Tumors of the pineal region represent a rare and heterogeneous group of pediatric intracranial neoplasms, accounting for 3-8% of childhood brain tumors. Their deep midline location and proximity to critical neurovascular structures make surgical management particularly challenging. Over the past two decades, refinements in microsurgical and endoscopic techniques have markedly changed the surgical landscape. This systematic review aims to provide an updated overview of surgical strategies, outcomes, and complications in pediatric pineal region tumors. A systematic review was conducted following PRISMA guidelines. PubMed and Embase were searched for studies published between January 2000 and December 2025 reporting surgical outcomes in patients aged ≤ 18 years with pineal region tumors. Methodological quality was assessed using the Newcastle-Ottawa Scale. Twenty-two studies including 624 pediatric patients were analyzed qualitatively. Patient age ranged from 4 months to 18 years (mean 10 years), with a male-to-female ratio of 2.5:1. Germ cell tumors were the most common histology, followed by pineoblastomas, teratomas, and astrocytomas. The main surgical approaches included supracerebellar infratentorial (SCITA), occipital transtentorial (OTA), occipital interhemispheric transtentorial (OITA), and transcallosal routes, as well as endoscopic or endoscope-assisted techniques. Gross total resection (GTR) was achieved in 40-100% of cases. Transient postoperative morbidity ranged between 15-25%, with Parinaud's syndrome and ataxia being most frequent. Permanent deficits occurred in < 10% of patients, and overall mortality varied by histology and ranged from 0 to 20%. Both microsurgical and endoscopic approaches achieve high rates of GTR with excellent functional outcomes. Minimally invasive techniques increasingly complement traditional microsurgical routes, aiming to reduce morbidity and improve CSF management. Future multicentric prospective studies are essential to establish evidence-based guidelines for pediatric pineal tumor surgery.

Pineal region tumors are rare intracranial lesions accounting for less than one percent, with heterogeneous histopathology. This study aimed to provide a comprehensive overview of pineal region lesions, their clinical and radiological presentation, and their management and outcomes. This was a retrospective case series conducted at the Departments of Punjab Institute of Neurosciences. The study included all patients who underwent surgical excision or supportive procedures (cerebrospinal fluid [CSF] diversion and biopsy) for pineal region space-occupying lesions (SOLs) between January 1, 2022, and December 31, 2024. A non-probability consecutive sampling technique was used to enroll all the patients who fitted inclusion criteria, during the defined study period. A total of 32 patients were included in the final analysis. A total of 32 patients were reviewed, 68.8% (22) males and 31.3%(10) females with a mean age of 24.1±12.3 years. The most common presentation was headache in 87.5% (28). Radiologically, the lesion was hypodense in 81.3% (26) on computed tomography (CT). On magnetic resonance imaging (MRI), 34.4% (11) were hypointense on the T1 weighted images and 90.6% (29) were hyperintense on the T2 weighted images, and 53.1%(17) was homogenously enhanced on contrast. Among all, 40.6% (13) had surgical excision,46.9% (15) had only biopsy, and 6.3%(2) underwent cerebrospinal fluid (CSF) diversion surgery. On histopathology, 21.9% (7) were pineocytoma, 21.9% (7) were germinomas,12.5% (4) were pineoblastomas. Post-operative weakness occurred in 6.2% (2) and cerebrospinal fluid (CSF) leak occurred in 3.1%(1) patients. This study reinforces the clinical and surgical complexity of managing pineal region tumors, which present with symptoms of increased intracranial pressure. Continued integration of minimally invasive techniques, neuronavigation, and multidisciplinary care is essential for optimizing outcomes in this diverse tumor group.

Pineal parenchymal tumors are rare central nervous system neoplasms, accounting for less than 1 % of all CNS tumors. We conducted a bibliometric analysis of the 100 most cited articles on pineal parenchymal tumors to identify research trends and highlight influential contributions. Tumor types analyzed included pineocytoma, pineal parenchymal tumor of intermediate differentiation (PPTID), pineoblastoma, and papillary tumor of the pineal region (PTPR), along with the recently classified desmoplastic myxoid tumor, SMARCB1-mutant. To our knowledge, this is the first bibliometric analysis dedicated to pineal parenchymal tumors. Articles were identified through the Web of Science database without time restriction, screened by title, keyword, and abstract. Tumor categorization followed the WHO 5th edition for CNS tumors. Articles discussing non-parenchymal pineal region tumors were excluded. Citation count determined article selection. Data collected included publication year, authorship, journal source, study category, pathology focus, and article type. Data collection and analysis was performed using Microsoft Excel and Google Sheets. A total of 573 articles were retrieved; the top 100 were analyzed, totaling 3845 citations. These articles were published in 42 journals from 16 countries and 74 institutions. The oldest article was published in 1970 and the most recent in 2021. Acta Neuropathologica contributed the most publications (14). The United States accounted for the highest number of articles (34) and citations (1,326). Histopathology was the most studied category (36 %), and case reports and series were the predominant article types. The papillary tumor of the pineal region was the most frequently discussed pathology. This bibliometric analysis reveals a dominance of histopathological studies and reveals critical gaps in clinical studies, surgical management, and patient outcomes. Targeted future research in these areas is needed to improve diagnosis, management, and patient care.

Pineal parenchymal body tumors are rare central nervous system tumors with a variety of presentations ranging from well-differentiated low-grade tumors to undifferentiated highly aggressive tumors. Recent molecular classification has described this heterogeneity, particularly among pineoblastomas (PB) and pineal parenchymal tumor of intermediate differentiation (PPTID). Our study analyzed 49 patients with pineal parenchymal tumors, including PBs (n = 39), papillary tumors of the pineal region (n = 5), PPTID (n = 2), pineocytoma (n = 1), and trilateral retinoblastoma (n = 2). Descriptive analysis of patients’ characteristics was done in percentages and numbers. Overall survival (OS) and event-free survival (EFS) analysis were evaluated in relation to age and metastatic status for PB cases. Molecular classification was performed using the EPIC methylation array and analyzed by Heidelberg Classifier on 20 cases, of which sixteen were histopathologically diagnosed as PB. Among PBs, univariate analysis showed that age significantly impacted OS and EFS (p-value = 0.003 and 0.021, respectively), while metastatic status only impacted EFS (p-value = 0.032). In Multivariate analysis, only age was of significance on OS (p-value 0.028). The identified methylation groups were PB-miRNA-1 (n = 10), PB-RB1 (n = 1), retinoblastoma-MYCN activated (n = 1), PPTID KBTBD4-altered (n = 1), papillary tumor of the pineal region (n = 1), medulloblastoma (MB) WNT activated (n = 1), MB non-WNT/SHH (n = 1), CNS embryonal tumor with BRD4-LEUTX fusion (n = 1) and unclassified N/A (n = 3). Our data identified age as a prognostic factor affecting survival among our PB cohort. We also highlighted the heterogeneity of pineal parenchymal body tumors, necessitating molecular classification for accurate diagnosis and for developing tailored treatment strategies. We demonstrate the feasibility of identifying new entities and MBs within pineal body tumors, thereby supporting the growing evidence that MBs originate in the pineal region. Not applicable. The online version contains supplementary material available at 10.1186/s12885-025-15331-1.

Collision tumors are exceptionally rare neoplasms characterized by simultaneous presence of two histologically distinct primary brain tumors in the same anatomic location. Their composition can pose a significant therapeutic challenge. Herein, we present a case of a successfully resected pineal region collision tumor composed of a pineocytoma and a pilocytic astrocytoma (PA). A 19-year-old female was admitted with obstructive hydrocephalus due to a pineal gland cyst and underwent surgical excision. Histopathological analysis confirmed a collision tumor comprising pineocytoma and PA. Collision tumors composed of both pineocytoma and PA are extremely rare, with limited cases reported. Their histological architecture presents a diagnostic challenge, requiring meticulous evaluation even by experienced neuropathologists. Given the rarity of such cases, increased awareness of these tumors will facilitate the development of standardized diagnostic criteria and treatment protocols. Multidisciplinary collaboration is essential for optimizing therapeutic strategies tailored to the specific histological composition of collision tumors.