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Pediatric Neurosurgery 2023Embryonal tumors are highly malignant cancers of the central nervous system, with a relatively high incidence in infants and young children. Even with intensive... (Review)
Review
BACKGROUND
Embryonal tumors are highly malignant cancers of the central nervous system, with a relatively high incidence in infants and young children. Even with intensive multimodal treatment, the prognosis of many types is guarded, and treatment-related toxicity is significant. Recent advances in molecular diagnostics allowed the discovery of novel entities and inter-tumor subgroups, with opportunities for improved risk-stratification and treatment approaches.
SUMMARY
Medulloblastomas separate into four distinct subgroups with distinct clinicopathologic characteristics, and data from recent clinical trials for newly diagnosed medulloblastoma support subgroup-specific treatment approaches. Atypical teratoid rhabdoid tumor (ATRT), embryonal tumor with multilayered rosettes (ETMR), and pineoblastoma, as well as other rare embryonal tumors, can be distinguished from histologically similar tumors by virtue of characteristic molecular findings, with DNA methylation analysis providing a strong adjunct in indeterminate cases. Methylation analysis can also allow further subgrouping of ATRT and pineoblastoma. Despite the dire need to improve outcomes for patients with these tumors, their rarity and lack of actionable targets lead to a paucity of clinical trials and novel therapeutics.
KEY MESSAGES
(1) Embryonal tumors can be accurately diagnosed with pediatric-specific sequencing techniques. (2) Medulloblastoma risk stratification and treatment decisions should take into account molecular subgroups. (3) There is a dire need for a novel collaborative clinical trial design to improve outcomes is rare pediatric embryonal tumors.
Topics: Child, Preschool; Humans; Infant; Brain Neoplasms; Central Nervous System Neoplasms; Cerebellar Neoplasms; Medulloblastoma; Neoplasms, Germ Cell and Embryonal; Pineal Gland; Pinealoma; Rhabdoid Tumor; Clinical Trials as Topic
PubMed: 37245504
DOI: 10.1159/000531256 -
Child's Nervous System : ChNS :... Sep 2023This study aimed to identify factors of a worse prognosis among different histological types of pineal region tumors in pediatric patients treat at a single institution...
PURPOSE
This study aimed to identify factors of a worse prognosis among different histological types of pineal region tumors in pediatric patients treat at a single institution in a 30-year period.
MATERIAL AND METHODS
Pediatric patients (151; < 18 years of age) treated between 1991 and 2020 were analyzed. Kaplan-Meyer survival curves were created, and the log-rank test was used to compare the main prognostic factors in the different histological types.
RESULTS
Germinoma was found in 33.1%, with an overall 60-month survival rate of 88%; the female sex was the only factor of a worse prognosis. Non-germinomatous germ cell tumors were found in 27.1%, with an overall 60-month survival rate of 67.2%; metastasis upon diagnosis, residual tumor, and the absence of radiotherapy were associated with a worse diagnosis. Pineoblastoma was found in 22.5%, with an overall 60-month survival rate of 40.7%; the male sex was the only factor of a worse prognosis; a tendency toward a worse outcome was found in patients < 3 years of age and those with metastasis upon diagnosis. Glioma was identified in 12.5%, with an overall 60-month survival rate of 72.6%; high-grade gliomas were associated with a worse prognosis. Atypical teratoid rhabdoid tumors was found in 3.3%, and all patients died within a 19-month period.
CONCLUSION
Pineal region tumors are characterized by the heterogeneity of histological types, which exert an influence on the outcome. Knowledge of the prognostic factors for each histological types is of extreme importance to the determination of guided multidisciplinary treatment.
Topics: Child; Humans; Male; Female; Pinealoma; Prognosis; Pineal Gland; Brain Neoplasms; Glioma
PubMed: 37202535
DOI: 10.1007/s00381-023-05952-x -
Journal of Neurosurgery. Pediatrics Aug 2023Pineal region tumors account for 2.7%-11% of all CNS tumors in children. In this series, the authors present their surgical results and long-term outcomes from a...
OBJECTIVE
Pineal region tumors account for 2.7%-11% of all CNS tumors in children. In this series, the authors present their surgical results and long-term outcomes from a pediatric pineal region tumor cohort.
METHODS
A total of 151 children aged 0-18 years were treated from 1991 to 2020. Tumor markers were collected in all patients; if positive, chemotherapy was performed, and if negative, biopsy was performed, preferably endoscopically. Resection was performed when there was a residual germ cell tumor (GCT) lesion after chemotherapy.
RESULTS
The distribution based on histological type, as verified by markers, biopsy, or surgery, was germinoma (33.1%), nongerminomatous GCT (NGGCT) (27.2%), pineoblastoma (22.5%), glioma (12.6%), and embryonal tumor (atypical teratoid rhabdoid tumor) (3.3%). A total of 97 patients underwent resection, and gross-total resection (GTR) was achieved in 64%; the highest GTR rate (76.6%) was found in patients with GCTs, and the lowest (30.8%) was found in those with gliomas. The supracerebellar infratentorial approach (SCITA) was the most common, performed in 53.6% of patients, followed by the occipital transtentorial approach (OTA), performed in 24.7% of patients. Lesions were biopsied in 70 patients, and the diagnostic accuracy was 91.4. The overall survival (OS) rates at 12, 24, and 60 months as stratified by histological type were 93.7%, 93.7%, and 88% for patients with germinomas; 84.5%, 63.5%, and 40.7% for patients with pineoblastomas; 89.4%, 80.8%, and 67.2% for patients with NGGCTs; 89.4%, 78.2%, and 72.6% for patients with gliomas; and 40%, 20%, and 0% for patients with embryonal tumors, respectively (p < 0001). The OS at 60 months was significantly higher in the group with GTR (69.7%) than in the group with subtotal resection (40.8%) (p = 0.04). The 5-year progression-free survival was 77% for patients with germinomas, 72.6% for patients with gliomas, 50.8% for patients with NGGCTs, and 38.9% for patients with pineoblastomas.
CONCLUSIONS
The efficacy of resection varies by histological type, and complete resection is associated with higher OS rates. Endoscopic biopsy is the method of choice for patients presenting with negative tumor markers and hydrocephalus. For tumors restricted to the midline and with extension to the third ventricle, a SCITA is preferred, whereas for lesions with extension toward the fourth ventricle, an OTA is preferred.
Topics: Male; Child; Humans; Pinealoma; Pineal Gland; Glioma; Germinoma; Brain Neoplasms
PubMed: 37148224
DOI: 10.3171/2023.3.PEDS22468 -
Journal of Neuro-oncology Apr 2023Pineal parenchymal tumors of intermediate differentiation (PPTIDs), which were recognized in the 2007 World Health Organization (WHO) classification, are rare,...
PURPOSE
Pineal parenchymal tumors of intermediate differentiation (PPTIDs), which were recognized in the 2007 World Health Organization (WHO) classification, are rare, accounting for less than 1% of all central nervous system tumors. This rarity and novelty complicate the diagnosis and treatments of PPTID. We therefore aimed to evaluate the clinicopathological significance of this tumor.
METHODS
At 11 institutions participating in the Kyushu Neuro-Oncology Study Group, data for patients diagnosed with PPTID were collected. Central pathology review and KBTBD4 mutation analysis were applied to attain the diagnostically accurate cohort.
RESULTS
PPTID was officially diagnosed in 28 patients: 11 (39%) with WHO grade 2 and 17 (61%) with WHO grade 3 tumors. Median age was 49 years, and the male:female ratio was 1:2.1. Surgery was attempted in all 28 patients, and gross total resection (GTR) was achieved in 46% (13/28). Adjuvant radiotherapy and chemotherapy were administered to, respectively, 82% (23/28) and 46% (13/28). The 5-year progression-free survival (PFS) and overall survival rates were 64.9% and 70.4% respectively. Female sex (p = 0.018) and GTR (p < 0.01) were found to be independent prognostic factors for PFS and female sex (p = 0.019) was that for OS. Initial and second recurrences were most often leptomeningeal (67% and 100% respectively). 80% (20/25) of patients harbored a KBTBD4 mutation.
CONCLUSIONS
Female sex and GTR were independent prognostic factors in our patients with PPTID. Leptomeningeal recurrence was observed to be particularly characteristic of this tumor. The rate of KBTBD4 mutation observed in our cohort was acceptable and this could prove the accuracy of our PPTID cohort.
Topics: Humans; Male; Female; Middle Aged; Pinealoma; Brain Neoplasms; Cohort Studies; Progression-Free Survival; Pineal Gland; Retrospective Studies
PubMed: 37052748
DOI: 10.1007/s11060-023-04310-w -
Clinical Radiology Jul 2023To differentiate between pineal germ cell tumour and pineoblastoma using apparent diffusion coefficient (ADC) values due to their overlapping imaging findings on...
AIM
To differentiate between pineal germ cell tumour and pineoblastoma using apparent diffusion coefficient (ADC) values due to their overlapping imaging findings on magnetic resonance imaging (MRI).
MATERIALS AND METHODS
This retrospective study was conducted on 33 patients with pineal germ cell tumours and eight patients with pineoblastoma who underwent pretreatment MRI. Twenty-seven patients (21 with pineal germ cell tumour and six with pineoblastoma) were included for ADC measurement. The minimum and mean ADC values of the tumours were measured, with normalized tumour to control ADC ratios generated. The MRI characteristics of the tumours were evaluated.
RESULTS
The mean and minimum ADC values, normalized mean and minimum ADC ratios of pineal germ cell tumours were significantly higher than those of pineoblastomas (all p<0.005). A cut-off value of 0.92 for the normalized mean ADC ratio was used to distinguish between pineal germ cell tumour and pineoblastoma and achieved an area under the curve of 0.95, sensitivity of 90.5%, specificity of 83.3%, and accuracy of 92.6%. An equal degree of contrast enhancement to the adjacent venous sinus was the only MRI characteristic that suggested the diagnosis of pineal germ cell tumour.
CONCLUSION
The ADC values could help differentiate between pineal germ cell tumour and pineoblastoma, specifically when conventional MRI findings are indeterminate.
Topics: Humans; Pinealoma; Brain Neoplasms; Retrospective Studies; Diffusion Magnetic Resonance Imaging; Diagnosis, Differential; Pineal Gland; Sensitivity and Specificity
PubMed: 37037704
DOI: 10.1016/j.crad.2023.03.008 -
Chinese Medical Journal Feb 2023
Topics: Humans; Child; Child, Preschool; Pinealoma; Prognosis; Brain Neoplasms; Pineal Gland
PubMed: 36989486
DOI: 10.1097/CM9.0000000000002063 -
Annals of Medicine and Surgery (2012) Mar 2023The epithalamus region contains the tiny, functionally endocrine pineal gland, which has the shape of a pinecone. Less than 1% of adult primary intracranial malignancies...
UNLABELLED
The epithalamus region contains the tiny, functionally endocrine pineal gland, which has the shape of a pinecone. Less than 1% of adult primary intracranial malignancies are pineal parenchymal tumors, which are incredibly uncommon brain tumors. A rare variety of pineal parenchymal tumors are those with intermediate differentiation. These tumors, whose namesake refers to a malignant pineal parenchymal tumor, are intermediate between pineoblastomas and pineocytomas (a benign pineal parenchymal tumor).
CASE PRESENTATION
A female patient, age 13, who had been experiencing terrible headaches on and off for a month, went to the emergency room. Along with the headache, she experienced nausea, vomiting, dizziness, and blurred eyesight. A nonenhanced computed tomography scan was used for the initial brain neuroimaging, which showed a hypodense mass posterior to the midbrain and superior to the cerebellum. A heterogeneous bulk was visible on MRI.
CLINICAL OUTCOME
The headache, vertigo, visual disturbance, nausea, and vomiting have all improved, according to the patient. Both postoperative MRIs with and without contrast revealed the resolution of the obstructive hydrocephalus and the absence of any residual enhancing mass. The patient was followed up for 2 months without any complications or adverse events.
CONCLUSION
One should carefully investigate a headache as the early symptom of many illnesses and rule out any other potential causes. This would therefore enable us to create a management structure for such a very unusual malignancy.
PubMed: 36923739
DOI: 10.1097/MS9.0000000000000147 -
Current Problems in Cancer Jun 2023
Topics: Humans; Pineal Gland; Brain Neoplasms; Treatment Outcome; Ventriculostomy
PubMed: 36870166
DOI: 10.1016/j.currproblcancer.2023.100954 -
World Neurosurgery May 2023Pineal parenchymal tumors are exceedingly rare, and optimal disease management has yet to be defined. In this study, we aimed to identify prognostic factors and...
BACKGROUND
Pineal parenchymal tumors are exceedingly rare, and optimal disease management has yet to be defined. In this study, we aimed to identify prognostic factors and establish a predictive model for the prognosis of patients with pineal parenchymal tumors.
METHODS
All patients with pineal parenchymal tumors in the Surveillance, Epidemiology and End Results database between 1975 and 2019 were reviewed. Data were summarized, and survival was modeled with Cox regression analyses. In addition, a nomogram predicting 5- and 10-year survival probability for pineal parenchymal tumors was developed and validated.
RESULTS
We found 691 pineal parenchymal and 1961 pineal region neoplasms during 1975 and 2019 resulting in an incidence of 35%. In total, 441 patients were excluded due to incomplete data. The final cohort was subdivided into groups based on tumor histology: pineocytomas, pineoblastomas, and pineal parenchymal tumors of intermediate differentiation. Multivariate Cox analysis identified age and beam radiation as prognostic factors in pineoblastomas. Age, histology, tumor size, extent of resection, radiation, and chemotherapy were selected to build a clinical nomogram. The C-index for the nomogram was 0.795 (95% confidence interval 0.738-0.852). The calibration curves of the 5- and 10-year survival rates showed good agreement between the nomogram predictions and actual observations.
CONCLUSIONS
This nomogram is a convenient and precise tool for clinicians to evaluate prognosis of pineal parenchymal tumors.
Topics: Humans; Pinealoma; Nomograms; Prognosis; SEER Program; Brain Neoplasms; Pineal Gland
PubMed: 36841539
DOI: 10.1016/j.wneu.2023.02.084 -
Journal of Clinical Neuroscience :... Apr 2023Access to the pineal region has always been a challenge for neurosurgeons. The parietooccipital interhemispheric transtentorial approach is a slight variation of the...
Access to the pineal region has always been a challenge for neurosurgeons. The parietooccipital interhemispheric transtentorial approach is a slight variation of the traditional occipital transtentorial approach that provides adequate exposure to the lesions of the pineal region without introducing additional risks. In this study, the modified parietooccipital interhemispheric transtentorial approach is discussed including step-by-step anatomical cadaveric dissections and operative images. 27 adult patients (age > 18) who were operated over a 30-year period (1992-2022) by the senior author (M.N.P.) at two clinics, Marmara University, Department of Neurosurgery, Istanbul, Turkey and Acibadem Mehmet Ali Aydinlar University, Department of Neurosurgery, Istanbul, Turkey were analyzed. Only pineal region tumors were included in the analysis. Falcotentorial meningiomas and vascular lesions including cavernomas were excluded. 5 cadaveric specimens were dissected step by step following the surgical approach. Each step was documented using a Canon EOS 5D Mark II camera with Canon 100 mm Macro Lens. Step by step images of the dissections were presented including comparison with surgical images. Additional illustrations were used to describe the surgical corridor. The surgical corridor is maintained anterior to the parietooccipital sulcus along the medial of the precuneus. No retraction to the calcarine sulcus resulted in no postoperative hemianopsia. The neurovascular structures along the surgical corridor along with the nuances of the tentorium incision and splenium resection are discussed. The parietooccipital interhemispheric transtentorial approach provides a wide and safe corridor for surgical resection of pineal tumors.
Topics: Adult; Humans; Middle Aged; Pinealoma; Pineal Gland; Meningeal Neoplasms; Brain Neoplasms; Cadaver
PubMed: 36791494
DOI: 10.1016/j.jocn.2022.12.017