Pediatric Pineal Region Tumors: Special Reference to Posterior Interhemispheric Trans-Tentorial Approach.

Pediatric pineal region tumors consist of tumors of pineal gland origin and parapineal origin. The former are comprised of germ cell tumor (GCT) and pineal parenchymal tumor. The latter originate from the surrounding neural structures, such as the midbrain and thalamus; thus, they are often benign gliomas during childhood. Pineal region tumors often cause obstructive hydrocephalus, which is the main cause of presenting symptoms. Advanced imaging discloses precise location and extension of the tumor and associated anomalies such as hydrocephalous, dissemination, hemorrhage, etc. Hydrocephalus has been managed with CSF diversion, mostly using an endoscopic third ventriculostomy. Because of different treatment paradigms for each tumor type, histological confirmation is needed either through biopsy, tumor markers for GCTs, and/or surgical resection sampling. Radical resection of these tumors remains a challenge due to their deep-seated location and involvement of delicate neural and vascular structures. Comparison of common craniotomy approaches, occipital transtentorial (OT) and infratentorial supracerebellar (ITSC), is reviewed for their advantages and disadvantages. Surgical area exposure and blind spots are important factors for successful tumor removal. The surgical techniques and nuances that the author employs for tumor resection via a posterior interhemispheric transtentorial approach are presented.

Pediatric pineal region tumors: institutional experience of surgical managements with posterior interhemispheric transtentorial approach.

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.

Staged operations for a hypervascular mixed germ cell tumor with growing teratoma syndrome: a case report.

INTRODUCTION: A mixed germ cell tumor with a teratoma component can become enlarged following chemotherapy, and such an event is diagnosed as growing teratoma syndrome. Removing large, hypervascular tumors including a tumor encased by developed vasculatures from the pineal region is challenging during a single operation. CASE REPORT: A 15-year-old male underwent chemotherapy for mixed germ cell tumors according to the KSPNO G082 protocol. This case of a mixed germ cell tumor with growing teratoma syndrome was recognized very early during chemotherapy. The tumor was completely removed during the staged operations. First, the anteriorly located tumor on the third ventricle was removed via the transcallosal interforniceal approach, and 1 month later, the occipital transtentorial approach was used for the pineal tumor with decreased vascularity. CONCLUSION: Performing staged operations could be recommended for large hypervascular pineal tumors, which can be safely removed during the second operation once vascularity has decreased.

Pineal anlage tumor: a case report and review of the literature.

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.

Pineal region tumors: an entity with crucial anatomical nuances.

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.

A case of rapid deterioration in a subacute period after endoscopic third ventriculostomy.

Background: Although generally a safe procedure, serious postoperative complications after endoscopic third ventriculostomy (ETV) for obstructive hydrocephaly have been rarely reported, such as delayed obstruction of the stoma at the third ventricle floor.Case description: A 20-year-old male was referred to our department because of severe headache and diplopia. A pineal tumour and obstructive hydrocephaly were detected in preoperative imaging. After tumour biopsy and ETV, the reduction of ventricle size and improvement of headaches were immediately observed. On the seventh day, however, he developed a rapidly progressing consciousness disturbance due to severe hydrocephalus leading to urgent secondary ETV. The original ventriculostomy stoma at the third ventricle floor was completely occluded by scar adhesion. The patient recovered well as previously and received additional treatment.Conclusion: Although very rare, occlusion of the ventriculostomy stoma can postoperatively occur in the subacute period. Patients undergoing ETV for obstructive hydrocephalus due to a pineal tumour should be carefully monitored to avoid serious consequences.

Pineal region glioblastomas display features of diffuse midline and non-midline gliomas.

INTRODUCTION: Pineal region glioblastomas (GBM) are very rare, with approximately 46 cases described in the literature. The epidemiology, pathogenesis, and treatment of these lesions are poorly characterized. METHODS: We identified all cases of pineal region GBM treated surgically at our institution between 1990 and 2017. Demographic and clinical follow-up data were extracted from the medical records for all cases. Pathology was reviewed and classified according to 2016 World Health Organization (WHO) criteria. Specific attention was given to the frequency of histone H3 K27M mutations in these midline gliomas. RESULTS: Eight patients (seven men, one woman) with pineal region GBM, WHO grade IV, were identified. The most common presenting symptoms were headache (75%), vision changes (75%), and gait imbalance/ataxia (50%). Median age at diagnosis was 48.5 years (range 36-74 years). Radical subtotal resection, via a supracerebellar infratentorial approach, was achieved in 75% of patients. Review of the surgical pathology revealed seven primary GBMs (including one giant cell GBM) and one pineal region GBM that developed three years after resection of a pineal parenchymal tumor of intermediate differentiation. No cases demonstrated evidence of IDH-1 R132H mutation (N = 6) or 1p/19q co-deletion (N = 3). One case tested positive for the histone H3 K27M-mutation. Targeted exome sequencing of 467 cancer-related genes revealed nonsense mutations in ATRX and NF1. Adjuvant radiation and chemotherapy was employed in 87.5% and 75.0% of patients, respectively. Median overall survival (OS) was 15 months (range 2-24 months) from GBM diagnosis. CONCLUSIONS: This study expands the clinical and pathologic spectrum of pineal region GBM, and provides the first report of the genetic landscape of these tumors.

Extending the Neuroanatomic Territory of Diffuse Midline Glioma, K27M Mutant: Pineal Region Origin.

Diffuse midline glioma, H3-K27M mutant (DMG-K27M) is a newly described, molecularly distinct infiltrative glioma that almost exclusively arises in midline CNS structures, including the brain stem, especially the pons, as well as the thalamus and spinal cord with rare examples seen in the cerebellum, third ventricle, and hypothalamus. To our knowledge, only 1 case of a molecularly confirmed DMG-K27M arising in the pineal region has been previously reported. We present the second occurrence of a tissue-confirmed DMG-K27M of the pineal region, which, to our knowledge, is the first case reported in a child and the first case with documented preoperative MRI. This case, in addition to a prior report described in an adult, defines the lower end of a broad age range of DMG-K27M onset (12-65 years) and establishes the pineal gland as a bona fide site of origin for this newly codified midline glioma.

Retractorless surgery for a pineal region tumor through an occipital transtentorial approach.

This video demonstrates surgical techniques of the occipital transtentorial approach to a pineal region tumor without using a fixed brain retractor, which may cause functional impairment or even tissue injury to the occipital visual cortex. There are several ways to facilitate retractorless surgery through this approach. A lateral-semiprone positioning of the patient can induce gravity retraction. The brain is relaxed by draining CSF fluid through lumbar drainage or lateral ventricular tap in the case of obstructive hydrocephalus. Dynamic retraction with handheld instruments after extensive dissection of the deep venous system, including basal veins, can provide a sufficient working space. The video can be found here: https://youtu.be/kQvEHiNcRow .

Long-term outcomes in patients with pineal nongerminomatous malignant germ cell tumors treated by radical resection during initial treatment combined with adjuvant therapy.

BACKGROUND: For pineal nongerminomatous malignant germ cell tumors (NGMGCTs), we mainly performed radical tumor resection during initial treatment combined with adjuvant therapy. METHODS: We retrospectively analyzed 17 patients treated for pineal NGMGCTs between 1986 and 2007 at the University of Niigata. RESULTS: Twelve patients underwent total or subtotal resection of their tumor via the occipital transtentorial approach. Five patients underwent partial resection, and four of them later underwent total resection by salvage surgery. After surgery, eight patients were treated with combined radiochemotherapy including whole-brain irradiation, two received radiation monotherapy, one had chemotherapy with local irradiation, and six were treated with chemotherapy alone. The median follow-up period for surviving patients was 179 months. The 10-year overall survival and progression-free survival rates for the radiochemotherapy group were both 75.0 % (two patients had a recurrence and died); the rates for other adjuvant therapies were 77.8 % (two died) and 22.2 % (seven had a recurrence), respectively. Radiochemotherapy was significantly associated with an increased rate of progression-free survival compared with the other adjuvant therapies (p = 0.0396). CONCLUSIONS: For pineal NGMGCTs, initial treatment strategies including gross total resection of the tumor before or after whole-brain irradiation and chemotherapy provided good therapeutic outcomes. Obtaining complete remission of the primary tumor, irrespective of the timing of surgical resection (i.e., before or after adjuvant therapies), or complete response by neoadjuvant radiochemotherapy during an initial treatment appears to be essential for improving therapeutic outcomes of intracranial NGMGCTs.

Pineal Tumor Surgery-The Choice of the Approach Related to Tumor Characteristics and Posterior Fossa Anatomy.

OBJECTIVE: This research aimed to determine whether an adequate surgical approach can be chosen based on clearly defined values of anatomical landmarks (tentorial angle) and tumor size and extension. METHODS: We conducted a retrospective analysis of patients operated on because of pineal tumors. The cohort was divided depending on the surgical approach. On preoperative magnetic resonance imaging, we measured maximal diameters, tumor volume, and tumor propagation. In the group of patients operated with the supracerebellar infratentorial approach, we also tested the correlation of tentorial angle with residual tumor. Differences among groups in resection, complications rate, and outcome were tested by the χ2 test. Finally, in both groups, the correlation of residual tumor with tumor volume, propagation, and diameters was tested using the receiver operating characteristic curve. RESULTS: In the group operated with a supracerebellar approach, total resection was achieved in 78% of the patients. The critical value of cranio-caudal diameter correlated with tumor residue was 31 mm, for lateral-lateral diameter 25 mm, for the lateral extension 14 mm, and tumor volume 12 cm3. Tentorial angle did not influence the extent of the resection. In the group operated with an occipital transtentorial approach, the critical tumor volume related to tumor residue was 9 mm3, anterior-posterior diameter 29 mm, and cranio-caudal diameter 28 mm. The extent of the resection was significantly higher in the supracerebellar group. CONCLUSIONS: In both approaches, tumors larger than 3 cm show an increased risk of subtotal resection. Except when most tumor volume is localized above the venous system, we advocate a supracerebellar corridor as an effective approach that is not limited by tentorial angle.

Good clinical outcomes and the necessity of CSF drainage in patients undergoing simultaneous biopsy and endoscopic third ventriculostomy in the region of pineal tumors: A systematic review and meta-analysis.

INTRODUCTION: Due to their delicate and deep-seated location, tumors in the pineal region of the brain pose exceptional challenges in neurosurgical management. Highly precise procedures have become crucial to address these complexities, such as the simultaneous performance of biopsy and endoscopic third ventriculostomy (ETV). Our aim was to assess the feasibility, safety, and efficacy of simultaneous biopsy and ETV for treating patients with pineal region tumors. METHODS: Medline, Embase, and Web of Science were searched for English studies from January 2000 to February 2024, following Cochrane and PRISMA guidelines. Eligible studies encompassed a minimum of four patients and examined at least one of the following outcomes: good clinical outcomes and the necessity of shunt placement. Single proportion analysis with 95% confidence intervals was conducted under a random-effects model, employing the I2 statistic to assess heterogeneity. Additionally, publication bias was evaluated using the ROBINS-I tool. RESULTS: After a meticulous selection process, eighteen studies involving 390 patients were included in the analysis. Overall, good clinical outcomes were observed in 131 out of 147 patients, representing a rate of 92 % (95 % CI: 84 % to 100 %, I2 = 62 %) through random effects analysis. Subgroup analysis showed that children exhibited a notably high rate of good clinical outcomes, reaching 100 % (95 % CI: 96 % to 100 %, I2 = 0 %). Regarding the need for shunt placement, out of the 356 patients assessed, only 39 required shunt placement, yielding a rate of 8 % (95 % CI: 4 % to 12 %, I2 = 63 %). Further sub-analyses indicated shunt requirement rates of 12 % for children and 3 % for adults. Specifically focusing on adults, data from 46 patients who underwent biopsy revealed a success rate of 84 % (95 % CI: 62 % to 100 %, I2 = 81 %). Remarkably, no major complications were reported among adults, resulting in a rate of 0 % (95 % CI: 0 % to 6 %, I2 = 0 %). Additionally, low rates of mortality related to the procedure were observed in adults, with two deaths recorded among the 46 patients analyzed, resulting in a mortality rate of 1 % (95 % CI: 0 % to 7 %, I2 = 0 %). CONCLUSION: In conclusion, our study aimed to assess the feasibility, safety, and efficacy of performing simultaneous biopsy and ETV for patients with pineal region tumors. We meticulously examined clinical aspects and patient outcomes, including good clinical outcomes, the requirement for shunt placement after ETV, biopsy success rates, mortality, and complications.

Occipital-interhemispheric transtentorial pineal mass resection.

The occipital approach for pineal tumors was first described by James Poppen in 1966. Since then, it has been widely used for accessing deep-seated tumors as it offers a wider surgical view than the supracerebellar transtentorial approach. This video demonstrates the technical nuances of the occipital transtentorial approach and the exoscopic dissection of a pineal gland tumor in a 66-year-old male. Use of the exoscope over the microscope provides certain ergonomic advantages and improves surgical workflow, as demonstrated here. The video can be found here: https://stream.cadmore.media/r10.3171/2023.10.FOCVID23161.

Pineal parenchymal tumor of intermediate differentiation with late spinal dissemination 13 years after initial surgery: illustrative case.

BACKGROUND: Pineal parenchymal tumors of intermediate differentiation (PPTIDs) are rare in the pineal gland. A case of PPTID that disseminated to the lumbosacral spine 13 years after the total resection of a primary intracranial tumor has been reported. OBSERVATIONS: A 14-year-old female presented with headache and diplopia. Magnetic resonance imaging revealed a pineal tumor that induced obstructive hydrocephalus. A biopsy and endoscopic third ventriculostomy were performed. Histological diagnosis revealed a grade II PPTID. Two months later, the tumor was removed via craniotomy because the postoperative Gamma Knife surgery was ineffective. Histological diagnosis confirmed PPTID, although the grade was revised from II to III. Postoperative adjuvant therapy was not performed, because the lesion had been irradiated and gross total tumor removal was achieved. She has had no recurrence in 13 years. However, pain around the anus newly appeared. Magnetic resonance imaging of the spine revealed a solid lesion in the lumbosacral spine. The lesion was subtotally resected, and histological diagnosis revealed grade III PPTID. Postoperative radiotherapy was performed, and she had no recurrence 1 year after radiotherapy. LESSONS: Remote dissemination of PPTID can occur several years after the initial resection. Regular follow-up imaging, including the spinal region, should be encouraged.

Pineal Parenchymal Tumors With Intermediate Differentiation to Pineoblastoma: A Transitional Neuroectodermal Tumor of the Pineal Gland.

Pineal tumors are quite rare and are fairly aggressive tumors seen in young adults and children. These tumors arise from the pineal region or recess from various types of cells in the gland and structures located in close propinquity to the gland. Pineal gland tumors have a heterogeneous spectrum that includes pineal parenchymal tumors (PPTs) and papillary tumors of the pineal region (PTPR). The PPTs are further subclassified into pineocytomas (Grade 1), PPTs of intermediate differentiation (grade 2 or 3), and pineoblastomas (grade 4) based on the World Health Organization (WHO) grades and histopathological features. We discuss the case of an 11-year-old male child who presented with complaints of headache for 15 days, vomiting for seven days, and diplopia for four days. On magnetic resonance imaging (MRI), a soft tissue density lesion was noticed in the posterior third ventricle region. Based on the location and the MRI findings, the differential diagnosis considered were a pineal lesion, a choroid plexus papilloma, or a meningioma. He underwent a right occipital ventriculoperitoneal shunt followed by total excision of the tumor, and the resected specimen was sent for histopathological examination. After pathologic examination, the diagnosis of pineoblastoma (grade 4) with features of a PPT of intermediate differentiation (grades 2-3) was revealed, and the same was confirmed on immunohistochemistry.

Outcomes and surgical approaches for pineal region tumors in children: 30 years' experience.

OBJECTIVE: Pineal region tumors account for 2.7%-11% of all CNS tumors in children. In this series, the authors present their surgical results and long-term outcomes from a pediatric pineal region tumor cohort. METHODS: A total of 151 children aged 0-18 years were treated from 1991 to 2020. Tumor markers were collected in all patients; if positive, chemotherapy was performed, and if negative, biopsy was performed, preferably endoscopically. Resection was performed when there was a residual germ cell tumor (GCT) lesion after chemotherapy. RESULTS: The distribution based on histological type, as verified by markers, biopsy, or surgery, was germinoma (33.1%), nongerminomatous GCT (NGGCT) (27.2%), pineoblastoma (22.5%), glioma (12.6%), and embryonal tumor (atypical teratoid rhabdoid tumor) (3.3%). A total of 97 patients underwent resection, and gross-total resection (GTR) was achieved in 64%; the highest GTR rate (76.6%) was found in patients with GCTs, and the lowest (30.8%) was found in those with gliomas. The supracerebellar infratentorial approach (SCITA) was the most common, performed in 53.6% of patients, followed by the occipital transtentorial approach (OTA), performed in 24.7% of patients. Lesions were biopsied in 70 patients, and the diagnostic accuracy was 91.4. The overall survival (OS) rates at 12, 24, and 60 months as stratified by histological type were 93.7%, 93.7%, and 88% for patients with germinomas; 84.5%, 63.5%, and 40.7% for patients with pineoblastomas; 89.4%, 80.8%, and 67.2% for patients with NGGCTs; 89.4%, 78.2%, and 72.6% for patients with gliomas; and 40%, 20%, and 0% for patients with embryonal tumors, respectively (p < 0001). The OS at 60 months was significantly higher in the group with GTR (69.7%) than in the group with subtotal resection (40.8%) (p = 0.04). The 5-year progression-free survival was 77% for patients with germinomas, 72.6% for patients with gliomas, 50.8% for patients with NGGCTs, and 38.9% for patients with pineoblastomas. CONCLUSIONS: The efficacy of resection varies by histological type, and complete resection is associated with higher OS rates. Endoscopic biopsy is the method of choice for patients presenting with negative tumor markers and hydrocephalus. For tumors restricted to the midline and with extension to the third ventricle, a SCITA is preferred, whereas for lesions with extension toward the fourth ventricle, an OTA is preferred.

Machine learning-based classification of pineal germinoma from magnetic resonance imaging.

Introduction: Surgical approaches for tissue diagnosis of pineal tumors have been associated with morbidity and mortality. The classification of images by machine learning (ML) may assist physicians in determining the extent of resection and treatment plans for a specific patient. Therefore, the present study aimed to evaluate the diagnostic performances of the ML-based models for distinguishing between pure and non-germinoma of the pineal area. In addition, the secondary objective was to compare diagnostic performances among feature extraction methods. Methods: This is a retrospective cohort study of patients diagnosed with pineal tumors. We used the RGB feature extraction, histogram of oriented gradients (HOG), and local binary pattern methods from magnetic resonance imaging (MRI) scans; therefore, we trained an ML model from various algorithms to classify pineal germinoma. Diagnostic performances were calculated from a test dataset with several diagnostic indices. Results: MRI scans from 38 patients with pineal tumors were collected and extracted features. As a result, the k-nearest neighbors (KNN) algorithm with HOG had the highest sensitivity of 0.81 (95% CI 0.78-0.84), while the random forest (RF) algorithm with HOG had the highest sensitivity of 0.82 (95% CI 0.79-0.85). Moreover, the KNN model with HOG had the highest AUC, at 0.845. Additionally, the AUCs of the artificial neural network and RF algorithms with HOG were 0.770 and 0.713, respectively. Conclusions: The classification of images using ML is a viable way for developing a diagnostic tool to differentiate between germinoma and non-germinoma that will aid neurosurgeons in treatment planning in the future.

Surgical Resection of a Third Ventricle Nongerminomatous Germ Cell Tumor: Two-Dimensional Operative Video.

Surgical resection of a pineal tumor growing into the third ventricle is difficult owing to the complex neurovascular structures, and nongerminomatous germ cell tumor is the most common malignant tumor in pediatric patients. Removing the tumor efficiently with minimal blood loss while protecting the surrounding neurovascular structure is challenging. We present a surgical case of a 9-year-old patient with a third ventricle nongerminomatous germ cell tumor (Video). Mass effect of the tumor or acute hydrocephalus is the possible reason for the coma. In this case, the reason of coma may be mass effect of the tumor, not the acute hydrocephalus. Informed consent was obtained from the patient's guardian. Intraoperatively we used a modified right head-up park bench position and a linear incision. The right occipital bone flap was designed to cross the superior sagittal sinus and transverse sinus. The primary surgical approach was the occipital transtentorial approach; an alternative was the supracerebellar infratentorial approach. After cutting the tentorium, a spatula was applied to retract the cerebellum and incised tentorium, with no extra brain retraction on the occipital lobe to minimize visual disturbance. The quadrigeminal cistern was opened, and the tumor was yellowish with heterogeneous consistency. Instead of rushing into the tumor debulking, we paid more attention to devascularization of the tumor from bilateral posterior medial choroidal arteries as much as possible. After debulking using an ultrasound aspirator, the tumor was removed in a piecemeal fashion, and the surgical field was inspected using an endoscope for any residue.

Radiotherapy After Endoscopic Biopsy in an Adult with Pineocytoma, the Rare Brain Tumor in an Adult: A Case Report and Literature Review.

Tumors in the pineal region usually present with acute hydrocephalus. Histologic diagnosis is most important, as it greatly influences treatment, because over 17 tumor types occur in this area. Biopsies of these lesions play an important role in further management. Pineocytomas are benign and rare tumors that typically exhibit a slow progression. However, the appropriate treatment for pineocytoma varies. Surgical excision was considered for good long-term outcomes; however, this may not always be possible. Radiotherapy also appears to be effective in patients with residual pineocytomas. We report a case of pineocytoma with hemorrhagic transformation and complicated hydrocephalus. The patient refused to undergo aggressive tumor excision. Thus, the patient only underwent endoscopic biopsy and external ventricular drain (EVD) implantation, but the outcome was acceptable. In addition, we reviewed the current management strategies for pineocytomas in the literature.

Papillary Tumor of the Pineal Region Rare Pediatric CNS Tumor Case Series Treated in King Fahad Medical City (KFMC).

The clinical behaviors, prognosis, and appropriate treatments of papillary tumors of the pineal region (PTPR) are not fully defined due to the rarity of these tumors. At diagnosis, PTPR may present with clinical symptoms, including headache with obstructive hydrocephalus, diplopia, vomiting, and lethargy, as well as neurological signs, including Argyll Robertson pupils and Parinaud's syndrome due to compression of the dorsal midbrain, specifically the periaqueductal region with horizontal nystagmus. Radiological assessment of pineal region lesions is challenging, with a wide range of potential differential diagnoses. PTPR typically presents as a heterogeneous, well-circumscribed mass in the pineal region, which might contain cystic areas, calcifications, hemorrhages, or protein accumulations. Here, we report three female pediatric patients with PTPR treated in King Fahad Medical City (KFMC) in Saudi Arabia. Histological and immunohistochemical diagnosis was confirmed by analysis of genome-wide DNA methylation profiles. This case series expands on the available reports on the clinical presentations of PTPR and provides important information on the responses to different treatment modalities.