Papillary tumor of the pineal region (PTPR) is a rare central nervous system neoplasm with an unpredictable clinical course. Gross total resection, the preferred treatment, is often unachievable due to the tumor's deep-seated location, necessitating alternative therapies. We report the case of a 24-year-old male with recurrent grade 3 PTPR. Initial subtotal resection followed by adjuvant volumetric modulated arc therapy (50.4 Gy in 28 fractions) failed to control tumor progression. After a second subtotal resection for recurrence, the patient underwent fractionated stereotactic radiotherapy (fSRT) using Gamma Knife Icon, delivering a total dose of 28 Gy in eight fractions. One year post-fSRT, significant tumor shrinkage was observed, and the patient maintained clinical stability. This case highlights fSRT's potential as an effective salvage treatment for recurrent PTPR, particularly in anatomically challenging regions where gross total resection is not feasible. To our knowledge, this is the first report detailing the successful use of fractionated Gamma Knife radiosurgery for a large, recurrent primary PTPR refractory to previous multimodal treatment, suggesting a valuable therapeutic option for this challenging condition.

Local recurrence of aggressive, pediatric brain tumors remains a challenge. We offered full-course re-irradiation with proton therapy (PT) to children with local failures in proximity to the brainstem. Eleven children (7 male, 4 female) underwent fractionated re-irradiation using pencil beam-based PT for local recurrence following maximum prior multidisciplinary treatment for ependymoma (N = 6), atypical teratoid rhabdoid tumor (N = 3), papillary tumor of the pineal region (N = 1) and atypical meningioma (N = 1).Median age at first radiotherapy (RT) was 4.5 years (range: 1.5-14.8); at re-RT was 6.7 years (range: 3.0-18.9); median interval between courses was 21 months (range: 13-160). In 9/11 children Gross Tumor was identifiable.Primary RT was with photons (N = 6) or protons (N = 5). Re-RT planning was based on cumulative dose summation. Permissible re-RT OAR doses were individualized. Median prescription dose was 54.0 Gy(RBE) (range 44.0-54.0). All re-treatments were delivered at 1.8-2.2 Gy(RBE) per fraction. A constant RBE value of 1.1 was used. At median follow-up of 42.5 months (range 7-63), 9 /11 children (82%) achieved local control. Actuarial 2- and 3-year local control and overall survival rates were 89% and 76%, and 82% and 73%, respectively.Strategies of balancing target dose with OAR considerations resulted in a median cumulative brainstem surface dose (D0.1cc) of 81.5 Gy(RBE) (range: 65.8-99.48). No CNS toxicities ≥ Grade 2 were observed, specifically no symptomatic brainstem or brain necrosis. Selective re-irradiation with pencil beam-based proton radiotherapy, using specifically modified treatment planning approaches, can be effective and safe, even in cases with tumor abutting the brainstem.

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.

Papillary tumors of the pineal region (PTPR) are rare brain tumors thought to originate from specialized ependymal cell remnants. Their clinical behavior varies, typically manifesting as symptoms of increased intracranial pressure. Due to their rarity, literature on PTPR is limited, complicating the development of evidence-based treatment guidelines. We present two illustrative cases describing their diagnostic workup, management and respective outcomes. This article presents two cases of PTPR, both diagnosed in adult females with a shared clinical presentation of hydrocephalus-associated symptoms. The first case, a 47-year-old patient, presented in October 2014 with progressive headaches, nausea, and vomiting. The second case, a 40-year-old patient, presented in October 2020 with a two-week history of diplopia, headache, and imbalance. Both patients underwent operative interventions, including biopsy and ventriculostomy, followed by tumor resection. Histological analysis confirmed PTPR in both cases, classified as WHO grade 2-3 and grade 3, respectively. The two cases also received radiation therapy, with case 1 demonstrating longstanding tumor control throughout follow-up, and case 2 experiencing aggressive metastatic activity in spite of repeated onco-surgical treatment. These cases highlight the complex management of PTPR as well as the significance of tailored interventions and long-term surveillance for improved outcomes. Surgical resection, in conjunction with adjuvant or salvage high dose radiation, remains of essence.