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Background: Pineal parenchymal cell tumors constitute a rare group of primary central nervous system neoplasms (less than 1%). Their classification, especially the intermediate subtype (PPTIDs), remains challenging. Methods: A literature review was conducted, navigating through anatomo-pathological, radiotherapy, and neurosurgical dimensions, aiming for a holistic understanding of these tumors. Results: PPTIDs, occupying an intermediate spectrum of malignancy, reveal diverse histological patterns, mitotic activity, and distinct methylation profiles. Surgical treatment is the gold standard, but when limited to partial removal, radiotherapy becomes crucial. While surgical approaches are standardized, due to the low prevalence of the pathology and absence of randomized prospective studies, there are no shared guidelines about radiation treatment modalities. Conclusion: Surgical removal remains pivotal, demanding a personalized approach based on the tumor extension. This review underscores the considerable variability in treatment approaches and reported survival rates within the existing literature, emphasizing the need for ongoing research to better define optimal therapeutic strategies and prognostic factors for PPTIDs, aiming for further and more detailed stratification among them. [ABSTRACT FROM AUTHOR]

This article discusses the case of an 18-year-old female who presented with a large tumor in the pineal region of her brain. The tumor caused symptoms such as headaches and vision loss. After undergoing surgery, the tumor was identified as a desmoplastic myxoid tumor (DMT) of the pineal region, with a mutation in the SMARCB1 gene. DMTs with SMARCB1 mutations are rare and have unique histological and molecular characteristics. The prognosis and clinical outcomes of this tumor are still uncertain due to its rarity. [Extracted from the article]

Purpose: Atypical teratoid/rhabdoid tumour (AT/RT) is a highly malignant central nervous system tumour of early childhood. According to the latest WHO classification, the diagnosis of AT/RTs needs to be confirmed by the absence of SMARCB1 (INI1) or SMARCA4 (BRG1) protein expression. AT/RT in the pineal region is infrequent and most have not been proven genetically. Here, we report a case of AT/RT in the pineal region, preoperatively misdiagnosed as a meningioma. Immunohistochemistry revealed the absence of INI1 protein expression. Method: A 29-month-old boy was admitted to the hospital after 14 days of emotional apathy and a 2-day vomiting history. AT/RT was not considered during the initial diagnosis because this tumour is rare in this region and is often accompanied by cystic degeneration and necrosis on imaging. Subsequently, the patient underwent surgery and the tumour was completely excised. Result: The pathological diagnosis was AT/RT. After discharge, the patient continued chemotherapy in other hospitals but died five months after surgery because of disease progression. Conclusion: To our knowledge, this is the fifth case of paediatric pineal AT/RT confirmed genetically. Although in children AT/RT in the pineal gland is rare, a differential diagnosis of AT/RT should be considered when new pineal masses appear in children. For this highly malignant disease with poor prognosis, it is very important to detect and recognize the disease as soon as possible, and to adopt surgery plus multiple treatment management. [ABSTRACT FROM AUTHOR]

Background: Management options for treatment of quadrigeminal arachnoid cysts (QAC) include microsurgical/endoscopic fenestration or shunt. There is an open debate about which method is the best. Microsurgical fenestration is well suited for treatment of QAC with predominant infratentorial component and without hydrocephalus making endoscopic procedures more challenging. Method: We describe the microsurgical technique and related anatomy to fenestrate infratentorial QAC through supracerebellar infratentorial approach. We also discuss our experiences with this approach, some of the drawbacks and nuances. Conclusion: Navigation-guided microsurgical fenestration of infratentorial QAC is the authors' surgical approach of choice for treating these rare challenging lesions when not associated with hydrocephalus. [ABSTRACT FROM AUTHOR]

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Background: Pineal parenchymal cell tumors constitute a rare group of primary central nervous system neoplasms (less than 1%). Their classification, especially the intermediate subtype (PPTIDs), remains challenging. Methods: A literature review was conducted, navigating through anatomo-pathological, radiotherapy, and neurosurgical dimensions, aiming for a holistic understanding of these tumors. Results: PPTIDs, occupying an intermediate spectrum of malignancy, reveal diverse histological patterns, mitotic activity, and distinct methylation profiles. Surgical treatment is the gold standard, but when limited to partial removal, radiotherapy becomes crucial. While surgical approaches are standardized, due to the low prevalence of the pathology and absence of randomized prospective studies, there are no shared guidelines about radiation treatment modalities. Conclusion: Surgical removal remains pivotal, demanding a personalized approach based on the tumor extension. This review underscores the considerable variability in treatment approaches and reported survival rates within the existing literature, emphasizing the need for ongoing research to better define optimal therapeutic strategies and prognostic factors for PPTIDs, aiming for further and more detailed stratification among them.

Objective To evaluate the safety and efficacy of stereotatic radiosurgery（SRS） in treating residual lesions of pineal non-germinomatous germ cell tumors（NGGCTs） after conventional radiotherapy. Methods The clinical data of patients with intracranial germ cell tumors admitted to Guangdong Sanjiu Brain Hospital from January 2008 to December 2018 who diagnosed with pineal NGGCTs pathologically or clinically were analyzed retrospectively. Among those, the patients received conventional radiotherapy with SRS were included. The residual lesions after radiotherapy were defined with a maximum diameter ＞10 mm. Prognosis related parameters such as local control rate, progress-free survival, overall survival and treatment-related toxicity were determined. Results Total of 27 patients were included in this study. Both of them were males with the median age of 16 years old. The median follow-up time was 34 months（range 8-142 months）. The objective response rate and disease control rate were 71.4% and 95.2%, respectively. Three-year progression-free survival rate was 85.2% and 3-year total survival rate was 88.0%. The univariate analysis revealed that both age and concurrent chemotherapy were not correlated with the prognosis（P=0.286,0.824）. Partial tumor resection before radiotherapy and chemotherapy, AFP＞500 ng/mL, and less than 4 cycles of adjuvant chemotherapy were poor prognostic factors（P=0.037,0.010,0.006）. Moreover, no acute radiation injury was observed after SRS. Only 1 out of 27 patients（ 3. 7%） had brain neurotoxicity related to a prolonged course of radiochemotherapy.Conclusions SRS for residual lesions of NGGCTs following conventional radiotherapy appears to be well tolerant and improved local control. However, the therapeutic efficacy of conventional radiotherapy combined with SRS warrants further investigations in a large-scale randomized controlled clinical trials. [ABSTRACT FROM AUTHOR]

The pineal region is an anatomical region that is difficult to access surgically, especially when it comes to removing neoplasms. Four main surgical approaches to this region are used as standards nowadays: infratentorial supracerebellar, occipital supra-/transtentorial, interhemispheric, and transventricular approaches. All methods have both advantages and disadvantages and are associated to any extent with intra- and postoperative risks. We have developed a lateral minimally invasive occipital infracortical supra-/transtentorial (OICST) approach, which retains the advantages of the standard occipital transtentorial approach while improving tumor exposure and minimizing its disadvantages. We describe 7 consecutive cases of successful complete removals of pineal tumor formations of various quality and size (3 pineal cysts, 2 pineocytomas, 1 meningioma, 1 medulloblastoma) using the OICST approach developed by us. Preoperative 3-dimensional and virtual reality-modeling and the use of a special retractor also contributed to reducing the size of the surgical approach. All patients underwent surgery for removal of a lesion in the pineal region and suffered from no new and permanent neurological deficits postoperatively. The mean size of the craniotomies was 2.3 × 1.85 cm. The minimally invasive approach developed by us carries the advantages of the standard occipital transtentorial approach, but minimizes its disadvantages. The main disadvantage of the standard occipital approach is excessive retraction of the occipital lobe, which is frequently associated with visual neurological deficits. Also, with occipital approach, the Rosenthal vein lying along the surgical corridor is frequently not good visible since the tumor is approached from its tip rather than side which limits the overview of the surgical field and can pose a risk. Damage to this vein can cause infarction of the basal ganglia. By approaching the pineal region from more laterally the size of the craniotomy can significantly be reduced, excessive retraction of the occipital lobe can be avoided and the risk of damage to large deep veins can be minimized. The cosmetic outcome with a small skin incision of only about 3 cm is also a very good side effect of this minimally invasive technique. The minimally invasive lateral OICST approach described by us can be successfully used in the surgery of pineal neoplasms. Reducing the size of the craniotomy does not limit the possibility of complete removal of tumors of various sizes and tissue consistency, and also minimizes the risks of both intra- and postoperative complications. [ABSTRACT FROM AUTHOR]

Background: Falcotentorial meningioma is a rare tumor of pineal region, arising from the dural folds where the tentorium and falx meet. Due to the deep location and near closeness to significant neurovascular structures, gross-total tumor resection in this area can be complicated. Pineal meningiomas can be resected using a variety of approaches; however, all these approaches are associated with a significant risk of postoperative complications. Case Description: A 50-year-old female patient who presented with several headaches and visual field defect and diagnosed with pineal region tumor is discussed in the case report. Patient was successfully managed surgically by combined supracerebellar infratentorial and right occipital interhemispheric approach. Cerebrospinal fluid circulation was restored after surgery and neurological defects were regressed. Conclusion: Our case shows that it is possible to completely remove giant falcotentorial meningiomas with minimal brain retraction, preserve the straight sinus and vein of Galen, and prevent neurological impairments by combining two approaches. [ABSTRACT FROM AUTHOR]

Abstract Background We report a rare case in medical literature of a patient with pineal gland teratoma and uncommon metastases. Usually, metastases of this kind of tumor are located in several organs such as lung and breast, but here we found metastases to the spinal cord and vertebrae. Case presentation A 35-year-old Asian white man presented with diplopia and acute neural symptoms in the lower limbs such as numbness, tingling, and paralysis. His medical history was notable for pineal teratoma, treated 1 year previously with surgery, radiotherapy, and chemotherapy. Physical examination of the lower limbs showed absent reflexes and sensation with muscle power scale score of 1 in both limbs. Magnetic resonance imaging of brain and spine revealed many lesions in various locations, most compatible with neural, spinal, and vertebral metastases. Unfortunately, the patient died suddenly before any intervention was carried out. Conclusion It is extremely rare for pineal region teratoma to metastasize to the spinal cord and vertebrae, thus more vigilant observation and examination should be provided to patients with pineal teratoma to detect any new lesions and prevent them from becoming dangerous.

Background and objectives: The occipital transtentorial approach (OT) is a useful surgical approach for the treatment of lesions of the pineal region. Because the approach provides wide operative views of superior cerebellar, splenium, quadrigeminal regions can also be operated using this technique. However it may be associated with complications due to damage to the occipital lobe, which can be caused by brain retraction injury. The study evaluates the effectiveness of the OT approach in terms of surgical outcome with special emphasis on occipital lobe functions which may be affected using this approach. Methodology: Seventeen patients of pineal region lesions were operated by OT approach, and preoperative evaluation of the anatomy of the tentorial angle was performed and its significance was studied. Various occipital lobe functions were evaluated postoperatively and compared with preoperative status. Surgical outcome was evaluated in terms of extent of tumour resection, postoperative occipital lobe functions and modified Rankin scale. Results: The mean angle between the tentorium and occipital surface of cerebellum (tentorial angle) was 62.08 ± 7.17 degrees. The maximum and minimum tentorial angle was 83 and 51 degrees respectively. Out of 13 patients in which the postoperative functional assessment of occipital lobe functions was possible, 2 patients developed homonymous hemianopia (one transient, one permanent). Other functions remain unaffected. Near and gross total resection was possible in 4 (23.5 %) and 8 (47.1 %) patients respectively. The mean modified Rankin scale score was 1.8 indicating slight post-operative disability. Conclusions: Amongst the various occipital lobe functions, the visual field defects are the most common functions affected postoperatively in the occipital transtentorial approach. The OT approach is an appropriate and reasonable approach for the surgical management of pineal region lesions where high resectability rates can be achieved irrespective of the status of anatomical factors like tentorial angle; with slight postoperative morbidity with respect to occipital lobe functions.

This study was to analyze the advantages and disadvantages of endoscopic midline and paramedian supracerebellar infratentorial approaches (EM-SCITA and EPM-SCITA) for pineal region tumors. We retrospectively analyzed the clinical data of 58 patients who underwent EM-SCITA and EPM-SCITA for pineal region tumors. Among them, 23 patients were treated with EM-SCITA, and 35 with EPM-SCITA. The patients were followed up for 6–84 months with magnetic resonance imaging and Karnofsky Performance Status scores. The average age of the patients was 37.98 years, and there were 16 women (27.6%). The average maximum diameter of the tumors was 2.92 cm. Gross total resection was achieved in 46 patients (79.31%). There were 45 patients (77.6%) whose Karnofsky Performance Status score was >70 at the final follow-up. There was no significant difference among the above items between EM-SCITA and EPM-SCITA. However, EM-SCITA required a longer craniotomy and closure time, with a larger bone and dural flap, with more bridging veins sacrificed. EPM-SCITA simplified the opening of the quadrigeminal cistern, and it was beneficial to expose the contralateral wall of the third ventricle. The longer and angled path limited the exposure of the anterior third ventricle and the ipsilateral wall of the third ventricle. Both approaches had remarkable clinical effects. The anatomy of EM-SCITA was easy to understand and has a larger operating space; it is suitable for neurosurgical beginners. In contrast, EPM-SCITA has limited operation space, an intricate anatomy, and is suitable for experienced operators. The occurrence of postoperative hydrocephalus should be alerted by EPM-SCITA. [ABSTRACT FROM AUTHOR]

Simple Summary: The WHO classification of tumors of the central nervous system described for the first time the extremely rare entity of rosette-forming glioneuronal tumors (RGNT, CNS WHO grade 1) in 2007. Due to the rarity of this entity in the pineal region, no specific therapy guidelines currently exist. With our large cohort of patients treated at a single center (from August 2018–June 2021) and with the already described cases in the literature, we would like to highlight possible therapy and follow-up concepts. After the main symptoms of headache or generalized epileptic seizure, cystic lesions adjacent to the pineal gland and the third ventricle were diagnosed in imaging. None of the patients underwent additional chemotherapy or radiotherapy after gross total (GTR)/subtotal resection or endoscopic biopsy. In cases where surgical resection seems feasible with a reasonable surgical risk, we advocate GTR. Long-term MRI follow-up is essential to detect a slow tumor progression. Background: Rosette-forming glioneuronal tumor (RGNT) is an extremely rare entity described for the first time in the WHO classification of tumors of the central nervous system in 2007. Predominantly, single case reports of RGNT in the pineal region have been published, and specific therapy concepts are pending. Methods: The study group comprised all patients with the RGNT (CNS WHO grade 1) in the pineal region that underwent microsurgical tumor removal in our center (August 2018–June 2021). Surgical strategy, histological findings, and clinical outcome are presented, and the results are evaluated and compared to published case reports. Results: Four male patients aged under 50 years (range between 20 and 48 years) and one female patient, 51 years old, were included in this study. Chronic headaches and generalized epileptic seizures were the main symptoms. Supra-cerebellar infratentorial gross total tumor resection (GTR) was performed in two cases, two patients underwent subtotal tumor resection, and an endoscopic biopsy was performed in case five. Conclusion: In cases where surgical resection seems feasible with a reasonable surgical risk, we advocate GTR. Regular and long-term MRI follow-up is essential to detect a slow tumor progression. The role of additional chemotherapy or radiotherapy remains unclear. [ABSTRACT FROM AUTHOR]

Introduction: Surgery of lesions in the posterior wall of the third ventricle requires great expertise due to its deep location and important surrounding structures. This region has been traditionally reached through a supracerebellar infratentorial approach, but new options have emerged, especially with the development of neuroendoscopy., Methods: One formalin-fixed cadaver human head was dissected. Five different endoscopic approaches were performed: interhemispheric transcallosal transchoroidal, frontal transforaminal transchoroidal, supraorbital subfrontal translamina terminalis, expanded endonasal, and supracerebellar infratentorial. An anatomical description of the different approaches was conducted and quantitative measurements (craniocaudal and latero-lateral distances) were taken using the StealthStation ® workstation after performing a CT scan of the specimen., Results: The interhemispheric transcallosal transchoroidal, frontal transforaminal transchoroidal, and supraorbital subfrontal translamina terminalis approaches provided great view of all the structures of the posterior wall of the third ventricle. Maximum craniocaudal distance was obtained through the supraorbital subfrontal translamina terminalis approach (10.6 mm), with great difference from the expanded endonasal approach (5.2 mm). The widest latero-lateral distance from inside the third ventricle was achieved through the interhemispheric transcallosal transchoroidal approach (4.6 mm), similar to the expanded endonasal (4.1 mm), and differing from the supraorbital subfrontal translamina terminalis (2.4 mm)., Conclusions: The endoscopic approaches provided an adequate alternative to more traditional microsurgical approaches to the posterior wall of the third ventricle, with a great view of all its structures. The selection of the approach must be taken under consideration in each case., Competing Interests: Declarations of interest None., (Copyright © 2024 The Authors. Published by Elsevier B.V. All rights reserved.)

Pineal region pilocytic astrocytomas are extremely rare, and there is limited information about their radiological features. We report the case of a 22-year-old woman with a cystic lesion in the pineal region. In the 2 years after diagnosis, the lesion irregularly extended along the bilateral internal cerebral veins and the inferolateral surface of the corpus callosum. Gross total resection was achieved, and the histopathological study revealed that the lesion was a pilocytic astrocytoma. The lesion exhibited uncommon growth, leading to difficulty in establishing an accurate preoperative diagnosis. It should be noted that pineal region pilocytic astrocytomas can demonstrate atypical appearances.

Abstract Background Pineal region tumors are commonly present in the pediatric age group. However, pleomorphic xanthoastrocytoma (PXA) is very rare at this region, and only few case reports have been reported till now in literature. Case presentation Here, we report a rare case of pineal region, juxta-thalamic, pleomorphic xanthoastrocytoma (PXA) in an 11-year-old male child. The child presented with severe headache after which MRI was done. It was suggestive of pineal region low-grade tumor. The patient was operated in Parkbench position with SCIT (supracerebellar approach) in a retractor-free manner. Gross total resection was done. However, the patient developed postoperative left-sided hemiparesis. It got improved in 1 month, and the patient became ambulatory. Histopathology came out as pleomorphic xanthoastrocytoma. Conclusion Surgical management should include careful resection near the internal capsule to avoid postoperative hemiparesis. Also, shunt should be delayed in the cystic cavity created by resection of tumor.

Background : The pineal region and dorsal midbrain are among the most challenging surgical targets. To approach lesions in this region that harbor a superior to inferior long axis, we describe the basic steps of the precuneal, interhemispheric, trans-tentorial approach and illustrate anatomical landmarks of this established, but not so popular, surgical trajectory. Method: To study the anatomical landmarks and safety of this approach, the neurovascular anatomy was studied on 22 sides of 11 formalin-fixed latex-injected anatomical specimens. A step-by-step dissection of the precuneal interhemispheric trans-tentorial approach and study of the key anatomical landmarks was performed. An illustrative clinical case of a pontomesencephalic cavernous malformation (CM) resected through this approach is also detailed. Results: The mean distance from the transverse sinus to the most posterior cortical vein draining into the superior sagittal sinus was 6.4 cm. The mean distance from the calcarine sulcus to the most posterior cortical vein was 5.3 cm. Key steps of the dissection are as follows: craniotomy exposing the posterior aspect of the superior sagittal sinus (SSS), durotomy and gentle retraction of the SSS edge, dissection of the interhemispheric fissure, linear incision of the tentorium that extends anteriorly to the incisura and lateral reflection of the tentorium, and arachnoidal dissection and exposure of the cerebellomesencephalic fissure. Conclusion: The precuneal, interhemispheric, trans-tentorial approach affords excellent access to the falcotentorial junction, splenium, pineal region, quadrigeminal cistern, and dorsal pons once the cerebellomesencephalic fissure has been dissected. [ABSTRACT FROM AUTHOR]

Vascular lesions of the pineal region comprise aneurysms of the pineal region, arteriovenous malformations, cavernous malformations, and vein of Galen malformations. In the present report, we have offered an extensive review of each vascular pineal region lesion. We performed an extensive literature review, focusing on the current therapeutic options available for the different vascular lesions of the pineal region. Vascular lesions of the pineal region are rare. Microneurosurgery remains a valid treatment of cavernomas, arteriovenous malformations, and aneurysms. Endovascular treatments seem to be the first option for the vein of Galen malformations, followed by microneurosurgery. Radiosurgery seems beneficial for small-size arteriovenous malformations. Complex and large vascular lesions will require a combination of multiple treatments. Vascular lesions of the pineal region are complex, uncommon diseases. Thus, definitive therapeutic modalities for these lesions require further research. [ABSTRACT FROM AUTHOR]

Purpose: Pineal region tumors are surgically demanding tumors to resect. Long term neuro-oncologic outcomes following surgical excision of tumors from this region have been underreported. We sought to define the long term outcomes of patients undergoing resection of pineal region tumors. Methods: A retrospective analysis of a prospectively maintained database was performed on patients who underwent intended surgical excision of pineal region tumors. Overall survival (OS) and progression free survival (PFS) were the primary endpoints of this study. Factors associated with OS, PFS and the degree of resection were analyzed, along with 30-day complication rates and dependence on CSF diversion. Results: Sixty-eight patients with a mean age of 30.9 ± 15.3 years were analyzed. The median clinical and radiographic follow-up was 95.7 and 48.2 months, respectively. The supracerebellar infratentorial and the occipital transtentorial corridors were utilized in the majority of cases (80.9%). The gross total resection (GTR) rate was 52.9% (n=36). The 5-year OS and PFS rates were 70.2% and 58.5%, respectively. Achieving GTR was associated with improved OS (HR 0.39, p = 0.03) and PFS (HR 0.4, p = 0.006). The 30-day mortality rate was 5.9%. The need for CSF diversion was high with 77.9% of patients requiring a shunt or ETV by last follow-up. Conclusions: This is the first modern surgical series providing long term follow-up for patients undergoing surgical resection of pineal region tumors. Obtaining a GTR of these challenging tumors is beneficial with regards to PFS/OS. Higher grade tumors have diminished PFS/OS and are treated with adjuvant chemotherapy and/or radiotherapy. [ABSTRACT FROM AUTHOR]

Background: Papillary tumor of the pineal region (PTPR) represents a rare and histologically distinct subgroup of tumors originating in the pineal region. Few pediatric cases have been reported so far in the literature; therefore, clinical data are scarce. Case Description: We describe a case of PTPR in a 9-year-old girl who presented with a 5-month history of excessive appetite and weight gain. The patient underwent neuroimaging procedures and total gross surgical resection with postoperative adjuvant local radiotherapy, which from our experience was the best treatment choice as an attempt to avoid local recurrence. During 78-month follow-up, the patient from our study manifested no disease recurrence. Conclusion: PTPR should be included in the differential diagnosis of pineal region masses. [ABSTRACT FROM AUTHOR]

Background: Pineal cysts (PCs) are benign lesions commonly found on intracranial imaging. Despite their high prevalence, there is no clear consensus on the most appropriate management of patients with PCs, especially those with symptomatic nonhydrocephalic cysts. Methods: A retrospective analysis was performed on 142 patients with PCs (103 surgical cases and 39 conservatively managed cases). Data were examined, including clinical presentation, imaging findings, ophthalmological status, natural course, postoperative outcomes, and complications. Results: Surgical group: the most common symptom was headache (92%), followed by signs of intracranial hypertension due to hydrocephalus (22%). New radiological feature of PCs was found in 11 patients. From 71 patients with long-term follow-up, headache completely resolved in 44 (62%) patients; marked improvement was observed in 20 (29%); in 7 (9%) - headache remained unchanged. The most common postoperative complication was neuro-ophthalmological disorders (23%), with a tendency for resolution in the long-term follow-up period. Neuro-ophthalmological symptoms at last follow-up included upward gaze palsy (6%) and skew deviation (5%), followed by convergence disorders (3%) and eyelid-retraction (2%). Natural course group: PC size remained stable in 34 (87%) patients during the follow-up period. The patient's gender or age was not a significant predictor of cyst growth (P = 0.4, P = 0.56). Conclusion: The majority of patients with a newly diagnosed PC remain clinically and radiologically stable. Patients with nonhydrocephalic PCs and intractable headaches experience significant relief in headache symptoms, but are at risk of mild to moderate neuro-ophthalmological disorders. The natural course of PCs and factors promoting their growth still remains poorly defined. [ABSTRACT FROM AUTHOR]

A 31‐year‐old man suffered from headaches and presented at a hospital after the symptom worsened. Obstructive hydrocephalus and a pineal tumor were identified, and he was transferred to our hospital for further investigation and treatment. Cranial computed tomography revealed a hypodense mass lesion on the right of the pineal region, and calcifications and enlargement of the lateral and third cerebral ventricles were also evident. Blood tests were negative for all tumor markers. Laparoscopic biopsy and third‐ventricle fenestration were performed that day as an emergency surgery to treat the obstructive hydrocephalus. Postoperative cranial magnetic resonance imaging revealed a solid tumor that was hypointense on T1‐weighted imaging, hyperintense on T2‐weighted imaging, and heterogeneously enhanced by Gd. Subsequently, the tumor increased in size, and craniotomy and tumorectomy were performed. Histologically, the tumor proliferated as round or short spindle‐shaped cells in a myxoid matrix, forming arrays that surrounded the blood vessels. As a few cells with eosinophilic cytoplasm were also present and immunostaining for INI‐1 was negative, the patient was diagnosed with atypical teratoid/rhabdoid tumor (AT/RT). AT/RT of the pineal region in adults is rare, and herein, we report the morphological characteristics of this case and reviewed the relevant literature. [ABSTRACT FROM AUTHOR]

Background: The occipital transtentorial (OT) approach is well-established approach for pineal region tumors and can be of choice for the lesions located around the suboccipital part of tentorium such as the quadrigeminal plate, posterior part of thalamus, tentorial surface of cerebellum, splenial region, posterior falx, and lesions around the tentorial incisura. However, it is not very much extensively used in the above-mentioned locations other than the pineal region. Methods: Thirty-one patients of pineal region lesions were operated by OT approach, the role of conventional preoperative evaluation of the anatomy of the venous sinuses, deep venous system, and tentorial angle was investigated. Results: A variety of lesions were operated using this approach achieving gross and near total resection in majority of the cases (76.6%), with acceptable postoperative mean modified Rankin scales (1.8). Conclusion: The OT is a preferable approach for pineal region lesions for patients of all ages and can be tailored for achieving high resectability rates irrespective of the status of the deep venous system and tentorial angle, with reasonable postoperative surgical outcome. [ABSTRACT FROM AUTHOR]

Desmoplastic myxoid tumor (DMT), SMARCB1-mutant is a recently proposed brain tumor that occurs in the pineal region of adults. This tumor is characterized by desmoplastic stroma and various degrees of myxoid matrix. Tumor cells with low-grade morphology show polyphenotypic immunoreactivity, and rhabdoid cells are rare. We herein present a case with some uncommon features such as no myxoid stroma and slightly elevated proliferating activity. To date, knowledge on the variety of SMARCB1/INI1-deficient tumors of the central nervous system is gradually increasing, encompassing highly aggressive to slow-growing varieties. DMT, SMARCB1-mutant seems to be relatively benign, but careful attention is necessary because SMARCB1/INI1 deficiency is generally a genetic signature of concern. [ABSTRACT FROM AUTHOR]

Abstract Background A series of patients harboring pineal region meningiomas were respectively analyzed to explore the origin of these tumors and the true meaning of the term “velum interpositum (VI) meningiomas”. Methods 21 patients with pineal meningiomas underwent operation in Nanfang Hospital of Southern Medical University from January 2005 to December 2016 were retrospectively included to analyze the clinical features, imaging findings and surgical video data of these patients. According to the method of literature, the data of this group were also divided into falcotentorial (FT) meningiomas and VI meningiomas, and the differences between the two types of tumors were compared. Results Among the 21 cases of tumor, there were 12 cases of FT meningiomas, including 4 cases originating from cerebral falx, 4 cases from tentorium of cerebellum and 4 cases from straight sinus; there were 9 cases of VI meningiomas, 7 of which originated from the arachnoid sleeve of the Galen vein, 1 from the posterior part of the internal cerebral vein and 1 from the posterior surface of the pineal gland. Postoperative pathological examination showed meningiomas in all the 21 patients, including 16 cases of total resection and 5 cases of subtotal resection. Postoperatively limitation of binocular vertical motion was found in 3 cases, homotropic hemianopia in 7 cases, hemiplegia in 1 case and death in 1 case. Conclusions This study suggests that pineal meningiomas are more suitable to be described by FT meningioma and meningiomas of the arachnoid of the pineal region by analyzing the origin of tumors. The term “VI meningiomas” can only reflect a part of meningiomas of the arachnoid of the pineal region. Before the removal of pineal meningiomas, more attention should be paid to the effects of the two types of tumors on the Galen vein and the straight sinus, and the establishment of venous collateral circulation.

Background: Papillary tumors of the pineal region are rare neuroepithelial lesions that were described for the 1st time in the WHO 2007 classification. Management of such lesions remains controversial. Case Description: We describe the case of a 26-year-old female who presented with intracranial hypertension syndrome secondary to a 1.9 cm3 lesion of the pineal region causing hydrocephalus. The patient benefited from an endoscopic third ventriculocisternostomy and a biopsy of her lesion in favor of a papillary tumor of the pineal region. After discussion of the surgical risks, the patient refused the surgical option and a stereotactic radiosurgery (SRS) was performed. She improved both clinically (allowing her to regain autonomy) and radiologically (reduction of 60% of tumor volume) at 1 year follow-up. Conclusion: Because of the rarity of the lesion, literature is yet not able to find consensus concerning management of such lesion, but SRS has proven efficiency for these Grades II or III lesions with high recurrence rates. Therefore, it should be considered as a primary therapeutic option allowing good outcome with low risks for the patient. [ABSTRACT FROM AUTHOR]

Background: Pineal region tumors are commonly present in the pediatric age group. However, pleomorphic xanthoastrocytoma (PXA) is very rare at this region, and only few case reports have been reported till now in literature. Case presentation: Here, we report a rare case of pineal region, juxta-thalamic, pleomorphic xanthoastrocytoma (PXA) in an 11-year-old male child. The child presented with severe headache after which MRI was done. It was suggestive of pineal region low-grade tumor. The patient was operated in Parkbench position with SCIT (supracerebellar approach) in a retractor-free manner. Gross total resection was done. However, the patient developed postoperative left-sided hemiparesis. It got improved in 1 month, and the patient became ambulatory. Histopathology came out as pleomorphic xanthoastrocytoma. Conclusion: Surgical management should include careful resection near the internal capsule to avoid postoperative hemiparesis. Also, shunt should be delayed in the cystic cavity created by resection of tumor. [ABSTRACT FROM AUTHOR]

Pilocytic astrocytoma is a low-grade tumor that can affect the pineal region and has a potential life-threatening presentation with obstructive hydrocephalus. This video presents the case of a child who underwent an endoscopic third ventriculostomy with tumor biopsy and for whom a supracerebellar infratentorial approach was chosen after confirmation of the diagnosis of a pilocytic astrocytoma. Herein, we discuss the technical details of both procedures, such as the single burr hole in the neuroendoscopic approach, the ventricular landmarks and identification of the venous sinus through neuronavigation, the anatomical landmarks during the supracerebellar infratentorial approach, and the use of ultrasonographic aspiration.

Background: Rosette-forming glioneuronal tumor (RGNT) is an extremely rare entity described for the first time in the WHO classification of tumors of the central nervous system in 2007. Predominantly, single case reports of RGNT in the pineal region have been published, and specific therapy concepts are pending. Methods: The study group comprised all patients with the RGNT (CNS WHO grade 1) in the pineal region that underwent microsurgical tumor removal in our center (August 2018–June 2021). Surgical strategy, histological findings, and clinical outcome are presented, and the results are evaluated and compared to published case reports. Results: Four male patients aged under 50 years (range between 20 and 48 years) and one female patient, 51 years old, were included in this study. Chronic headaches and generalized epileptic seizures were the main symptoms. Supra-cerebellar infratentorial gross total tumor resection (GTR) was performed in two cases, two patients underwent subtotal tumor resection, and an endoscopic biopsy was performed in case five. Conclusion: In cases where surgical resection seems feasible with a reasonable surgical risk, we advocate GTR. Regular and long-term MRI follow-up is essential to detect a slow tumor progression. The role of additional chemotherapy or radiotherapy remains unclear.

Given the rarity of patients with pineal glioblastoma (GBM), clinical characteristics, treatment, and prognostic factors are not well characterized. This study aimed to investigate these characteristics and identify the prognostic factors of overall survival (OS). A retrospective analysis of newly diagnosed patients with pineal GBM, including our 3 cases and an additional 44 cases from published articles, was conducted. Survival analysis was performed by Kaplan-Meier analysis and Cox regression analysis was used to determine the prognostic factors. A total of 47 patients (28 males and 19 females) were enrolled, with a median age of 46 years (range, 5–74 years). Forty-four patients (90.9%) had preoperative obstructive hydrocephalus. Among 38 patients, 21 (55.3%) had distal leptomeningeal dissemination. Forty-five patients (95.7%) had resection/biopsy, 6 of whom had gross total resection, 22 had subtotal resection, 7 had partial resection, and 10 had biopsy. Adjuvant therapy included radiotherapy in 36 patients and chemotherapy in 27 patients. The median OS was 10.0 months. The 6-month, 1-year, and 2-year survival was 68.0%, 42.6%, and 17.0%, respectively. Cox regression analysis showed that patients receiving biopsy (P = 0.042) or chemotherapy (P = 0.029) had the better OS and these were regarded as independent prognostic factors. Further survival analysis showed that chemoradiotherapy had better survival benefit than other regimens. In this study, we summarized the characteristics of patients with pineal GBM and showed the correlation between clinical characteristics and prognosis. This study may give readers a deep understanding of these rare GBMs and provide some references for future management. [ABSTRACT FROM AUTHOR]

Purpose: Intra-axial "pineal region" tumors include pineal, tectal, and aqueductal tumors. All three tumor subgroups cause obstruction of the aqueduct; however, they differ in radiological nuances, pathology, differential diagnosis, and treatment. The goal of this manuscript is to describe the radiological, clinical, and pathological nuances that differentiate between these subgroups. Methods: All patients with intra-axial pineal region tumors were analyzed retrospectively, including demographics, radiological characteristics, pathology, treatment, and outcome. Results: Forty-nine patients (1–69 years of age) were included: 19 pineal, 10 tectal, 10 aqueductal, 4 periaqueductal, and 6 complex. The 3 main subgroups differed in various radiological and anatomical nuances. Age and gender did not differ between groups. Other factors that did not differ between groups included T1 and T2 signals, presence of blood products, a normally located (non-displaced) tectum, anterior tectal displacement, thalamic involvement, and presence of hydrocephalus. The pathological spectrum differed between the 3 main subgroups, as well as the surgical treatment, and outcome. Conclusions: Despite sharing a close anatomical location, as well as all causing obstruction of the aqueduct with secondary hydrocephalus, the differential diagnosis, diagnostic methods, and possible treatment and surgical options differ between the various subgroups. Anatomical nuances are described to better delineate the various tumor subgroups and recommend specific treatment approaches. [ABSTRACT FROM AUTHOR]

Introduction: The pineal region is a hard-to-reach part of the brain. There is no unequivocal opinion on the choice of a surgical approach to the pineal region. The surgical approaches described differ in both trajectory (infra- and supratentorial, interhemispheric) and size of craniotomy. They have advantages and disadvantages. The minimally invasive lateral occipital infracortical supra-/transtentorial (OICST) approach we have described has all the advantages of the standard supratentorial approach and minimizes its disadvantages, namely, compression and contusion of the occipital lobe. The minimally invasive craniotomy and small surgical corridor facilitate that., Methods: We describe 11 consecutive patients with various pineal region tumors (7 cases of pineal cysts, 2 cases of pinealocytoma, 1 case of medulloblastoma, and 1 case of meningioma) who were operated on in our hospital using the lateral OICST approach. Preoperative planning was performed using Surgical Theater®. The surgical corridor was formed using a retractor made from half of a syringe shortened according to the length of the surgical corridor. Preoperative lumbar drain was used., Results: The pineal region tumors were completely resected in all cases. The mean craniotomy size was 2.22 × 1.79 cm. No long-term neurological deficits were reported., Conclusions: The use of semicircular retractors and intraoperative CSF drainage via a lumbar drain allows to form a small surgical corridor to the pineal region via minimally invasive craniotomy. This reduces traction and traumatization of the occipital lobe, as well as minimizes intra- and postoperative risks., (Copyright © 2023 Elsevier B.V. All rights reserved.)

The supracerebellar infratentorial (SCIT) approach is frequently used for the treatment of lesions in the pineal and posterior third ventricle region and can be performed in a sitting or prone position. We describe our experience with the purely endoscopic SCIT approach, using a prone position, with neck extension and head rotation. We analysed all paediatric and adult patients operated on between July 2013 and January 2018 using the purely endoscopic SCIT approach. The surgical procedure was conducted through a paramedian subtorcular key-hole suboccipital craniotomy. The assistant held the endoscope and the surgeon used fine, long shaft instruments for bimanual tumour dissection. The prone position with head retroflexion allowed a gravity-aided enlargement of the anatomical surgical corridor between tentorium and cerebellum and enhanced venous blood drainage, similarly to the sitting or semi-sitting position, but with a decreased risk of air embolism. The approach allowed a complete removal of lesions located in the pineal region and in the posterior part of the third ventricle. Seven patients have been operated with this approach. All of them had the tumour completely removed. No permanent neurological deficits have been observed. The endoscopic SCIT approach enables effective and safe removal of pineal and posterior third ventricle lesions, even of moderate dimensions (up to 3 cm). The endoscope guarantees a detailed view and illumination of the deep-seated structures. The tumour resection can be performed with bimanual use of microsurgical instruments. [ABSTRACT FROM AUTHOR]

The paramedian supracerebellar craniotomy is an underrecognized route to the midline and paramedian regions of the upper posterior brainstem. As compared with its midline supracerebellar counterpart, this less disruptive approach preserves the majority of the midline bridging veins, requires less cerebellar retraction, and is significantly more efficient. In this offering, I will emphasize the realities of this flexible route and its remarkable advantages in reaching deep-seated lesions. [ABSTRACT FROM AUTHOR]

Background: Surgery on posterior cranial fossa (PCF) and pineal region (PR) carries the risks of intraoperative trauma to the brainstem structures, blood loss, venous air embolism (VAE), cardiovascular instability, and other complications. Success in surgery, among other factors, depends on selecting the optimal patient position. Our objective was to find associations between patient positioning, incidence of intraoperative complications, neurological recovery, and the extent of surgery.Methods: This observational study was conducted in two medical centers: The Ohio State University Wexner Medical Center (USA) and The Burdenko Neurosurgical Institute (Russian Federation). Patients were distributed in two groups based on the surgical position: sitting position (SP) or horizontal position (HP). The inclusion criteria were adult patients with space-occupying or vascular lesions requiring an open PCF or PR surgery. Perioperative variables were recorded and summarized using descriptive statistics. The post-treatment survival, functional outcome, and patient satisfaction were assessed at 3 months.Results: A total of 109 patients were included in the study: 53 in SP and 56 in HP. A higher proportion of patients in the HP patients had >300 mL intraoperative blood loss compared to the SP group (32 vs. 13%; p = 0.0250). Intraoperative VAE was diagnosed in 40% of SP patients vs. 0% in the HP group (p < 0.0001). However, trans-esophageal echocardiographic (TEE) monitoring was more common in the SP group. Intraoperative hypotension was documented in 28% of SP patients compared to 9% in HP group (p = 0.0126). A higher proportion of SP patients experienced a new neurological symptom compared to the HP group (49 vs. 29%; p = 0.0281). The extent of tumor resection, postoperative 3-months survival, functional outcome, and patient satisfaction were not different in the groups.Conclusions: The SP was associated with, less intraoperative bleeding, increased intraoperative hypotension, VAE, and postoperative neurological deficit. More HP patients experienced macroglossia and increased blood loss. At 3 months, there was no difference of parameters between the two groups.Clinical Trial Registration:ClinicalTrials.gov: registration number NCT03364283.

Background: Pleomorphic xanthoastrocytoma (PXA) is a rare and often focal glioma that most commonly affects children and young adults. Lesions are preferentially supratentorial and superficial, although infratentorial masses have been described, along with occasional involvement of the leptomeninges. The World Health Organization (WHO) categorizes these tumors as grade II, with surgical resection carrying a favorable prognosis. However, these tumors may undergo malignant degeneration and must be identified for appropriate treatment and prognosis. PXA has been associated with neurofibromatosis type 1 (NF1), although it is not the classic phenotype of NF1. We present a novel report of PXA, atypically located in the pineal region of a patient with a history of NF1. Case Report: A 17-year-old male with a history of NF1 presented with 1 month of bifrontal headaches. Magnetic resonance imagingwas significant for a heterogeneous tectal mass, suspicious for a glioma extending to the fourth ventricle and causing displacement of the cerebral aqueductwithout obstructive hydrocephalus. Following an infratentorial-supracerebellar approach for tumor resection, histopathology confirmed a low-grade variable neoplasm consistent with PXA. Postoperative imaging confirmed gross total resection with no evidence of recurrence at 9 months postoperatively. Conclusion: To our knowledge, this case is the fifth report of pineal PXA and the first associated with NF1. Because PXA presents similarly to other NF1-related intracranial tumors, careful diagnosis via immunohistochemistry is imperative. Gross tumor resection is usually curative; however, PXA has the propensity to undergo malignant degeneration and may require adjuvant treatment. [ABSTRACT FROM AUTHOR]