...
BACKGROUND
Standard treatment strategies for embryonal central nervous system (CNS) tumors have not yet been established. We treated these tumors using an original chemoradiation therapy protocol; the clinical outcomes and toxicities were retrospectively evaluated.
METHODS
Twenty-four patients were enrolled including sixteen with medulloblastoma, four with supratentorial primitive neuroectodermal tumor (sPNET), three with atypical teratoid/rhabdoid tumor, and one with pineoblastoma. Immediately after diagnosis, all patients underwent surgery initially. They were then categorized as high- or average-risk groups independent of tumor type/pathogenesis. The average-risk group included patients who were aged ≥3 years at diagnosis, had non-metastatic disease at diagnosis (M0), and had undergone gross total resection. Other patients were categorized as the high-risk group; this group received more intensive treatment than the average-risk group, including high-dose chemotherapy with autologous stem-cell transplantation. All patients received craniospinal irradiation (CSI). The CSI dose was 23.4 Gy for M0 patients aged ≥5 years, 18 Gy for M0 patients aged <5 years, and 30-36 Gy for all patients with M + disease. The total dose to the primary tumor bed was 54 Gy.
RESULTS
The median follow-up time was 73.5 (range, 19-118) months. The 5-year progression-free survival (PFS) and overall survival (OS) rates were 71.1 and 88.9%, respectively in the average-risk group (n = 9) and 66.7 and 71.1%, respectively in the high-risk group (n = 15). The PFS and OS rates were not significantly different between the average- and high-risk groups. In patients with medulloblastoma only, these rates were also not significantly different between the average- and high-risk groups. Three of four patients with sPNET were disease free. The height standard deviation score (SDS) was significantly decreased at the last assessment relative to that at diagnosis (P < 0.0001). The latest median height SDS was -1.6 (range, 0.9 to -4.8), and the latest median full-scale intelligence quotient (FSIQ) score was 86 (range, 59-128). The CSI doses and age at the start of radiation therapy did not influence clinical outcomes, height SDSs, and FSIQ scores.
CONCLUSIONS
Our original protocol for patients with embryonal CNS tumors was feasible and yielded favorable clinical outcomes.
Topics: Adolescent; Central Nervous System Neoplasms; Child; Child, Preschool; Combined Modality Therapy; Disease-Free Survival; Female; Humans; Infant; Kaplan-Meier Estimate; Male; Neoplasms, Germ Cell and Embryonal; Treatment Outcome
PubMed: 25209395
DOI: 10.1186/1748-717X-9-201 -
Journal of the Neurological Sciences Dec 2015The purpose of this study was to evaluate the morphologic magnetic resonance imaging (MRI) characteristics of the pineal gland in retinoblastoma (Rb) patients without...
PURPOSE
The purpose of this study was to evaluate the morphologic magnetic resonance imaging (MRI) characteristics of the pineal gland in retinoblastoma (Rb) patients without and with pineoblastoma in comparison to age-matched controls to improve early identification of pineoblastomas (trilateral retinoblastoma, TRb).
METHODS AND MATERIALS
80 patients with retinoblastoma and 80 age-matched controls who had undergone brain MRI were included in this retrospective institutional review board approved cohort study. Two readers analyzed the following MR characteristics of the pineal gland: signal intensity on T1- and T2-weighted images, enhancement pattern, delineation of the gland, presence of cystic component, size of pineal gland and size of pineal cysts, respectively. A third reader assessed all images for the presence or absence of pineoblastoma.
RESULTS
3 patients were positive (TRb cohort) and 77 negative for pineoblastoma (non-TRb cohort). The mean maximum diameter of the pineal gland was 6.4mm in Rb patients and 6.3mm in age-matched controls. The mean volume of the pineal gland in Rb patients was 93.1mm(3) and was 87.6mm(3) in age-matched controls. Considering all available MRI scans the mean maximum diameter of the pineal gland in TRb patients was 11.2mm and the mean volume in TRb patients was 453.3mm(3). The third reader identified pineoblastomas with a sensitivity of 100% (3 of 3) and a specificity of 94% (72 of 77).
CONCLUSION
Our non-TRb patients did not show significant differences in the size of the pineal gland and pineal gland cysts compared to age-matched controls. The presented data can serve as a reference for the volume of normal pineal glands and pineal cysts in the diagnostic work-up of Rb patients with suspected pineoblastoma.
Topics: Adolescent; Adult; Brain Neoplasms; Case-Control Studies; Child; Child, Preschool; Female; Follow-Up Studies; Humans; Imaging, Three-Dimensional; Infant; Infant, Newborn; Magnetic Resonance Imaging; Male; Pineal Gland; Pinealoma; Retinal Neoplasms; Retinoblastoma; Retrospective Studies; Young Adult
PubMed: 26671110
DOI: 10.1016/j.jns.2015.10.046 -
Neuro-oncology Apr 2017Pineoblastoma is a rare pineal region brain tumor. Treatment strategies have reflected those for other malignant embryonal brain tumors.
Evaluation of age-dependent treatment strategies for children and young adults with pineoblastoma: analysis of pooled European Society for Paediatric Oncology (SIOP-E) and US Head Start data.
BACKGROUND
Pineoblastoma is a rare pineal region brain tumor. Treatment strategies have reflected those for other malignant embryonal brain tumors.
PATIENTS AND METHODS
Original prospective treatment and outcome data from international trial groups were pooled. Cox regression models were developed considering treatment elements as time-dependent covariates.
RESULTS
Data on 135 patients with pineoblastoma aged 0.01-20.7 (median 4.9) years were analyzed. Median observation time was 7.3 years. Favorable prognostic factors were age ≥4 years (hazard ratio [HR] for progression-free survival [PFS] 0.270, P < .001) and administration of radiotherapy (HR for PFS 0.282, P < .001). Metastatic disease (HR for PFS 2.015, P = .006), but not postoperative residual tumor, was associated with unfavorable prognosis. In 57 patients <4 years old, 5-year PFS/overall survival (OS) were 11 ± 4%/12 ± 4%. Two patients survived after chemotherapy only, while 3 of 16 treated with craniospinal irradiation (CSI) with boost, and 3 of 5 treated with high-dose chemotherapy (HDCT) and local radiotherapy survived. In 78 patients aged ≥4 years, PFS/OS were 72 ± 7%/73 ± 7% for patients without metastases, and 50 ± 10%/55 ± 10% with metastases. Seventy-three patients received radiotherapy (48 conventionally fractionated CSI, median dose 35.0 [18.0-45.0] Gy, 19 hyperfractionated CSI, 6 local radiotherapy), with (n = 68) or without (n = 6) chemotherapy. The treatment sequence had no impact; application of HDCT had weak impact on survival in older patients.
CONCLUSION
Survival is poor in young children treated without radiotherapy. In these patients, combination of HDCT and local radiotherapy may warrant further evaluation in the absence of more specific or targeted treatments. CSI combined with chemotherapy is effective for older non-metastatic patients.
Topics: Adolescent; Adult; Antineoplastic Agents; Brain Neoplasms; Child; Child, Preschool; Combined Modality Therapy; Databases, Factual; Disease-Free Survival; Europe; Female; Humans; Infant; Male; Pineal Gland; Pinealoma; Prospective Studies; Treatment Outcome; United States; Young Adult
PubMed: 28011926
DOI: 10.1093/neuonc/now234 -
World Neurosurgery May 2023Obtaining a prompt diagnosis, avoiding indwelling ventriculoperitoneal shunt, and enhancing the predictive value of pathologic examinations are only some of the... (Review)
Review
Obtaining a prompt diagnosis, avoiding indwelling ventriculoperitoneal shunt, and enhancing the predictive value of pathologic examinations are only some of the advantages conferred by a simultaneous third ventriculostomy and tumor biopsy in patients with pineal region tumors. The objective of this study was to retrospectively search the literature on concomitant, single burr hole endoscopic third ventriculostomy (ETV) and tumor biopsy (TB) for pineal region tumors and to analyze the feasibility, surgical safety, and benefits of these 2 combined procedures. Consequently, a comprehensive, systematic literature search was performed in compliance with the updated PRISMA 2020 guidelines within electronic databases MEDLINE/PubMed, EMBASE, PLOS, and Cochrane Library. Statistical analysis was performed with IBM SPSS 28.0.1.1(14), using Kendall's and Spearman's tests, with a P < 0.05 considered significant. A total of 25 studies were selected and included in this review, for a total of 368 patients (mean age 20.6 years; range 1-86 years; SD 17.5). More than two-thirds of the procedures were operated with a rigid endoscope and 27.6% were performed with either a flexible endoscope, a combination of the 2, or not otherwise specified. Germinoma represented the most frequent diagnosis (20.1%) followed by astrocytoma (12.9%) and pineocytoma (9.9%). The single-entry approach allowed a correct histologic diagnosis in 88.7% of the examined cases. Summing up, concomitant ETV and TB represent a valuable option for the management of non-communicating hydrocephalus and the initial assessment of pineal region tumors. The histologic confirmation rate was 88.7% in the examined cohort, with only 10% of the biopsies yielding inconclusive results.
Topics: Humans; Young Adult; Adult; Ventriculostomy; Retrospective Studies; Feasibility Studies; Third Ventricle; Pineal Gland; Pinealoma; Neuroendoscopy; Biopsy; Hydrocephalus; Brain Neoplasms
PubMed: 36764448
DOI: 10.1016/j.wneu.2023.01.082 -
AJNR. American Journal of Neuroradiology Jan 2008Pineocytomas have been described as well-circumscribed, homogeneously enhancing masses. However, there is considerable variability in this appearance, and certain... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND AND PURPOSE
Pineocytomas have been described as well-circumscribed, homogeneously enhancing masses. However, there is considerable variability in this appearance, and certain pineocytomas may have a predominantly cystic appearance on imaging. This has led some to suggest that differentiation between pineocytomas and pineal cysts may not be possible. We have attempted to determine if cystic pineocytomas could be found in a series of these tumors evaluated by CT and MR imaging.
MATERIALS AND METHODS
We searched the radiology literature as well as the medical records from our own institution for pathologically proved pineocytomas with available preoperative imaging or imaging reports, with specific focus on whether postcontrast MR imaging was included. In cases in which images were available, they were evaluated by a Certificate of Added Qualification (CAQ)-certified neuroradiologist and a radiology resident, who attempted to determine if the pineocytomas had any MR imaging characteristics of typical pineal cysts. To be considered a typical pineal cyst, an area of signal-intensity abnormality must be centered on the pineal recess, demonstrating internal homogeneity on T2-weighted imaging, following CSF signal intensity on T1- and T2-weighted images, without any marginal lobularity or nodular contrast enhancement and a wall thickness of <2 mm. In cases in which imaging was not available, radiology reports and/or descriptions provided in the radiology literature were reviewed by a CAQ-certified neuroradiologist. For any lesion described as cystic, we again attempted to elucidate the exact extent of imaging that was performed, note specific lesion characteristics, and determine if the lesion met the criteria described previously. Finally, for tumors in which image size was provided, the mean value of maximal tumor dimension, SD, median, and range were calculated.
RESULTS
Forty-four pathologically proved cases of pineocytomas from the radiology literature, as well as 8 pathologically proved cases of pineocytomas from our institution with available imaging studies and/or reports, were reviewed. Of these, 23 were solid masses, and 7 were partially solid and cystic, whereas 14 tumors could not be completely characterized due to incomplete imaging evaluation. Eight were primarily cystic; however, none of these could be confidently characterized as meeting the criteria for a typical cyst.
CONCLUSION
In our analysis, no truly cystic pineocytomas were identified.
Topics: Brain Neoplasms; Cysts; Diagnosis, Differential; Humans; Magnetic Resonance Imaging; Pineal Gland; Pinealoma; Prevalence; Terminology as Topic; Tomography, X-Ray Computed
PubMed: 17925371
DOI: 10.3174/ajnr.A0750