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INTRODUCTION: Outcomes for pineal region and superior cerebellar tumors in young children often hinge on extent of microsurgical resection, and thus choosing an approach that provides adequate visualization of pathology is essential. The occipital interhemispheric transtentorial (OITT) approach provides excellent exposure while minimizing cerebellar retraction. However, this approach has not been widely accepted as a viable option for very young children due to concerns for potential blood loss when incising the tentorium. The aim of this paper is to characterize our recent institutional experience with the occipital interhemispheric transtentorial approach (OITT) for tumor resection in infants and toddlers. METHODS: A retrospective study was performed between 2016 and 2023 of pediatric patients less than 36 months of age who underwent OITT for tumor resection at a high-volume referral center. Patients with at least 3 months of postoperative follow-up and postoperative MRI were included. Primary outcomes included extent of resection, intraoperative and postoperative complications, and neurologic outcome. Secondary outcomes included length of stay and estimated blood loss. RESULTS: Eight patients, five male, were included. The median age at the time of surgery was 10 months (range 5-36 months). Presenting symptoms included macrocephaly, nausea/vomiting, strabismus, gait instability, or milestone regression. Hydrocephalus was present preoperatively in all patients. Average tumor volume was 38.6 cm3, ranging from 1.3 to 71.9 cm3. All patients underwent an OITT approach for tumor resection with stereotactic guidance. No intraoperative complications occurred, and no permanent neurologic deficits developed postoperatively. Gross total resection was achieved in all cases per postoperative MRI report, and no instances of new cerebellar, brainstem, or occipital lobe ischemia were noted. CONCLUSIONS: OITT approach for tumor resection in very young children (≤ 36 months) is an effective strategy with an acceptable safety profile. In our series, no significant intraoperative or postoperative complications occurred. To our knowledge, this is the first report describing this technique specifically in patients less than 36 months of age.

Asunto(s)

Procedimientos Neuroquirúrgicos , Complicaciones Posoperatorias , Humanos , Lactante , Masculino , Femenino , Estudios Retrospectivos , Preescolar , Procedimientos Neuroquirúrgicos/métodos , Complicaciones Posoperatorias/etiología , Resultado del Tratamiento , Neoplasias Cerebelosas/cirugía , Neoplasias Encefálicas/cirugía , Pinealoma/cirugía

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PURPOSE: Access to the pineal region has always represented a fascinating challenge to the neurosurgeons. Almost equally thrilling is the historical evolution from the hard beginnings with extremely high mortality rates to the current surgical outcomes, with excellent resection rates without long-term morbidity for most of the patients. The purpose of this paper is to provide an overview of the historical evolution of the occipital interhemispheric transtentorial (OITT) approach and its role in the development of access to the pineal region. METHODS: Review of the literature highlights the occipital transtentorial approach from the historical context prior its description and the beginning to the current modifications and new recent insights. RESULTS: The occipital transtentorial approach described initially by Poppen in 1966 has played a key role in the progress and success accessing the pineal area. CONCLUSION: This historical review aims to highlight the extraordinary effort of those neurosurgeons who guided and led the development of one of the most important approaches to the pineal region.

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Procedimientos Neuroquirúrgicos , Glándula Pineal , Humanos , Historia del Siglo XX , Procedimientos Neuroquirúrgicos/historia , Procedimientos Neuroquirúrgicos/métodos , Glándula Pineal/cirugía , Historia del Siglo XXI , Historia del Siglo XIX , Lóbulo Occipital/cirugía

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OBJECTIVE: To assess the efficacy and surgical outcomes of the simultaneous single-trajectory endoscopic biopsy and third ventriculostomy (ETV) in pineal region tumors. METHODS: A systematic review and meta-analysis adhering to Cochrane Standards and PRISMA framework were conducted. PubMed, Embase, and Web Of Science databases were searched until December 2023. Outcomes included rate of histopathologic diagnosis success, ETV success, complications, required VPS, and mortality. RESULTS: Seventeen studies (N = 388) met inclusion criteria. Histopathologic diagnosis success rate was 90% for general population (95% CI: 86%-95%; I2 = 42%) and 94% for pediatric patients (95% CI: 89%-98%; I2 = 19%). ETV Success rate was 93% (95% CI: 88%-97%; I2 = 60%). An estimated risk of postoperative ETV complications was found to be 16% for the general population (95% CI: 5%-28%; I2 = 90%) and 5% for pediatric patients (95% CI: 0%-13%; I2 = 51%). The risk of requiring VPS was estimated as 2% (95% CI: 0%-4%; I2 = 39%) and for the pediatric population it was 7% (95% CI: 0%-16%; I2 = 69%). Mortality risk was found to be 1% (95% CI: 0%-3%; I2 = 0%). CONCLUSIONS: Simultaneous endoscopic biopsy and ETV demonstrated high diagnostic and therapeutic success rates. The procedure's safety profile, with low mortality and complications, supports its role in treating hydrocephalus associated to pineal region tumors. Subgroup analyses revealed higher diagnostic success rates and required VPS in the pediatric population, whilst it had lower complication rates.

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Neuroendoscopía , Glándula Pineal , Pinealoma , Tercer Ventrículo , Ventriculostomía , Humanos , Ventriculostomía/métodos , Pinealoma/cirugía , Neuroendoscopía/métodos , Tercer Ventrículo/cirugía , Biopsia/métodos , Glándula Pineal/cirugía , Glándula Pineal/patología , Neoplasias Encefálicas/cirugía , Complicaciones Posoperatorias/epidemiología

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BACKGROUND: Papillary tumors of pineal region (PTPR) comprise a very rare subset of pineal region tumors that have been recently described. Literature on the management and outcome of PTPR is scarce owing to the rarity of these tumors. To address this lacuna, we analyzed our experience in management of PTPR. METHODS: We retrospectively analyzed the outcome of 11 patients with histopathologically proven PTPR who underwent surgical excision at our center. RESULTS: Mean patient age was 33.3 years (range, 12-45 years), and male-to-female ratio was 1.75:1. Headache was the most common presentation followed by visual disturbances, altered sensorium, Perinaud syndrome, and seizures. Cerebrospinal fluid diversion was required in 6 patients. Krause approach was the most common approach used for tumor excision (9/11 cases). There was no perioperative mortality. Two patients were lost to follow-up. In the remaining 9 patients, the average follow-up period was 45 months (range, 12-79 months). On first postoperative magnetic resonance imaging, 8 patients showed no evidence of residual tumor (gross total resection), while 1 patient had small residual tumor (near-total resection) that remained stable during follow-up. Four patients underwent adjuvant chemoradiotherapy. None of the patients developed recurrence during follow-up. CONCLUSIONS: PTPR are a rare subgroup of pineal region tumors with distinct cells of origin but presentation similar to other pineal region tumors. Surgical resection constitutes the mainstay of management, and the extent of resection appears to be the most important determinant of prognosis. The role of adjuvant therapy still needs to be determined.

Asunto(s)

Neoplasias Encefálicas , Glándula Pineal , Pinealoma , Humanos , Masculino , Femenino , Niño , Adolescente , Adulto Joven , Adulto , Persona de Mediana Edad , Estudios Retrospectivos , Neoplasia Residual/patología , Glándula Pineal/diagnóstico por imagen , Glándula Pineal/cirugía , Glándula Pineal/patología , Pinealoma/cirugía , Pinealoma/patología , Neoplasias Encefálicas/patología

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BACKGROUND: A pineal region tumor is a rare intracranial tumor, and its specific location leads to its own characteristics. This study aimed to provide some insight for medical practice in the care of pineal region tumors. We investigated the key epidemiological characteristics and survival prognosis of pineal tumors based on the epidemiological data from the Surveillance, Epidemiology, and End Results database. METHODS: Data of pineal region tumor patients from 1975 to 2019 were extracted from the Surveillance, Epidemiology, and End Results database. The data were divided into 3 pathologic groups: germ cell tumors, pineal parenchymal tumors, and other. The patients' overall survival (OS) was analyzed using the Kaplan-Meier method. The prognostic effects of the patient characteristics on OS were explored using the Cox proportional hazard model. The analysis results are presented as tabular data, Kaplan-Meier plots, forest plots, and nomograms. A calibration curve was used to verify the nomograms. All analyses were performed for all patients overall and stratified by pathological group using SPSS and R language. RESULTS: Based on the predefined inclusion and exclusion criteria, 628 patients were included in this study, of whom 440 (70.1%) were male and 188 (29.9%) were female. Most patients were aged 0-19 years. The pathological type was germinoma for 225 patients (35.8%). Age, surgery, behavioral code, and pathology were significant factors for OS. A calibration curve was used to verify that the nomograms had a good prediction effect. CONCLUSIONS: An intuitive nomogram was developed and verified and can predict the prognosis of patients with pineal tumors.

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Neoplasias Encefálicas , Glándula Pineal , Pinealoma , Humanos , Masculino , Femenino , Pinealoma/epidemiología , Estadificación de Neoplasias , Estudios Retrospectivos , Programa de VERF , Pronóstico , Nomogramas , Neoplasias Encefálicas/epidemiología

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Pineal region tumors fall into five broad categories: benign pineal region tumors, glial tumors, papillary tumors, pineal parenchymal tumors, and germ cell tumors. Genetic and transcriptional studies have identified key chromosomal alterations in germinomas (RUNDC3A, ASAH1, LPL) and in pineocytomas/pineoblastomas (DROSHA/DICER1, RB1). Pineal region tumors generally present with symptoms of hydrocephalus including nausea, vomiting, papilledema, and the classical Parinaud's triad of upgaze paralysis, convergence-retraction nystagmus, and light-near pupillary dissociation. Workup requires neuroimaging and tissue diagnosis via biopsy. In germinoma cases, diagnosis may be made based on serum or CSF studies for alpha-fetoprotein or beta-HCG making the preferred treatment radiosurgery, thereby preventing the need for unnecessary surgeries. Treatment generally involves three steps: CSF diversion in cases of hydrocephalus, biopsy through endoscopic or stereotactic methods, and open surgical resection. Multiple surgical approaches are possible for approach to the pineal region. The original approach to the pineal region was the interhemispheric transcallosal first described by Dandy. The most common approach is the supracerebellar infratentorial approach as it utilizes a natural anatomic corridor for access to the pineal region. The paramedian or lateral supracerebellar infratentorial approach is another improvement that uses a similar anatomic corridor but allows for preservation of midline bridging veins; this minimizes the chance for brainstem or cerebellar venous infarction. Determination of the optimal approach relies on tumor characteristics, namely location of deep venous structures to the tumor along with the lateral eccentricity of the tumor. The immediate post-operative period is important as hemorrhage or swelling can cause obstructive hydrocephalus and lead to rapid deterioration. Adjuvant therapy, whether chemotherapy or radiation, is based on tumor pathology. Improvements within pineal surgery will require improved technology for access to the pineal region along with targeted therapies that can effectively treat and prevent recurrence of malignant pineal region tumors.

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Neoplasias Encefálicas , Glioma , Hidrocefalia , Glándula Pineal , Pinealoma , Humanos , Pinealoma/diagnóstico , Pinealoma/genética , Pinealoma/cirugía , Glándula Pineal/patología , Glándula Pineal/cirugía , Neoplasias Encefálicas/genética , Neoplasias Encefálicas/cirugía , Neoplasias Encefálicas/patología , Glioma/patología , Hidrocefalia/patología , Ribonucleasa III , ARN Helicasas DEAD-box

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Histological and molecular characterization is essential for the diagnosis of pediatric brain tumors. In the pineal region tumors, it is necessary to remove a sufficient tumor volume to make a diagnosis. However, surgery in this region is challenging due to its deep anatomical location and surrounded by critical structures and complex venous system. Knowledge of the anatomy and function of the pineal region and tumor histological types is imperative for the successful management of pineal region tumors. This article describes surgical approaches to pineal tumors, focusing on the occipital transtentorial approach and adding the author's experience to what has been known in the literature. Recent innovations have made this approach more popular and can be applied to occipital fossa lesions.

Asunto(s)

Neoplasias Encefálicas , Glándula Pineal , Pinealoma , Niño , Humanos , Pinealoma/diagnóstico por imagen , Pinealoma/cirugía , Neoplasias Encefálicas/diagnóstico por imagen , Neoplasias Encefálicas/cirugía , Neoplasias Encefálicas/patología , Glándula Pineal/diagnóstico por imagen , Glándula Pineal/cirugía , Procedimientos Neuroquirúrgicos

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BACKGROUND: Tectal region tumors often clinically present as obstructive hydrocephalus due to mass effect on the outflow of the third ventricle and cerebral aqueduct. Pathology in this region varies; thus, biopsy can be of great value in the management decision making. Appropriate instrumentation remains an area of interest to further advance flexible neuroendoscopic techniques and applications. OBSERVATIONS: The authors report an illustrative case using flexible neuroendoscopy through a single burr hole for simultaneous endoscopic third ventriculostomy (ETV) and tectal tumor biopsy using urological cup forceps in a 13-year-old boy who had presented with obstructive hydrocephalus. LESSONS: The authors demonstrate the feasibility of simultaneous ETV and tectal lesion biopsy via flexible neuroendoscopy to address obstructive hydrocephalus and obtain a tissue biopsy in a single-site procedure. They found that the use of flexible cup forceps designed for uroscopy is an important complement to flexible neuroendoscopy. Given the evolving applications of flexible neuroendoscopy, this has implications for instrumentation adaptation and future design.

RESUMEN

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.

Asunto(s)

Neoplasias Encefálicas , Neoplasias Meníngeas , Glándula Pineal , Pinealoma , Adulto , Humanos , Persona de Mediana Edad , Pinealoma/diagnóstico por imagen , Pinealoma/cirugía , Pinealoma/patología , Glándula Pineal/cirugía , Glándula Pineal/patología , Neoplasias Meníngeas/cirugía , Neoplasias Encefálicas/cirugía , Neoplasias Encefálicas/patología , Cadáver

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BACKGROUND: The pineal tumor was once considered as a restricted area for surgery. Such cases are rare, with many different opinions on surgical treatment. This study aimed to review our experience of tumor treatment in the pineal region and explore the optimal treatment strategy. METHODS: The clinical data of 72 patients with pineal tumors from January 1997 to May 2015 (18 years) were retrospectively analyzed. Preoperative preparation, pathology type, tumor resection rate, surgical approach, and follow-up outcomes were used as the indicators to evaluate the treatment efficacy. RESULTS: The Krause approach was used in 46 cases, the Poppen approach in 10 cases, and the transcallosal-lateral ventricle-choroid fissure approach in 16 cases. The postoperative pathological results were as follows: 24 cases of germinoma, 11 of teratoma, 15 of glioma, 6 of meningioma, 11 of Pineocytoma, 2 of cholesteatoma, 2 of cavernous hemangioma, and 1 of choriocarcinoma. Further, the study included 64 cases of total surgical resections, 8 of subtotal resections, and 2 deaths. The follow-up period was from 7 months to 10 years. Further, 51 (70.8%) patients were followed up. The multivariate regression model showed that the surgical method and the pathological type contributed significantly to predicting outcomes. CONCLUSIONS: The type of pathology, extent of excision, and surgical approach had a significant impact on the prognosis of patients. The transcallosal-lateral ventricle-choroid fissure approach for large and medium-sized pineal tumors near the posterior part of the third ventricle had good efficacy.

Asunto(s)

Neoplasias Encefálicas , Neoplasias Meníngeas , Glándula Pineal , Pinealoma , Humanos , Pinealoma/cirugía , Pinealoma/patología , Estudios Retrospectivos , Glándula Pineal/cirugía , Glándula Pineal/patología , Neoplasias Encefálicas/cirugía , Neoplasias Encefálicas/patología , Neoplasias Meníngeas/patología

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PURPOSE: To evaluate the effectiveness of diffusion-weighted imaging (DWI) and susceptibility-weighted imaging (SWI) for differentiation between germinoma and other pineal region tumors. METHOD: This retrospective study consisted of 72 patients with pathologically proven pineal region tumors between January 2010 and August 2020. Tumors were classified as germinomas (40), non-germinomatous germ cell tumors (11) (NGGCT), pineal parenchymal tumors (10) (PPT), and other types of tumors (11). Visual scale score, ADC values and SWI intratumoral susceptibility signal (ITSS) score were analyzed and compared to histopathology data. RESULTS: The mean apparent diffusion coefficient (ADCmean) and minimum apparent diffusion coefficient (ADCmin) ratio of germinoma were significantly lower than NGGCT. ADCmean or ADCmin cut-off ratio of ≤ 1.48 or ≤ 1.32 allowed for discrimination between germinoma and NGGCT with sensitivity and specificity of 100 % and 63.6 %. An ADCmin cut-off ratio of ≥ 0.93 allowed for discrimination between germinoma and PPT with sensitivity and specificity of 60 % and 80.0 %. ADCmin cut-off ratio of ≤ 1.15 allowed for discrimination of germinoma from other types of tumors with sensitivity and specificity of 87.5 % and 54.5 %. CONCLUSIONS: ADC ratio can differentiate germinoma from other types of pineal region tumors. Our initial results suggest that ITSS score was not significantly correlated with specific histology subtype.

Asunto(s)

Neoplasias Encefálicas , Germinoma , Neoplasias de Células Germinales y Embrionarias , Glándula Pineal , Pinealoma , Humanos , Pinealoma/diagnóstico por imagen , Pinealoma/patología , Estudios Retrospectivos , Imagen por Resonancia Magnética/métodos , Imagen de Difusión por Resonancia Magnética/métodos , Germinoma/diagnóstico por imagen , Germinoma/patología , Neoplasias de Células Germinales y Embrionarias/diagnóstico por imagen , Diferenciación Celular , Neoplasias Encefálicas/diagnóstico por imagen , Neoplasias Encefálicas/patología , Glándula Pineal/diagnóstico por imagen , Glándula Pineal/patología

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PURPOSE: Surgical resection of pineal region tumors is challenging because of close proximity to the vein of Galen draining system and the quadrigeminal plate. Surgical resection usually is performed through the narrow corridor by piecemeal resection and en bloc resection is difficult in cases of large tumors. Moreover, in cases of hypervascular tumors, surgical resection through the narrow corridor could entail massive intraoperative bleeding. The effectiveness of neoadjuvant chemotherapy and second-look surgery for pineal region tumors for maximal safe resection was evaluated. METHODS: Retrospective institutional review of pediatric patients with pineal region tumors who underwent second look surgery after neoadjuvant chemotherapy was performed. RESULTS: Nine patients underwent surgical resection after neoadjuvant chemotherapy over the period of September 2017 to February 2022. The mean age was 7.7 years (ranged from 1.4 to 15.3 years). Three patients underwent partial resection via open craniotomy, and 6 underwent endoscopic biopsy as an initial surgery. The histopathological diagnoses were germ cell tumors in 5 patients (yolk sac tumors in 2, germinoma in 2, choriocarcinoma in 1), /rhabdoid tumor (AT/RT) in 2, medulloblastoma in 1, and high-grade glioneuronal tumor in 1. After several courses of chemotherapy, the second-look surgery was performed. The tumor volume was reduced in 8 patients (89%) after chemotherapy except for 1 case of growing teratoma syndrome. The tumor was extended laterally to the ambient cistern in 2 patients, and posteriorly to the tentorial surface of the cerebellum in 3 patients. The lesion was approached through occipital transtentorial approach in 8 patients and infratentorial supracerebellar approach in 1. Intraoperatively, the high vascularity of the tumor was not observed in all cases. Gross total resection (8 patients, 89%) or near total resection (1, 11%) was achieved in all cases. No complications were observed postoperatively in all cases. Eight patients subsequently underwent additional chemo-radiation therapy according to the initial diagnosis. All patients are alive with no evidence of recurrence with a mean follow-up of 33 months. CONCLUSIONS: Neoadjuvant chemotherapy and second-look surgery for pediatric pineal region tumors was considered to be effective in reducing the tumor volume and vascularity, which facilitates the safe maximal tumor resection.

Asunto(s)

Neoplasias Encefálicas , Neoplasias Cerebelosas , Glándula Pineal , Pinealoma , Humanos , Niño , Lactante , Preescolar , Adolescente , Segunda Cirugía , Estudios Retrospectivos , Pinealoma/cirugía , Pinealoma/patología , Neoplasias Encefálicas/diagnóstico por imagen , Neoplasias Encefálicas/cirugía , Neoplasias Encefálicas/tratamiento farmacológico , Glándula Pineal/cirugía , Neoplasias Cerebelosas/patología

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PURPOSE: Resecting pineal region tumors in children is often challenging. Several approaches have been proposed and practiced. A personal series of pediatric pineal region tumors resected through craniotomy with posterior interhemispheric occipital transtentorial (OT) approach are reviewed. We present the surgical techniques, pitfalls, and their results. MATERIAL AND METHODS: Eighty patients ranging in age from 3 months to 21 years old, and treated over 3 decades were reviewed. Hydrocephalus caused the main presenting symptoms and was noted in 74 patients. It was treated prior to the craniotomy for tumor resection with endoscopic third ventriculostomy (ETV) in 33, external ventricular drainage in 26, and precraniotomy shunt in 15. Nine patients had ETV together with endoscopic biopsy. All patients had a parieto-occipital craniotomy in a prone position. Through a tentorial section, a gross total resection of the tumor was attempted except for germinomas. RESULTS: The tumor pathology showed 32 germ cell tumors (GCT), 22 benign astrocytomas, 13 pineal parenchymal tumors, 5 ATRTs, 3 papillary tumors, and 5 others. Of GCTs, 18 were teratomas. The extent of resection consisted of 55 gross total resections, 13 subtotal resections, 10 partial, and 2 biopsies with one postoperative death. Hemiparesis in 2, cerebellar ataxia in another 2, and hemiballismus in 1 were transient and improved over time. One had permanent hemisensory loss and another patient had bilateral oculomotor palsy. Postoperative homonymous hemianopia occurred in 2 patients but subsided over a short period of time. Parinaud's sign was noted in 24 patients, of which 16 were transient. CONCLUSION: The posterior interhemispheric OT approach provides a safe route and comfortable access to the pineal region in children. A great majority of postoperative neurological complications are the results of direct manipulations of the midbrain at tumor resection. Identification and preservation of the tumor-brain interface are of paramount importance. GCTs other than teratomas are treated with neoadjuvant chemotherapy and may eliminate the need for craniotomy. Exophytic midbrain JPAs are amenable to resection.

Asunto(s)

Neoplasias Encefálicas , Glándula Pineal , Pinealoma , Teratoma , Niño , Humanos , Pinealoma/cirugía , Pinealoma/patología , Estudios Retrospectivos , Neoplasias Encefálicas/cirugía , Glándula Pineal/cirugía , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/etiología

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Background: Controversies exist regarding the ideal surgical approach for tumors in posterior third ventricular region (PTV). Objective: To evaluate the results of occipital interhemispheric transtentorial (OITT) approach for tumors in PTV. Materials And Methods: Thirty-three patients underwent surgery via OITT approach for PTV tumors at Sri Sathya Sai Institute of Higher Medical Sciences during the study period of 5 years (June 2011-May 2016). Ideal trajectory for OITT approach was determined by neuronavigation. Endoscope was used for removing any residual lesion at the blind spots. Results: Postoperative magnetic resonance imaging (MRI) performed in all the patients revealed gross total or near-total (>95%) excision of tumor in 31 patients (93.9%). Preoperative neurological deficits improved either completely or significantly following excision of the tumor in 73.3% (11/15) of the patients. Outcome was good (modified Rankin scale ≤2) at discharge in 93.9% (31/33) and at a final follow-up of 3 months or more in 96.8% (30/31) of the patients. None of the patients died during the postoperative period. Complications included upgaze palsy (transient- 6.1% [2/33], persisting- 3% [1/33]), visual field defects (transient- 3% [1/33], persisting- 3% [1/33]), transient third nerve paresis (1/33-3%), transient hemiparesis (1/33-3%), operative site hematoma (1/33-3%), small posterior cerebral artery (PCA) territory infarct (1/33-3%), and small venous infarct (1/33-3%). At least one follow-up MRI could be performed in 23 patients. Final follow-up MRI revealed no recurrence or increase in the size of the residual lesion compared to postoperative images in 20 patients (20/23-87%) and large recurrence in 3 patients (3/23-13%) with high-grade lesions. Conclusion: Gross total/near-total excision can be performed in majority of the PTV tumors through OITT approach with minimal morbidity and mortality.

Asunto(s)

Neoplasias Meníngeas , Meningioma , Glándula Pineal , Tercer Ventrículo , Humanos , Infarto/patología , Neoplasias Meníngeas/cirugía , Meningioma/cirugía , Procedimientos Neuroquirúrgicos/métodos , Glándula Pineal/patología , Tercer Ventrículo/cirugía

RESUMEN

Pineal region tumors are rare intracranial tumors, accounting for less than 1% of all adult intracranial tumor lesions. These lesions represent a histologically heterogeneous group of tumors. Among these tumors, pineal parenchymal tumors and germ cell tumors (GCT) represent the most frequent types of lesions. According to the new WHO 2021 classification, pineal parenchymal tumors include five distinct histotypes: pineocytoma (PC), pineal parenchymal tumors of intermediate differentiation (PPTID), papillary tumor of the pineal region (PTPR), pinealoblastoma (PB), and desmoplastic myxoid tumor of the pineal region, SMARCB1-mutant; GCTs include germinoma, embryonal carcinoma, yolk sac tumor, choriocarcinoma, teratoma, mixed GCTs. Neuroradiological assessment has a pivotal role in the diagnostic work-up, surgical planning, and follow-up of patients with pineal masses. Surgery can represent the mainstay of treatment, ranging from biopsy to gross total resection, yet pineal region tumors associated with obstructive hydrocephalus may be surgically managed via ventricular internal shunt or endoscopic third ventriculostomy. Radiotherapy remains an essential component of the multidisciplinary treatment approach for most pineal region tumors; however, treatment volumes depend on the histological subtypes, grading, extent of disease, and the combination with chemotherapy. For localized germinoma, the current standard of care is chemotherapy followed by reduced-dose whole ventricular irradiation plus a boost to the primary tumor. For pinealoblastoma patients, postoperative radiation has been associated with higher overall survival. For the other pineal tumors, the role of radiotherapy remains poorly studied and it is usually reserved for aggressive (grade 3) or recurrent tumors. The use of systemic treatments mainly depends on histology and prognostic factors such as residual disease and metastases. For pinealoblastoma patients, chemotherapy protocols are based on various alkylating or platinum-based agents, vincristine, etoposide, cyclophosphamide and are used in association with radiotherapy. About GCTs, their chemosensitivity is well known and is based on cisplatin or carboplatin and may include etoposide, cyclophosphamide, or ifosfamide prior to irradiation. Similar regimens containing platinum derivatives are also used for non-germinomatous GCTs with very encouraging results. However, due to a greater understanding of the biology of the disease's various molecular subtypes, new agents based on targeted therapy are expected in the future. On behalf of the EURACAN domain 10 group, we reviewed the most important and recent developments in histopathological characteristics, neuro-radiological assessments, and treatments for pineal region tumors.

RESUMEN

BACKGROUND/AIM: To review the current literature on pineal region gliomas, summarizing the clinical characteristics and treatment outcomes. MATERIALS AND METHODS: PubMed, Scopus, and Cochrane databases were used to identify relevant articles. Comprehensive clinical characteristic review and survival analysis were conducted. RESULTS: Twelve studies describing 81 patients were included. The median age was 39 years (male=54.3%). Fifty patients (61.7%) had obstructive hydrocephalus requiring cerebrospinal fluid diversion with either ventriculoperitoneal shunt (VPS) (40.0%) or endoscopic third ventriculostomy (ETV) (24.0%). Patients who underwent VPS had significant survival benefits compared to ETV (p<0.05). All patients in our review underwent surgery, and gross-total resection (≥98%) was achieved in 34.6%. The supracerebellar infratentorial approach was the most employed surgical approach (62.3%). Chemotherapy was administered in 32.1% of cases, and radiotherapy in 40.7%. The median overall survival (OS) was 12 months, and the overall one-year survival rate was 60%. CONCLUSION: This study could not establish a correlation between the extent of tumor resection and positive treatment outcomes. However, among cases with hydrocephalus, patients who underwent VPS placement had better survival as compared to ETV.

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Neoplasias Encefálicas/cirugía , Glioma/cirugía , Glándula Pineal/cirugía , Derivación Ventriculoperitoneal , Ventriculostomía , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Neoplasias Encefálicas/mortalidad , Neoplasias Encefálicas/patología , Quimioterapia Adyuvante , Femenino , Glioma/mortalidad , Glioma/patología , Humanos , Masculino , Persona de Mediana Edad , Recurrencia Local de Neoplasia , Glándula Pineal/patología , Supervivencia sin Progresión , Radioterapia Adyuvante , Factores de Riesgo , Factores de Tiempo , Derivación Ventriculoperitoneal/efectos adversos , Derivación Ventriculoperitoneal/mortalidad , Ventriculostomía/efectos adversos , Ventriculostomía/mortalidad , Adulto Joven

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The chapter outlines the management of a great variety of rare intraparenchymal tumors. For some like medulloblastomas or ependymomas, GKNS has little to offer. For others like neurocytomas, primary central nervous system lymphomas, and papillary pineal tumors the current findings look most hopeful. For choroid plexus papillomas there is currently not enough information to define the role of GKNS in their treatment. Pineal region tumors are a complex and varied group of neoplasms. Their complexity, variety and ethnic variability means that defining the role of GKNS will require continuing research before a consensus about management can be reached.

Asunto(s)

Ependimoma , Ependimoma/patología , Humanos

RESUMEN

BACKGROUND: High-grade pineal region tumors are rare and heterogeneous types of primary central nervous system neoplasms; radiological differential diagnosis is challenging but it is important because it has a therapeutic relevance. PURPOSE: To discriminate among high-grade pineal region tumors by combining apparent diffusion coefficient (ADC) volumetric values and qualitative features in order to predict their histology. MATERIAL AND METHODS: Twenty-two patients with high-grade pineal region tumors were assessed by qualitative and quantitative analysis. Margins, T2-weighted signal intensity, contrast enhancement, hemorrhage, calcifications, different volumetric ADC fractions (ADCmean, ADCmax, ADCmin) were evaluated and were compared to the histopathologic findings (cell count and proliferation index). RESULTS: Our qualitative imaging data showed that only margins were different among different tumors and each tumor type showed peculiar age onset. ADCmean was found the best quantitative value to discriminate high-grade tumors of the pineal region. ADCmean correlated with proliferation index but not with cell count. ADCmean values were lower in tumors with higher proliferation rate and a significant difference in ADCmean values were found between germinomas and pineoblastomas, between germinomas and papillary tumors and between papillary tumors and pineoblastomas. Moreover, the cut-off value of 0.865 × 10-3 mm2/s for ADCmean (ADC mean threshold value) could differentiate germinoma from pineoblastomas with the best combination of sensitivity and specificity. CONCLUSION: The ADCmean value measured on the whole tumor, reflecting tumor proliferative activity, may be a practical and non-invasive marker for predicting tumor histology in high-grade pineal region lesions and might be useful in preoperative assessment.

Asunto(s)

Neoplasias Encefálicas/diagnóstico por imagen , Neoplasias Encefálicas/patología , Imagen de Difusión por Resonancia Magnética , Neuroimagen , Glándula Pineal/diagnóstico por imagen , Glándula Pineal/patología , Adolescente , Adulto , Calcinosis/diagnóstico por imagen , Calcinosis/patología , Proliferación Celular , Hemorragia Cerebral/diagnóstico por imagen , Hemorragia Cerebral/patología , Niño , Preescolar , Femenino , Humanos , Masculino , Persona de Mediana Edad , Clasificación del Tumor , Estudios Retrospectivos , Sensibilidad y Especificidad , Adulto Joven

RESUMEN

Anaplastic ependymoma in the pineal region is rare. Here, we present a rare case of anaplastic ependymoma of the pineal region on a 42-year-old woman who came to our hospital with headache associated with blurring of vision since one month. MRI brain showed a contrast enhancing mass lesion measuring 30 × 30 × 35 mm in the pineal region with obstructive hydrocephalus. Initially, the ventriculoperitoneal (VP) shunt was done, followed by total tumor excision by the infratentorial supracerebellar approach. Histopathological and immunohistochemistry examinations of the tumor showed the features of anaplastic ependymoma (WHO Grade III). Patient made uneventful recovery and underwent radiotherapy. Only 12 cases of pineal ependymoma have been reported so far, of which only three have been anaplastic ependymoma.

Asunto(s)

Ependimoma , Hidrocefalia , Glándula Pineal , Adulto , Ependimoma/diagnóstico por imagen , Ependimoma/cirugía , Femenino , Humanos , Imagen por Resonancia Magnética , Glándula Pineal/diagnóstico por imagen , Glándula Pineal/cirugía , Derivación Ventriculoperitoneal

RESUMEN

Background: 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. Method: 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. Result: Eighteen children successfully underwent tumor resection via MS (n = 8) or EAM (n = 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). Conclusion: 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.