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Introduction: Pineal region tumors have historically been challenging to treat. Advances in surgical techniques have led to significant changes in care and outcomes for these patients, and this is well demonstrated by our single institution's experience over a 17-year-period in which the evolution of diagnosis, treatment, and outcomes of pineal tumors in pediatric patients will be outlined. Methods: We retrospectively collected data on all pediatric patients with pineal region lesions treated with surgery at Children's National Hospital (CNH) from 2005 to 2021. Variables analyzed included presenting symptoms, presence of hydrocephalus, diagnostic and surgical approach, pathology, and adverse events, among others. IRB approval was obtained (IRB: STUDY00000009), and consent was waived due to minimal risk to patients included. Results: A total of 43 pediatric patients with pineal region tumors were treated during a 17-year period. Most tumors in our series were germinomas (n = 13, 29.5%) followed by pineoblastomas (n = 10, 22.7%). Twenty seven of the 43 patients (62.8%) in our series received a biopsy to establish diagnosis, and 44.4% went on to have surgery for resection. The most common open approach was posterior interhemispheric (PIH, transcallosal) – used for 59.3% of the patients. Gross total resection was achieved in 50%; recurrence occurred in 20.9% and mortality in 11% over a median follow-up of 47 months. Endoscopic third ventriculostomy (ETV) was employed to treat hydrocephalus in 26 of the 38 patients (68.4%) and was significantly more likely to be performed from 2011 to 2021. Most (73%) of the patients who received an ETV also underwent a concurrent endoscopic biopsy. No difference was found in recurrence rate or mortality in patients who underwent resection compared to those who did not, but complications were more frequent with resection. There was disagreement between frozen and final pathology in 18.4% of biopsies. Conclusion: This series describes the evolution of surgical approaches and outcomes over a 17-year-period at a single institution. Complication rates were higher with open resection, reinforcing the safety of pursuing endoscopic biopsy as an initial approach. The most significant changes occurred in the preferential use of ETVs over ventriculoperitoneal shunts. Though there has been a significant evolution in our understanding of and treatment for these tumors, in our series, the outcomes for these patients have not significantly changed over that time. [ABSTRACT FROM AUTHOR]

Pineal region tumors (PTs) represent extremely rare pathologies, characterized by highly heterogeneous histological patterns. Most of the available evidence for Gamma Knife radiosurgical (GKSR) treatment of PTs arises from multimodal regimens, including GKSR as an adjuvant modality or as a salvage treatment at recurrence. We aimed to gather existing evidence on the topic and analyze single‐patient‐level data to address the efficacy and safety of primary GKSR. This is a systematic review of the literature (PubMed, Embase, Cochrane, Science Direct) and pooled analysis of single‐patient‐level data. A total of 1054 original works were retrieved. After excluding duplicates and irrelevant works, we included 13 papers (n = 64 patients). An additional 12 patients were included from the authors' original series. A total of 76 patients reached the final analysis; 56.5% (n = 43) received a histological diagnosis. Confirmed lesions included pineocytoma WHO grade I (60.5%), pineocytoma WHO grade II (14%), pineoblastoma WHO IV (7%), pineal tumor with intermediate differentiation WHO II/III (4.7%), papillary tumor of pineal region WHO II/III (4.7%), germ cell tumor (2.3%), neurocytoma WHO I (2.3%), astrocytoma WHO II (2.3%) and WHO III (2.3%). Presumptive diagnoses were achieved in the remaining 43.5% (n = 33) of cases and comprised of pineocytoma (9%), germ cell tumor (6%), low‐grade glioma (6%), high‐grade glioma (3%), meningioma (3%) and undefined in 73%. The mean age at the time of GKSR was 38.7 years and the mean lesional volume was 4.2 ± 4 cc. All patients received GKSR with a mean marginal dose of 14.7 ± 2.1 Gy (50% isodose). At a median 36‐month follow‐up, local control was achieved in 80.3% of cases. Thirteen patients showed progression after a median time of 14 months. Overall mortality was 13.2%. The median OS was not reached for all included lesions, except high‐grade gliomas (8mo). The 3‐year OS was 100% for LGG and pineal tumors with intermediate differentiation, 91% for low‐grade pineal lesions, 66% for high‐grade pineal lesions, 60% for germ cell tumors (GCTs), 50% for HGG, and 82% for undetermined tumors. The 3‐year progression‐free survival (PFS) was 100% for LGG and pineal intermediate tumors, 86% for low‐grade pineal, 66% for high‐grade pineal, 33.3% for GCTs, and 0% for HGG. Median PFS was 5 months for HGG and 34 months for GCTs. The radionecrosis rate was 6%, and cystic degeneration was observed in 2%. Ataxia as a presenting symptom strongly predicted mortality (odds ratio [OR] 104, p =.02), while GCTs and HGG histology well predicted PD (OR: 13, p =.04). These results support the efficacy and safety of primary GKSR treatment of PTs. Further studies are needed to validate these results, which highlight the importance of the initial presumptive diagnosis for choosing the best therapeutic strategy. [ABSTRACT FROM AUTHOR]

Background: Two major approaches exist for the surgical removal of pineal region tumors: the supracebellar infratentorial and the sub-occipital transtentorial. Methods: We present the Lyon's technique of the sub-occipital transtentorial approach for pineal region tumors and our tricks to avoid complications. The principle is to expose the pineal region under the occipital lobe and not through the interhemispheric fissure. Conclusions: The sub-occipital transtentorial approach is a direct, extra cerebral, safe, and effective way to access tumors of the pineal region. [ABSTRACT FROM AUTHOR]

Abstract Background The goal of this study is to show the feasibility and benefits of using the simultaneous biportal endoscopic procedure to treat pineal tumors in patients with obstructive hydrocephalus. Methods We retrospectively reviewed three patients with pineal tumors and acute obstructive hydrocephalus who were treated in one session with a frameless stereotactic guided simultaneous biportal endoscopic third ventriculostomy and endoscopic tumor biopsy performed through two separate ports using one rigid ventriculoscope. Results In the three patients, ventriculostomy and endoscopic biopsies were conducted. There was no death or morbidity throughout the 45-min procedure. All of the patients’ histological findings were confirmed. Germinoma was diagnosed in two patients who recieved postoperative radiotherapy, and the third patient diagnosed with a pineocytoma. Magnetic resonance imaging with flow-sensitive sequences was used to confirm ventriculostomy patency in all patients 6 months after the surgery. Conclusion Biportal endoscopic approach enables better visual control of both procedures. Furthermore, it allows the surgeon to safely pass the ventriculoscope via the foramen of monro, even if it is narrow. Moreover, during endoscopic tumor biopsy and third ventriculostomy, the intracranial pressure can be smoothly managed using the outlet tubes accessible. This treatment may be an alternative to traditional uniportal endoscopic operations in certain patients.

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. [ABSTRACT FROM AUTHOR]

Background: The goal of this study is to show the feasibility and benefits of using the simultaneous biportal endoscopic procedure to treat pineal tumors in patients with obstructive hydrocephalus. Methods: We retrospectively reviewed three patients with pineal tumors and acute obstructive hydrocephalus who were treated in one session with a frameless stereotactic guided simultaneous biportal endoscopic third ventriculostomy and endoscopic tumor biopsy performed through two separate ports using one rigid ventriculoscope. Results: In the three patients, ventriculostomy and endoscopic biopsies were conducted. There was no death or morbidity throughout the 45-min procedure. All of the patients' histological findings were confirmed. Germinoma was diagnosed in two patients who recieved postoperative radiotherapy, and the third patient diagnosed with a pineocytoma. Magnetic resonance imaging with flow-sensitive sequences was used to confirm ventriculostomy patency in all patients 6 months after the surgery. Conclusion: Biportal endoscopic approach enables better visual control of both procedures. Furthermore, it allows the surgeon to safely pass the ventriculoscope via the foramen of monro, even if it is narrow. Moreover, during endoscopic tumor biopsy and third ventriculostomy, the intracranial pressure can be smoothly managed using the outlet tubes accessible. This treatment may be an alternative to traditional uniportal endoscopic operations in certain patients. [ABSTRACT FROM AUTHOR]

Purpose : Pineal anlage tumors (PAT) are extremely rare malignant tumors in the pediatric population considered to be a variant of pineoblastomas. These tumors are characterized by a combination of neuroectodermal and heterologous ectomesenchymal components. PAT can be clinically aggressive and has a propensity to cerebrospinal fluid (CSF) dissemination. So far, only 13 cases of these tumors have been reported in the literature. Case Article: In this article, we report the case of an anlage tumor in a 3-year-old girl with a rapidly aggressive course. [ABSTRACT FROM AUTHOR]

Purpose: We sought to characterize clinical outcomes for adult and pediatric patients with primary CNS tumors harboring DICER1 mutations or loss of DICER1. Methods: We conducted a retrospective cohort study of 98 patients who were treated between 1995 and 2020 for primary CNS tumors containing DICER1 mutations or loss of DICER1 on chromosome 14q, identified by targeted next generation sequencing. Kaplan–Meier plots and log rank tests were used to analyze survival. Cox proportional-hazards model was used for univariate and multivariable analyses for all-cause mortality (ACM). Results: Within our cohort, the most common malignancies were grade 3/4 glioma (61%), grade 1/2 glioma (17%), and CNS sarcoma (6%). Sarcoma and non-glioma histologies, and tumors with biallelic DICER1 mutations or deletions were common in the pediatric population. Mutations occurred throughout DICER1, including missense mutations in the DexD/H-box helicase, DUF283, RNaseIIIa, and RNaseIIIb domains. For patients with grade 3/4 glioma, MGMT methylation (Hazard ratio [HR] 0.35, 95% Confidence Interval [CI] 0.16–0.73, p = 0.005), IDH1 R132 mutation (HR 0.11, 95% CI 0.03–0.41, p = 0.001), and missense mutation in the DexD/H-box helicase domain (HR 0.06, 95% CI 0.01–0.38, p = 0.003) were independently associated with longer time to ACM on multivariable analyses. Conclusion: DICER1 mutations or loss of DICER1 occur in diverse primary CNS tumors, including previously unrecognized grade 3/4 gliomas as the most common histology. While prior studies have described RNaseIIIb hotspot mutations, we document novel mutations in additional DICER1 functional domains. Within the grade 3/4 glioma cohort, missense mutation in the DexD/H-box helicase domain was associated with prolonged survival. [ABSTRACT FROM AUTHOR]

Objective: Pineal tumors are relatively rare central nervous system lesions with a predilection for the pediatric population. This article aims to explore the clinical effects of neuroendoscopic infratentorial supracerebellar approach for resecting tumors in the pineal area., Methods: This is a retrospective study that included patients who underwent neuroendoscopic infratentorial supracerebellar approach to resect nine tumors in the pineal area at the Department of Neurosurgery of the Second Hospital of Lanzhou University from December 2017 to October 2023., Results: The results of postoperative MRI revealed that all tumors were resected. Five patients received postoperative radiotherapy, three patients received radiotherapy along with chemotherapy, and one patient received neither radiotherapy nor chemotherapy. The pathological results showed that four patients were diagnosed with germinoma, two patients with teratoma, two patients with mixed germ cell tumors, and one patient with central neurocytoma. After surgery, one patient developed psychiatric symptoms, two patients developed binocular upward vision and diplopia, and one patient developed unstable walking and diplopia. With a follow-up of 1.7-4.8 years, all nine patients lived normally. Furthermore, none of them had tumor recurrence or death., Conclusion: The simple neuroendoscopic infratentorial supracerebellar approach has some safety and efficacy. It is suitable for tumors in the pineal region where the disease is mainly located below the Galen vein complex., (Copyright © 2024. Published by Elsevier Masson SAS.)

Background: Diffuse midline glioma (DMG) is an invasive astrocytic tumor arisen from midline structures, such as the pons and thalamus. Five cases of DMG in the pineal region have been reported, but the clinical course was poor; there was no case of survival for more than 2 years. Case Description: We report the case of a 12-year-old boy with DMG in the pineal region who is living a normal daily life for more than 6 years following multimodal treatment. He complained of a headache accompanied by vomiting that had gradually worsened 1 month previously, and initial magnetic resonance imaging revealed a pineal tumor. Germinoma was initially suspected; however, a combination of chemotherapy using carboplatin and etoposide was ineffective. The first surgery was performed through the left occipital transtentorial approach (OTA); the diagnosis was DMG. After 60 Gy radiotherapy concomitant with temozolomide (TMZ), the tumor enlarged. Second surgery was performed through bilateral OTAs, and 90% of the tumor was removed. In addition, stereotactic radiotherapy (30 Gy, six fractions) was administered, and the local equivalent dose in 2 Gy/fraction reached 97.5 Gy. Maintenance chemotherapy using TMZ and bevacizumab was continued for 2 years. After finishing chemotherapy, the enhancing lesion enlarged again, and bevacizumab monotherapy was effective. Now, at 6 years after diagnosis, the patient leads an ordinary life as a student. Conclusion: Maximum resection and high-dose radiotherapy followed by bevacizumab may have been effective in the present case. [ABSTRACT FROM AUTHOR]

Different treatment options have been proposed for obstructive hydrocephalus associated with pineal lesions. We discuss the obstructive hydrocephalus management associated with pineal region tumors and cysts in Helsinki Neurosurgery. In this article, hydrocephalus treatment by tumor-cyst removal (n = 40), shunt surgery (n = 25), and endoscopic ventriculostomies (n = 3) is evaluated in 68 patients with obstructive hydrocephalus among 136 patients with pineal region tumor and cyst. Multivariate statistical analysis was followed by univariate and multivariate regression models of last functional status, last tumor-free imaging, and disease-specific mortality of the study population. Preoperative hydrocephalus was linked to higher World Health Organization tumor grades, poor functional status, higher mortality, and incomplete resection of pineal region cysts and tumors. Preoperative hydrocephalus remained a predictor of poor last functional status after multivariate regression. Pineal lesion removal with the posterior third ventricle opening as primary hydrocephalus treatment resulted in better last functional status, fewer postoperative shunts, fewer hydrocephalus-related procedures, and fewer postoperative infections than in the shunt-treatment group. Multivariate regression analysis linked higher World Health Organization tumor grade, poor immediate functional status, postoperative complications, and incomplete surgical resection as independent predictors of disease mortality in patients with hydrocephalus. Same variables (except immediate modified Rankin Scale score) and higher number of shunt surgeries became independent predictors of poor last functional status at multivariate analysis. Incomplete resection was the only independent predictor of tumor-free magnetic resonance imaging at the last evaluation. Direct removal of pineal lesions with the opening of the posterior third ventricle could represent effective and reliable management of the associated obstructive hydrocephalus. Further research is required to generalize our inferences. [ABSTRACT FROM AUTHOR]

Biopsy of pineal region neoplasms is frequently accomplished by way of endoscopic transventricular access or using an image-guided, computer-assisted stereotactic approach. We evaluated a nonorthogonal lateral temporal approach for stereotactic biopsy of pineal region tumors as a variation of previously described stereotactic methods. Magnetic resonance imaging–guided frameless stereotaxy was used to plan and perform biopsies of pineal region tumors using a nonorthogonal trajectory extending from the superior or middle temporal gyri through the temporal stem, anterior to the atrium of the lateral ventricle, and posterior to the corticospinal tract. All patients had an uncomplicated postoperative course and remained at neurologic baseline. No parenchymal or ventricular hemorrhage was present on postoperative scans. A tissue diagnosis was obtained in all patients. This method appears to be a safe alternative to stereotactic biopsy using other trajectories and provides adequate tissue for definitive diagnosis. [ABSTRACT FROM AUTHOR]

Overall, tumors of primary central nervous system (CNS) are quite common in adults with an incidence rate close to 30 new cases/100,000 inhabitants per year. Significant clinical and biological advances have been accomplished in the most common adult primary CNS tumors (i.e., diffuse gliomas). However, most CNS tumor subtypes are rare with an incidence rate below the threshold defining rare disease of 6.0 new cases/100,000 inhabitants per year. Close to 150 entities of primary CNS tumors have now been identified by the novel integrated histomolecular classification published by the World Health Organization (WHO) and its updates by the c-IMPACT NOW consortium (the Consortium to Inform Molecular and Practical Approaches to CNS Tumor Taxonomy). While these entities can be better classified into smaller groups either by their histomolecular features and/or by their location, assessing their treatment by clinical trials and improving the survival of patients remain challenging. Despite these tumors are rare, research, and advances remain slower compared to diffuse gliomas for instance. In some cases (i.e., ependymoma, medulloblastoma) the understanding is high because single or few driver mutations have been defined. The European Union has launched European Reference Networks (ERNs) dedicated to support advances on the clinical side of rare diseases including rare cancers. The ERN for rare solid adult tumors is termed EURACAN. Within EURACAN, Domain 10 brings together the European patient advocacy groups (ePAGs) and physicians dedicated to improving outcomes in rare primary CNS tumors and also aims at supporting research, care and teaching in the field. In this review, we discuss the relevant biological and clinical characteristics, clinical management of patients, and research directions for the following types of rare primary CNS tumors: medulloblastoma, pineal region tumors, glioneuronal and rare glial tumors, ependymal tumors, grade III meningioma and mesenchymal tumors, primary central nervous system lymphoma, germ cell tumors, spinal cord tumors and rare pituitary tumors.

A combination of modern methods of high-tech microneurosurgery, neuroanesthesia and neuroreanimation allows a successful realization of the program for the surgical treatment of pineal tumors. Such operations belong to the highest difficulty category and carry a high surgical risk.

Overall, tumors of primary central nervous system (CNS) are quite common in adults with an incidence rate close to 30 new cases/100,000 inhabitants per year. Significant clinical and biological advances have been accomplished in the most common adult primary CNS tumors (i.e., diffuse gliomas). However, most CNS tumor subtypes are rare with an incidence rate below the threshold defining rare disease of 6.0 new cases/100,000 inhabitants per year. Close to 150 entities of primary CNS tumors have now been identified by the novel integrated histomolecular classification published by the World Health Organization (WHO) and its updates by the c-IMPACT NOW consortium (the Consortium to Inform Molecular and Practical Approaches to CNS Tumor Taxonomy). While these entities can be better classified into smaller groups either by their histomolecular features and/or by their location, assessing their treatment by clinical trials and improving the survival of patients remain challenging. Despite these tumors are rare, research, and advances remain slower compared to diffuse gliomas for instance. In some cases (i.e., ependymoma, medulloblastoma) the understanding is high because single or few driver mutations have been defined. The European Union has launched European Reference Networks (ERNs) dedicated to support advances on the clinical side of rare diseases including rare cancers. The ERN for rare solid adult tumors is termed EURACAN. Within EURACAN, Domain 10 brings together the European patient advocacy groups (ePAGs) and physicians dedicated to improving outcomes in rare primary CNS tumors and also aims at supporting research, care and teaching in the field. In this review, we discuss the relevant biological and clinical characteristics, clinical management of patients, and research directions for the following types of rare primary CNS tumors: medulloblastoma, pineal region tumors, glioneuronal and rare glial tumors, ependymal tumors, grade III meningioma and mesenchymal tumors, primary central nervous system lymphoma, germ cell tumors, spinal cord tumors and rare pituitary tumors. [ABSTRACT FROM AUTHOR]

The midline supracerebellar infratentorial (SCIT) approach and its paramedian development are commonly used for dealing with pineal lesions. Comparative clinical studies are lacking, however. We aim to establish the better performance of the paramedian SCIT approach in terms of clinical safety in surgically treated pineal cysts and pineal region tumors. Procedural functionality and effectiveness have been also analyzed. A comparative analysis of clinical, radiologic, pathologic, and surgical features, and outcome was performed between 55 midline and 57 paramedian SCIT approaches that were exclusively performed in 112 patients (57 pineal cysts and 55 tumors of the pineal region) operated in sitting position by a single surgeon. Information was retrieved from hospital records and microsurgical videos. The paramedian SCIT approach linked with fewer postoperative complications (odds ratio [OR]: 0.40) and fewer approach-related complications (OR: 0.28) than the midline SCIT approach. The SCIT paramedian approach was achieved in a shorter time, by a smaller bone flap, and with fewer complex procedural steps than the midline approach. The SCIT paramedian approach did not require the opening of the falx cerebelli, midline cerebellar retraction, section of the midline cerebellar draining veins, nor wide opening of the dura. Gross total resection, size of the lesion, microsurgical time for removal, histopathological diagnosis and postoperative outcome were statistically similar in both groups. The SCIT approach represents a safer and more functional approach for the removal of cysts and tumors of the pineal region than the classic midline approach, while maintaining the same effectiveness. [ABSTRACT FROM AUTHOR]

Pineal region tumors represent challenging surgical lesions with wide ranges of survival reported in different surgical series. In this article, we emphasize the role of complete microsurgical resection (CMR) to obtain a favorable long-term outcome of pineal region tumors. We report a retrospective study of pineal region tumors operated on in Helsinki Neurosurgery between 1997 and 2015. Information was obtained from the hospital records, and an evaluation of the Finnish population register was conducted in July 2018 to determine the current status of the patients. A total of 76 pineal region tumors were operated on. The survival was 62% at a mean follow-up of 125 ± 105 months (range, 0–588 months), and the disease-related mortality was limited to 14 patients (18.4%). Up to July 2018, 29 patients had died. Two patients died 1 and 3 months after surgery of delayed thalamic infarctions, 12 patients of disease progression, and 15 had non–disease-related deaths. Only 1 patient was lost in the long-term follow-up. Ten of 14 disease-related deaths occurred during the first 5 years of follow-up: 5 diffuse gliomas, 3 germ cell tumors, 1 grade II–III pineal parenchymal tumor of intermediate differentiation, and 1 meningioma. CMR was linked to better tumor-free survival and long-term survival, with the exception of diffuse gliomas. CMR, in the setting of a multidisciplinary management of pineal region tumors, correlates with favorable survival and with minimal mortality. Surgically treated grade II–IV gliomas constitute a particular group with high mortality within the first 5 years independently of the microsurgical resection. [ABSTRACT FROM AUTHOR]

Background: Tumors within the pineal region represent 1.5 to 8.5% of the pediatric brain tumors and 1.2% of all brain tumors. A management algorithm has been proposed in several publications. The algorithm includes endoscopic third ventriculostomy (ETV) and biopsy in cases presenting with hydrocephalus. In this series, we are presenting the efficacy of a single-trajectory approach for both ETV and biopsy.Methods: Eleven cases were admitted to Alexandria main university hospital from 2013 to 2016 presenting with pineal region tumors and hydrocephalus. Mean age at diagnosis was 11 years (1-27 years). All cases had ETV and biopsy using rigid ventriculoscope through a single trajectory from a burr hole planned on preoperative imaging. Follow-up period was 7-48 months.Results: All 11 cases presented with hydrocephalus and increased intracranial pressure manifestations. Histopathological diagnosis was successful in 9 out of 11 cases (81.8%). Three cases were germ-cell tumors, two cases were pineoblastomas, two cases were pilocytic astrocytomas, and two cases were grade 2 tectal gliomas. Five of the ETV cases (45.5%) failed and required VPS later on. Other complications of ETV included one case of intraventricular hemorrhage and a case with tumor disseminated to the basal cisterns.Conclusion: In our series, we were able to achieve ETV and biopsy through a single trajectory and a rigid endoscope with results comparable to other studies in the literature. [ABSTRACT FROM AUTHOR]

Pineal region tumors represent less than 1% and 3-8% of brain tumors in adults and children respectively. There is a wide range of pineal masses, with the majority being germ cell and pineal parenchymal tumors. Magnetic resonance imaging (MRI) is the modality of choice for the assessment of pineal masses. It is considered as the gold standard for the evaluation of the central nervous system. MRI has the ability to produce very detailed images of the brain anatomy and is used to distinguish true pineal masses from parapineal with invasion of the gland. Specific MRI findings are helpful to the differential diagnosis of pineal tumors and the distinction between benign from malignant tumors. Pineal neoplasms may seed the subarachnoid space resulting in the development of intradural extramedullary metastases, known as drop metastases. MRI is the most sensitive method for the assessment of the spinal cord, meninges and nerve roots and the differentiation of the spinal lesions into intra/extra medullary and extradural. Because of its high sensitivity and the advances of the method, drop metastases can be easily diagnosed at an earlier stage than in the past, contributing to the selection of the appropriate treatment. Therefore, the entire neuroaxis should be investigated with MRI for the presence of intradural extramedullary lesions. The present study focuses on the main MR imaging characteristics of pineal masses and drop metastases with reference to the differential diagnosis. There is also a detailed approach to the MR protocol which should be obtained in order to evaluate the lesions.

Pineal region tumors represent less than 1% and 3-8% of brain tumors in adults and children respectively. There is a wide range of pineal masses, with the majority being germ cell and pineal parenchymal tumors. Magnetic resonance imaging (MRI) is the modality of choice for the assessment of pineal masses. It is considered as the gold standard for the evaluation of the central nervous system. MRI has the ability to produce very detailed images of the brain anatomy and is used to distinguish true pineal masses from parapineal with invasion of the gland. Specific MRI findings are helpful to the differential diagnosis of pineal tumors and the distinction between benign from malignant tumors. Pineal neoplasms may seed the subarachnoid space resulting in the development of intradural extramedullary metastases, known as drop metastases. MRI is the most sensitive method for the assessment of the spinal cord, meninges and nerve roots and the differentiation of the spinal lesions into intra/extra medullary and extradural. Because of its high sensitivity and the advances of the method, drop metastases can be easily diagnosed at an earlier stage than in the past, contributing to the selection of the appropriate treatment. Therefore, the entire neuroaxis should be investigated with MRI for the presence of intradural extramedullary lesions. The present study focuses on the main MR imaging characteristics of pineal masses and drop metastases with reference to the differential diagnosis. There is also a detailed approach to the MR protocol which should be obtained in order to evaluate the lesions. [ABSTRACT FROM AUTHOR]

Introduction: Intracranial teratomas are rare germ cell neoplasms that contain tissues derived from all three germ cell layers and most commonly occurring during childhood. This is the first report of pineal region mixed mature teratoma and germinoma in two fraternal brothers of fraternal triplets. Case presentation: We report the case of a mixed mature teratoma and germinoma of the pineal region in two brothers of fraternal triplets. Older brother was initially diagnosed at the age of 11 years with the pure teratoma of the pineal region but the review of the pathology 3 years after initial surgery revealed the mixed mature teratoma with 5% germinomatous component. The younger brother was diagnosed at the age of 13 years with the mixed mature teratoma with 10% germinomatous component tumor of the pineal region. Younger brother has been treated with adjuvant chemo-radiotherapy and older brother was treated without adjuvant therapy. Both brothers had no recurrence. Conclusion: Pineal mature teratomas have a good prognosis, in contrast to their immature or mixed counterparts. A rigorous histological examination of the tumor samples is mandatory, in order to not omit a mixed contingent within the tumor. [ABSTRACT FROM AUTHOR]

Meningiomas are uncommon tumors in children and either more rarely encountered in the pineal region. We report two cases of meningioma of the pineal region in children. One of these cases was a five years-old girl and the other a one year-old boy. No specific clinical presentation or tomographic examinations findings was identified before treatment, suggestive of a diagnosis of menigioma. The clinical and laboratory features were very similar to the most common tumors of the pineal region. Prior to the surgery, the histology of these tumors was not suspected. Both patients underwent direct surgery and complete removal was achieved by a suboccipital transtentorial approach. The tumors originated from velum interpositum in both cases. At the follow up, one case presented with recurrence six years later, and she underwent a reoperation with total resection without morbidity. Long-term follow up presented no other recurrences.Meningiomas são tumores poucos frequentes em crianças, e mais raramente encontrados na região da pineal. Relatamos dois casos de meningioma da região da pineal em crianças, uma menina de cinco anos e um menino de um ano de idade. Não foi identificada nenhuma forma de apresentação clinica ou caracteristica tomográfica, antes do tratamento, que sugerisse o diagnóstico de meningioma. As características clinicas e laboratoriais encontradas foram similares às de tumores mais frequentes da região da pineal. Ambos os pacientes foram submetidos ao tratamento cirúrgico e a remoção completa foi obtida por abordagem suboccipital transtentorial. Durante o seguimento, um dos pacientes foi reoperado por recorrencia do tumor seis anos após o tratamento inicial. Atualmente, os pacientes encontram-se livres de recorrência tumoral.

Objective To assess the histologic accuracy of endoscopic biopsy samples of the pineal region. Pineal region tumors usually present with acute hydrocephalus. Histologic diagnosis is paramount, as it greatly influences treatment. Endoscopic techniques can combine histologic diagnosis with relief of the obstructive hydrocephalus in a single operation. Because pineal region tumors can be heterogeneous, initial biopsy samples may not represent the most aggressive portion of the tumor. Methods This retrospective study reviews our experience of endoscopic third ventriculostomy combined with biopsy of the lesion. The histologic diagnosis as a result of the initial biopsy was compared with the final histologic diagnosis to establish the accuracy of the endoscopic biopsy sample in aiding diagnosis. Results Forty-seven patients underwent an endoscopic third ventriculostomy. All but 1 patient underwent a concurrent biopsy of the space-occupying lesion and 39 of 46 patients (85%) had a histologic diagnoses. In the remaining 7 patients (15%), the histology was negative; in 6 cases, the second attempt to obtain a histologic diagnosis was successful (2 repeat endoscopic biopsy samples, 2 resections, 2 stereotactic biopsy samples). In 1 patient a presumed low-grade tectal tumor was followed up with sequential scanning. Twenty-eight patients underwent subsequent operations (24 resections, 4 stereotactic biopsies). In 6 of 28 patients (21%), the histologic report was amended after the second procedure. Conclusions The endoscopic biopsy sample yields an accurate histologic diagnosis for most pineal region tumors, with a positive histologic sample in about 85% of patients. However, the results must be interpreted cautiously, as the heterogeneous nature of these tumors may lead to an approximately 21% error rate in the initial tumor diagnosis. [ABSTRACT FROM AUTHOR]

Cerebral metastases from colorectal adenocarcinomas occur in 8% of the cases. Diagnosis is usually made when primary disease and widespread metastases are already known. We report the case of a patient with single metastases in the pineal region as the first clinical manifestation of a colorectal adenocarcinoma. A 48-year-old female with Parinaud's syndrome for 15 days prior her admission was evaluated in our clinic. She had no symptoms or signs of colorectal disease. MRI examination revealed an heterogeneous lesion with peritumoral gadolinium enhancement, located in the pineal region. The tumor was radically resected through an infratentorial/supracerebellar approach. Histology showed metastatic carcinoma and immunohistochemical examination showed gastrointestinal tract adenocarcinoma. Metastases to the pineal region are extremely rare and should be considered in the differential diagnosis of tumors of this region.

Introduction: Our study aimed to elucidate the imaging features for the differentiation of pineal germinoma and other pineal region tumors. Methods: Image data sets of computed tomographic (CT) scan and magnetic resonance imaging (MRI) data of 93 pineal region tumors including 33 germinomas, 30 nongerminomatous germ cell tumors (NGGCTs), 20 pineal parenchymal tumors (PPTs), and 10 miscellaneous tumors of pineal region were reviewed. Imaging features on CT and MRI were qualitatively assessed by three readers. To know the reasons for morphological differences between germinomas and NGGCTs, histological investigation was done. Results: Localized calcification was seen in more than 70 % of germ cells tumors (GCTs: germinomas and NGGCTs) while it was scattered in more than half of PPTs. Cystic components in tumors were most frequent in NGGCTs (62 %). Multiplicity of lesion was restricted to GCTs: 39.4 % in germinoma and 10.0 % in NGGCTs. Thick peritumoral edema was more frequent in germinoma than in NGGCT: 40.6 vs. 14.8 % ( p = 0.0433, Fisher's test). Bithalamic extension of tumor was seen in 78.8 % of germinomas. It was significantly rare in other groups of tumors ( p < 0.0001, Fisher's test). The relative collagen amount per unit area was significantly lower in germinoma than in NGGCTs. Conclusion: By paying attention to characteristic features as bithalamic extension, thick peritumoral edema, calcification pattern, multiplicity, and their combination, the preoperative differential diagnosis of pineal germinoma will become more accurate. [ABSTRACT FROM AUTHOR]