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Pineal region tumors account for less than 1% of adult supratentorial tumors. Their treatment requires a multimodality approach. Previously, the treatment of choice was direct surgery, which is associated with high surgical risk. Advances in minimally invasive techniques and onco-radiotherapy offer a safe and multimodal personalized therapy. The aim of our study was to describe the practice of our Institute based on combined endoscopic and radiotherapy techniques. We performed a retrospective clinical study. We processed data from 23 adult patients who underwent endoscopic third ventricle fenestration and pineal tumor biopsy between 2014 and 2023. Descriptive statistics, t-test, Fisher's exact test and Kaplan-Meier analysis were performed. Clinical improvement with endoscopic intervention was achieved in 78.3% of cases. Significant increase in preoperative performance status was observed in the postoperative period (p=2.755e-5), and radiotherapy resulted in regression or stable disease. Our results suggest a safe treatment with good clinical outcome and an excellent alternative to direct surgery.

Tumors of the pineal region (TPRs) are rare neoplasms that are surgically challenging to resect. Conventional treatment strategies are available, but gamma knife radiosurgery (GKRS) is an alternative approach. This study presents a single-center experience with GKRS performed for TPR with and without histopathological diagnoses. The cases of 25 patients with TPRs treated with GKRS were retrospectively analyzed. Thirteen of these 25 patients had histopathological confirmation, and 13 had elevated serum alpha-fetoprotein and beta-human chorionic gonadotropin levels. The 25 patients had a mean follow-up duration of 61 months. The total response rate to GKRS was 60%, and a 53.8% decrease in the alpha-fetoprotein and beta human chorionic gonadotropin levels was observed. The findings of this study indicate that GKRS is a safe procedure for TPRs, even in the event of insufficient histopathological findings. This treatment approach provides increased Karnofsky performance scores and an extended life expectancy., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)

Objective: Pineoblastomas (PBLs) are rare high-grade tumors treated variably with surgery and/or radiation. The role of surgical extent of resection and radiotherapy (RT) in adult PBL remains unclear. We queried the Surveillance, Epidemiology, and End Results (SEER) database to assess these variables' effects on overall survival (OS) in adult PBL., Methods: The SEER (1975-2016) database was queried for adult patients with diagnosis of PBL (ICD-0-3: 9362/3). Variables extracted included age, sex, race, geographical region, extent of tumor resection, RT, chemotherapy (CT), and OS data. Comparisons were performed with the χ 2 test for categorical variables, Cox proportional hazards models to assess the association of clinical variables on OS, and Kaplan-Meier curves were generated., Results: A total of 201 patients with PBL were identified with mean age 40.0 years (interquartile range 27.0-51.0) and most patients being male (53%) and Caucasian (77%). 101 (50%) patients received RT, and gross total resection was achieved in 83 (41%). Age stratification by decade revealed statistically significant poorer OS in patients aged ≥70 years. In bivariate analysis, RT with or without surgery was associated with improved 5-year OS compared with no RT (77.3% vs. 63.2%, P = 0.020). In multivariate analysis, age was a poor prognostic factor for OS (P < 0.001) and RT did appear to improve survival (P = 0.020). Extent of surgical resection was not significantly associated with improved survival., Conclusions: In adult patients with PBL, RT may offer an OS benefit irrespective of surgery or extent of surgical resection. Patients ≥70 years of age are associated with poorer OS., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Pineocytoma is a rare tumor. It is rare for pineocytoma to present as leptomeningeal metastasis. We present a rare case of pineocytoma with malignant transformation and leptomeningeal metastasis after subtotal tumor resection and adjuvant radiotherapy. This case was a 58-year-old male with an unsteady gait for 2 months. Enhanced brain magnetic resonance imaging revealed a heterogeneous mass involving the pineal region. The initial pathological diagnosis of pineocytoma was confirmed after subtotal tumor resection. Two years after adjuvant radiotherapy to the primary site, the magnetic resonance imaging showed C2 and T2 metastatic lesions, with the final pathological diagnosis being pineal parenchymal tumor (PPT) with intermediate differentiation after the removal of T2 intramedullary tumor. After that adjuvant radiotherapy at the cervical and thoracic spinal cord was completed. There was no recurrence of the tumor 1 year after the radiotherapy. We report a rare case of pineocytoma with malignant transformation to PPT with intermediate differentiation and leptomeningeal dissemination., Competing Interests: None

Background: The survival of pineoblastoma patients is low, particularly in infants and those with metastatic disease. This study aimed to analyze the prognostic factors affecting the outcome of Pineoblastoma in different age groups., Methods: A retrospective study included 33 patients. Twenty-two patients older than 3 years had upfront surgery, followed by induction CSI then 6 cycles of chemotherapy. Eleven patients younger than 3 years underwent surgery, followed by induction chemotherapy then radiation therapy. Focal irradiation (54 Gy) was administrated in six patients, and CSI (23.4 Gy) with booster dose 30.6 Gy to the tumor bed in two patients followed by 4 cycles of chemotherapy., Results: Patient's age showed a significant impact on the outcome (P value = 0.001 for EFS and 0.002 for OS). The metastases' presence did not impact the outcome negatively. The survival of patients with metastatic disease did not differ between age groups. However, age had a significant impact on the outcome of M0 disease, with 3-year EFS and OS of 65.3% and 74%, respectively, in the older group compared to 0% for both rates in younger patients. CSI showed a positive impact on survival. For all cases, the 3-year OS and EFS were 46.7% and 44.4%, respectively., Conclusions: A multimodality approach is needed to treat this aggressive disease. Inadequate dose intensity affected our patients' outcome negatively. A more aggressive approach using high-dose chemotherapy or CSI may be required to improve infantile pineoblastoma's dismal outcome. Focal radiotherapy is not an efficacious treatment in infants due to its high-metastatic potential. Molecular typing should be considered to label patients who need a more intensified approach., (© 2021. The Author(s).)

Little is known about pseudoprogression in brain tumours other than gliomas. A 9-year-old male child with a pineal teratoma/germinoma underwent surgical resection followed by adjuvant chemo-radiotherapy. The magnetic resonance imaging scan 4 months post-radiotherapy showed a contrast-enhancing lesion within the surgical cavity suspicious of recurrence. These radiological findings subsequently resolved without any specific intervention. The child continues in remission 2 years post-treatment. This case illustrates the occurrence of pseudoprogression post-radiotherapy in intracranial GCT and highlights an unmet need for greater implementation of functional imaging techniques in paediatric neuro-oncology to avoid undue discontinuation of effective treatments or inappropriate enrolment in clinical trials., (© 2020 The Authors. Pediatric Blood & Cancer published by Wiley Periodicals, Inc.)

Papillary tumor of the pineal region (PTPR) is a rare neuroepithelial brain tumor, characterized by a high risk of local recurrence (greater than 70 % at 6 years). The aim of our study was to review the available literature on radiotherapy for PTPR in order to evaluate timings, schedules, outcomes and toxicities of this treatment modality. In our review, 72.4 % (84) of the patients diagnosed with PTPR received radiation therapy. There is heterogeneity in the dose prescription, ranging from 45 Gy (25 × 1.8 Gy) to 60 Gy (30 × 2 Gy) for 3D Conformal Radiation Therapy and from 12 Gy to 36 Gy for Stereotactic Radiosurgery. Being considered as a grade II or III tumor, PTPR should receive higher total radiation dose in the adjuvant setting. Our analysis showed a very limited treatment-related toxicity with an expected 10-y OS of 72.5 %. At 5-years from the diagnosis, about 60 % of the patients experienced a local recurrence, whereas at 10 years the rate is higher than 80 %. In the literature, conflicting data about radiotherapy for PTPR are reported, in particular regarding disease progression. Although radiotherapy represents a fundamental treatment in the management of PTPR, prospective studies are required to better define its impact on overall survival and progression-free survival., Competing Interests: Declaration of Competing Interest All authors certify that they have no affiliations with or involvement in any organization or entity with any financial interest (such as honoraria; educational grants; participation in speakers' bureaus; membership, employment, consultancies, stock ownership, or other equity interest; and expert testimony or patent-licensing arrangements), or non-financial interest (such as personal or professional relationships, affiliations, knowledge or beliefs) in the subject matter or materials discussed in this manuscript., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Purpose: To assess the efficacy of upfront chemotherapy followed by response-adapted reduced-dose/reduced-volume radiotherapy (RT) for intracranial germinoma., Materials and Methods: Ninety-one patients from five institutions were registered in the KSPNO G051/G081 Protocol. Germinomas were classified as solitary or multiple/disseminated diseases, and upfront chemotherapy was administered. For all patients with multiple or disseminated disease, and patients with partial response after chemotherapy, 19.5-24 Gy of craniospinal irradiation plus 10.8-19.8 Gy of tumor bed boost were planned. For patients with complete response (CR), reduced-dose RT (30.6 Gy) was planned, along with a reduced field for solitary lesions., Results: The median patient age was 14 (range, 3-30) years. Sixty-five patients (71.4%) had a solitary lesion. The median follow-up duration was 67.9 (range, 6.6-119.3) months. Recurrence was not observed in 32 patients in the protocol compliant group. Four patients (4.4%) in the protocol non-compliant group experienced relapse after CR and one patient died of the disease. The 5-year and 7-year overall survival rates were 98.8% and 98.8%, while the corresponding event-free survival rates were 96.6% and 93.8%, respectively. All three patients with basal ganglia germinomas who were treated with local RT experienced recurrence outside the RT field. Among the 23 patients with pineal or suprasellar lesions who received whole-ventricle RT, there was no recurrence., Conclusions: Currently used upfront chemotherapy followed by reduced-dose, reduced-volume RT appears acceptable, when whole-ventricle RT for pineal or suprasellar tumors and, at minimum, whole-brain RT for basal ganglia/thalamus lesions are applied., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Background/aim: Pineoblastoma of the adult age is an uncommon tumor with only 200 cases reported. A standardized approach for an optimal adjuvant strategy is currently lacking. The case presented herein also deals with the issue of central nervous system tumors in pregnancy., Case Report: A 21-year-old pregnant woman presented with massive hydrocephalus due to a mass in the pineal region detected with MRI. After positioning an urgent ventricular derivation, a cesarean section was performed. During a third ventriculocisternostomy, a biopsy revealed a pineoblastoma. After a maximal safe resection, postoperative craniospinal irradiation for a total dose of 36 Gy plus a sequential boost to the tumor bed to 54 Gy, and adjuvant chemotherapy with CDDP plus CCNU plus vincristine were performed. After one year, the patient is alive with no evidence of disease., Conclusion: The use of adjuvant radio-chemotherapy provided excellent outcomes in our case. The advanced gestational age facilitated the choice of the therapeutic strategy., (Copyright© 2019, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.)

Pineal region tumors represent a heterogeneous group of different histologic entities, for which the management can be a significant challenge, due to their critical location and frequent aggressive behavior. Traditional management includes surgical resection, fractionated radiation therapy, and chemotherapy. Stereotactic radiosurgery (SRS) is being increasingly used in the treatment of these tumors. It is used as primary therapy for pineocytomas and papillary tumors of the pineal region, as an adjuvant radiation boost in combination with radiation or chemotherapy for pineoblastomas and germ cell tumors, or in the context of tumor recurrence. The reported morbidity is low, consisting in transient oculomotor disturbance in most cases. As a non-invasive alternative to microsurgical resection, SRS should always be considered when discussing these challenging cases., (© 2019 S. Karger AG, Basel.)

Objectives: Surgical excision of pineal parenchymal tumors(PPT), though desirable for good long term outcome, may not always be possible. Role of adjuvant radiotherapy (RT) in residual PPT's is not well delineated. The purpose of this single institutional retrospective study is to assess the role of radiotherapy in residual PPT's., Patients and Methods: Between 2006-2016, fourteen patients with residual PPT's were treated with adjuvant radiotherapy (12 with 3D conformal RT). Eight had pineocytomas (PC), 4 pineal parenchymal tumors with intermediate differentiation (PPID) and 2 pineoblastomas (PB). Doses of 45-54 Gy was delivered in 1.8-2 Gy per fraction. Cranio-spinal irradiation(CSI) was given to one case of PB and PPID each, with drop lesions at presentation. Patients were followed up at regular intervals., Results: The average Planning Target Volume (PTV) was 279.4cc. Clinico-radiological response was noted in all with complete disappearance of tumor in 4 patients with PC and 2 with PPID. Delayed recurrence (36-72 months later, median 42.5 months) was seen in 3 patients with PC and early recurrence in both with PB (median 28 months). Overall median follow-up for PC and PB was 54 and 31.5 months respectively. Two patients with PC and 2 with PB expired. The median follow-up for PPID was 21.5 months with no deaths or recurrences., Conclusions: Some PC and PPID may behave aggressively and recur or spread along neural axes requiring close follow up. Radiotherapy appears to be effective in patients with residual PC, PPID. Re-irradiation or radiosurgery may help in recurrences., (Copyright © 2018 Elsevier B.V. All rights reserved.)

The developing hippocampus is highly sensitive to chemotherapy and cranial radiation treatments for pediatric cancers, yet little is known about the effects that cancer treatents have on specific hippocampal subfields. Here, we examined hippocampal subfield volumes in 29 pediatric brain tumor survivors treated with cranial radiation and chemotherapy, and 30 healthy developing children and adolescents. We also examined associations between hippocampal subfield volumes and short-term verbal memory. Hippocampal subfields (Cornus Ammonis (CA) 1, CA2-3, dentate gyrus (DG)-CA4, stratum radiatum-lacunosum-moleculare, and subiculum) were segmented using the Multiple Automatically Generated Templates for Different Brains automated segmentation algorithm. Neuropsychological assessment of short-term verbal associative memory was performed in a subset of brain tumor survivors (N = 11) and typically developing children (N = 16), using the Children's Memory Scale or Wechsler's Memory Scale-third edition. Repeated measures analysis of variance showed that pediatric brain tumor survivors had significantly smaller DG-CA4, CA1, CA2-3, and stratum radiatum-lacunosum-moleculare volumes compared with typically developing children. Verbal memory performance was positively related to DG-CA4, CA1, and stratum radiatum-lacunosum-moleculare volumes in pediatric brain tumor survivors. Unlike the brain tumor survivors, there were no associations between subfield volumes and memory in typically developing children and adolescents. These data suggest that specific subfields of the hippocampus may be vulnerable to brain cancer treatments, and may contribute to impaired episodic memory following brain cancer treatment in childhood., (© 2017 Wiley Periodicals, Inc.)

Craniospinal irradiation is standard radiotherapy (RT) for localized intracranial nongerminoma germ cell tumors (NGGCT). Given its toxicity, there is interest in using smaller fields. We examined outcomes of NGGCT patients receiving reduced-volume RT at a single institution. Records of 16 patients who received reduced-volume RT as part of definitive treatment between 1996 and 2016 were reviewed. Median age at presentation was 10.8 years (range 4.6-41.0 years). Ten patients had pineal tumors and 6 had suprasellar tumors. All received chemotherapy and 9 patients received second-look surgery thereafter. RT volume was tumor-only to a median of 54 Gy (range 50.4-54 Gy) in 3 patients and whole-ventricle irradiation to a median of 30.6 Gy (range 30.6-36 Gy) with a boost to 54 Gy in 13 patients. Median follow-up was 4.1 years (range 1.9-19.3 years). Three patients recurred locally at a median 9.9 months (range 9.6-10.6 months) after diagnosis, and one of these developed leptomeningeal relapse after 30 months. One patient expired from disease 2.6 years post-diagnosis and another due to stroke 19.3 years post-diagnosis. Fourteen patients are alive with no evidence of disease. Kaplan-Meier estimates of the 4-year overall survival and failure-free survival are 92% (95% confidence interval [CI], 57-99%) and 81% (95% CI 53-94%), respectively. Excellent disease control was observed in these patients with no initial relapses outside of these RT fields. The results of ACNS1123 may better delineate patterns of failure and identify subgroups likely to benefit from this approach.

Background: Pineoblastoma is a rare pineal region brain tumor. Treatment strategies have reflected those for other malignant embryonal brain tumors., Patients and Methods: Original prospective treatment and outcome data from international trial groups were pooled. Cox regression models were developed considering treatment elements as time-dependent covariates., Results: Data on 135 patients with pineoblastoma aged 0.01-20.7 (median 4.9) years were analyzed. Median observation time was 7.3 years. Favorable prognostic factors were age ≥4 years (hazard ratio [HR] for progression-free survival [PFS] 0.270, P < .001) and administration of radiotherapy (HR for PFS 0.282, P < .001). Metastatic disease (HR for PFS 2.015, P = .006), but not postoperative residual tumor, was associated with unfavorable prognosis. In 57 patients <4 years old, 5-year PFS/overall survival (OS) were 11 ± 4%/12 ± 4%. Two patients survived after chemotherapy only, while 3 of 16 treated with craniospinal irradiation (CSI) with boost, and 3 of 5 treated with high-dose chemotherapy (HDCT) and local radiotherapy survived. In 78 patients aged ≥4 years, PFS/OS were 72 ± 7%/73 ± 7% for patients without metastases, and 50 ± 10%/55 ± 10% with metastases. Seventy-three patients received radiotherapy (48 conventionally fractionated CSI, median dose 35.0 [18.0-45.0] Gy, 19 hyperfractionated CSI, 6 local radiotherapy), with (n = 68) or without (n = 6) chemotherapy. The treatment sequence had no impact; application of HDCT had weak impact on survival in older patients., Conclusion: Survival is poor in young children treated without radiotherapy. In these patients, combination of HDCT and local radiotherapy may warrant further evaluation in the absence of more specific or targeted treatments. CSI combined with chemotherapy is effective for older non-metastatic patients., (© The Author(s) 2016. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com)

Background: Papillary tumor of the pineal region (PTPR) is a recently defined tumor entity. Its clinical course is characterized by frequent local recurrence, and patients may experience the burden of symptoms due to the anatomical location of the growing mass. Guidelines for treatment protocols, and the role of radiotherapy are still being investigated., Case: We report the case of a 27-year old woman who was referred to our department after she was diagnosed with PTPR and had undergone multiple surgical interventions. We delivered adjuvant conformal radiotherapy on the gross residual tumor to a total dose of 59.4 Gy (33 × 1.8 Gy)., Discussion: After a follow-up period of 41 months, we obtained a complete response to the treatment, according to the Response evaluation criteria in solid tumors criteria (RECIST). Radiation treatment was well tolerated, and the patient did not develop acute and late side effects. The neurological symptoms, which were documented at the diagnosis and after the surgical procedure, have not been recorded at last follow-up., Conclusions: Formal consensus for managing patients with a diagnosis of PTPR are nonexistent. Despite surgery, this tumor has a tendency to recur. Radiotherapy could have a role in the adjuvant setting and needs to be investigated in a multicenter setting with a long follow-up.Key words: radiotherapy - neurosurgery - magnetic resonance - pineal region - brain tumor.

Clinical Case: A 33-year-old male diagnosed with Parinaud's syndrome, exotropia and post-papillary oedema optic atrophy in his left eye. A pineal germinoma was diagnosed after performing neuroimaging scans and a stereotactic biopsy. He was treated with chemotherapy and radiotherapy, showing a complete pathological response. The Parinaud's syndrome persists one year after diagnosis and the patient has refused to have strabismus surgery., Discussion: Parinaud's syndrome consists of a supranuclear vertical gaze palsy resulting from damage to the midbrain tectum. The involvement of adjacent structures leads to the «Parinaud-plus» syndrome. When a Parinaud's syndrome is accompanied by diplopia («Parinaud-plus» syndrome), extension of the injury into adjacent areas must be considered., (Copyright © 2016 Sociedad Española de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.)

Aim: Pineal tumors represent uncommon intracranial tumors with highly diverse histologic subtypes. There still exists a controversy in literature about what influences overall survival and outcome., Material and Methods: We present the results of 48 patients with pineal tumor treated either by stereotactic biopsy followed by adjuvant therapy (23 patients) or open surgical resection without (18 patients) or with (7 patients) adjuvant therapy in Shohada Tajrish Hospital, Iran (1993-2008)., Results: Unremarkable pathology yield was 3/23 in the biopsy and 1/25 in the surgical group. Perioperative mortality and morbidity were 4.3% and 0% in the biopsy group and 32.0% and 4.0% in the surgical group. Analysis showed that age, gender, cranial nerve deficit, motor deficit, preoperative Karnofsky Performance Score (KPS), midbrain involvement, and brain stem involvement had no effect on neither perioperative mortality nor long-term survival, while local invasion and pineocytoma pathology increased perioperative mortality and presence of hydrocephalus and pineoblastoma pathology significantly decreased long-term survival. Hospitalization length was shorter in the stereotactic biopsy plus adjuvant therapy group., Conclusion: The results of the study suggests that although gross total resection is the standard of care in most pineal tumors nowadays, stereotactic biopsy followed by adjuvant therapy may still be a safe and viable option.

Background: Papillary tumor of the pineal region (PTPR) is a rare neuroectodermal tumor that was first described in 2003 and formally codified by the World Health Organization in 2007. Limited reports suggest surgical resection is the mainstay of treatment; however, the role of multimodality therapy is not well defined. We evaluated our institutional experience in the treatment of PTPR., Methods: A retrospective review of 8 patients with pathologically confirmed PTPR diagnosed between 1999 and 2013 was performed., Results: The median age at diagnosis was 37 years (range, 25-56 years). After a median follow-up period of 60 months (range, 10-170 months), 7 of 8 patients were still living. All patients underwent maximum safe surgical resection; 5 received adjuvant radiation (63%). Overall and progression-free survival 5 years after diagnosis were 100% and 51%, respectively. Progression-free survival 5 years after completion of adjuvant radiotherapy was 64%. Crude recurrence rates for patients receiving adjuvant radiotherapy (n = 5) and patients not receiving adjuvant radiotherapy (n = 3) were 20% and 67%, respectively. Crude recurrence rate after gross total resection (GTR) and no adjuvant radiotherapy (n = 2) was 100% versus 0% when adjuvant radiotherapy was administered after GTR (n = 2). After subtotal resection, 3 patients received adjuvant radiotherapy; 1 of these patients had out-of-field recurrence at 46 months (crude recurrence rate 33%). In all cases, salvage with radiation at the time of recurrence was effective., Conclusions: Our institutional experience confirms a recent multicenter retrospective series showing excellent survival but high risk of local recurrence for PTPR. Our findings suggest that radiotherapy provides durable local control, particularly when administered in the adjuvant setting after GTR., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Pineal tumor management in pediatric patients must be based on close co-operation between oncologists, surgeons, radiation oncologists, neurologists, ophthalmologists, and endocrinologists. Radiation therapy (RT) remains critical in most situations and should be assessed as soon as the diagnosis is made, in order to optimize the radiation technique. This paper will focus on RT modalities, indications, as well as modalities in main pediatric pineal tumors (germ cell tumors and pineal parenchyma tumors). RT modalities are presently being debated and new RT techniques (intensity-modulated RT, proton therapy etc.) that are now available for pineal lesions need to be evaluated. Radiation strategies are also controversial for germ cell tumors: cranio-spinal radiation versus chemotherapy followed by focal radiation, which also requires discussion., (Copyright © 2015. Published by Elsevier Masson SAS.)

We report a 44-year-old with progressive quadriparesis due to a dumbbell malignant peripheral nerve sheath tumour (MPNST) of the second cervical nerve, 17 years after whole brain radiotherapy for a pineal germinoma. To our knowledge this is the first case of radiation induced high cervical MPNST arising from a benign neurofibroma., (Crown Copyright © 2014. Published by Elsevier Ltd. All rights reserved.)

Purpose: The prognosis for children with central nervous system primitive neuroectodermal tumor (CNS-PNET) or pinealoblastoma is still unsatisfactory. Here we report the results of patients between 4 and 21 years of age with nonmetastatic CNS-PNET or pinealoblastoma diagnosed from January 2001 to December 2005 and treated in the prospective GPOH-trial P-HIT 2000-AB4., Methods and Materials: After surgery, children received hyperfractionated radiation therapy (36 Gy to the craniospinal axis, 68 Gy to the tumor region, and 72 Gy to any residual tumor, fractionated at 2 × 1 Gy per day 5 days per week) accompanied by weekly intravenous administration of vincristine and followed by 8 cycles of maintenance chemotherapy (lomustine, cisplatin, and vincristine)., Results: Twenty-six patients (15 with CNS-PNET; 11 with pinealoblastoma) were included. Median age at diagnosis was 11.5 years old (range, 4.0-20.7 years). Gross total tumor resection was achieved in 6 and partial resection in 16 patients (indistinct, 4 patients). Median follow-up of the 15 surviving patients was 7.0 years (range, 5.2-10.0 years). The combined response rate to postoperative therapy was 17 of 20 (85%). Eleven of 26 patients (42%; 7 of 15 with CNS-PNET; 4 of 11 with pinealoblastoma) showed tumor progression or relapse at a median time of 1.3 years (range, 0.5-1.9 years). Five-year progression-free and overall survival rates (± standard error [SE]) were each 58% (± 10%) for the entire cohort: CNS-PNET was 53% (± 13); pinealoblastoma was 64% (± 15%; P=.524 and P=.627, respectively)., Conclusions: Postoperative hyperfractionated radiation therapy with local dose escalation followed by maintenance chemotherapy was feasible without major acute toxicity. Survival rates are comparable to those of a few other recent studies but superior to those of most other series, including the previous trial, HIT 1991., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Radiation-induced cerebral aneurysms are rare. We describe a case of radiation-induced cerebral aneurysm successfully treated with endovascular coil embolization. A 39-year-old man received 60 Gy of radiation to a pineal germinoma at eight years old. The left internal carotid artery (ICA) aneurysm which developed within the irradiated field and stenotic change in the left ICA due to radiation-induced vasculopathy were detected incidentally. Because these aneurysms show a high risk of rupture and mortality, and even small aneurysms are prone to rupture, any such suspected aneurysm should be treated with surgical or endovascular procedures. Endovascular treatment is probably useful if the aneurysm is inaccessible to direct surgery. Special attention must be paid to treatment because of stenotic changes in cerebral vessels within the irradiated field.

Introduction: The usual endoscopic approach in the management of pineal region tumours consists of inserting the scope into the frontal horn of the lateral ventricle and advancing it through the foramen of Monro into the third ventricle. We report the case of a patient with a pineal tumour on whom we used an endoscopic approach through the ventricular atrium to obtain a biopsy by opening the choroidal fissure., Clinical Case: This young 25-year-old man presented with headache and double vision. Papilloedema and Parinaud's syndrome were found on physical examination. Cranial magnetic resonance revealed a pineal mass and hydrocephalus. We initially performed a third ventriculostomy and a tumour biopsy through a frontal burr hole. The tissue sample was not useful for pathological diagnosis and we decided to perform a second endoscopic biopsy., Conclusions: The endoscopic approach to pineal region masses, reaching the ventricular atrium through a parietal burr hole and opening the choroidal fissure, makes it possible to take a biopsy using a single endoscopic approach without needing to cross other ventricular structures., (Copyright © 2012 Sociedad Española de Neurocirugía. Published by Elsevier España. All rights reserved.)

Histiocytic sarcoma (HS) is a rare neoplasm that occurs most commonly in the intestinal tract, skin, soft tissue, and lymph node. The incidence of primary central nervous system (CNS) HS is even rarer, with a total of 6 cases reported in the literature. An etiologic link has not been identified for CNS HS, and the current case of primary CNS HS is unique in that an etiologic link to prior radiation therapy is identified, associated with complex cytogenetic abnormalities in the tumor. Although radiation-associated sarcomas can present as any number of different pathologic entities, this is the first reported case of a radiation-associated CNS HS. The pathologic and immunophenotypic characteristics of this case, with a nearly obscuring heavy inflammatory infiltrate and expression of monocytic/histiocytic markers (CD163, CD68, CD4, fascin), are characteristic of CNS HS. A discussion of the differential diagnosis and review of relevant literature are presented., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Pineocytomas are rare intracranial tumors occurring in the pineal gland region. The curative therapy of choice is gross total resection, which cannot be performed in all patients because of the frequent eloquent location of these tumors. Percutaneous fractionated radiotherapy is an alternative treatment approach that may result in high local control rates. Nevertheless, our knowledge of this tumor entity is limited and based on retrospective case series only. We present a patient with a papillary tumor of the pineal region who was treated with highly conformal proton-beam therapy at the Heidelberg Ion Therapy Center (HIT) using the raster-scanning technique.

We evaluated the efficacy of interstitial brachytherapy (IBT) using (125)Iodine ((125)I) seeds for treatment of papillary tumors of the pineal region. Between September 2003 and September 2010, four patients (M/F = 2/2; median age, 57.3 years; range 29.2-69.1 years) with papillary tumors of the pineal region underwent IBT using (125)I seeds. Before brachytherapy two patients underwent endoscopic ventriculo-cisternotomy, because of occlusive hydrocephalus, and subsequent microsurgical resection was performed on one; three patients were primarily treated with IBT. Median tumor volume was 3.3 ml (range 1.6-4 ml), the tumor surface dose ranged between 50 and 65 Gy. For three patients the seeds were implanted permanently whereas one patient received temporary implants (28 days). The median follow-up time was 53.6 months (range 13-103.4 months). After brachytherapy, follow-up MR images revealed complete remission in one patient, partial remission in two, and stable disease in the remaining patient. Neurological status improved in all patients (reduced headache and nausea/vomiting for four patients; improvement of oculomotor dysfunction for two of three patients partially and for one of three patients completely). Neither treatment-related morbidity nor mortality was observed. Our results are indicative of good local control of papillary tumors of the pineal region after IBT, without treatment-related morbidity.

Background: Adult pineoblastomas (PBL) are rare central nervous system tumors. Patient and treatment factors associated with outcome are poorly defined and limited to small retrospective case series and single case reports. Using the Surveillance, Epidemiology, and End Results (SEER) cancer registry, we investigated clinicopathological factors associated with outcome in adult PBL., Methods: Adult patients (≥16 years old) with PBL diagnosed between 1990 and 2007 were identified from the SEER database. Kaplan-Meier survival analysis and Cox models were used to examine the effect of variables on overall survival. The variables analyzed included patient's age at diagnosis, gender, race, tumor location, uni-focal or multi-focal tumor, tumor size, surgical resection, and the use of adjuvant radiotherapy., Results: Ninety-five patients were identified, with a median age at diagnosis of 39.2 years. Sixty-one patients (64 %) underwent surgery and 44 patients (47.4 %) received adjuvant radiotherapy. Forty-two patients (44 %) had both surgery and radiotherapy. The median overall survival was 176 months. Univariate analysis identified younger age at diagnosis, uni-focal and localized disease as important predictors of overall survival. On multivariate analysis, only age at diagnosis and localized disease emerged as important prognostic factors., Conclusions: Despite the numerous limitations of the SEER database, this study represents the largest analysis of adult PBL to date. Clinically relevant prognostic factors were younger age of diagnosis and localized disease. The role of surgery and adjuvant radiotherapy remains to be defined. Our data suggest these treatment modalities may not influence overall survival.

Papillary tumor of the pineal region (PTPR) is a rare neuroepithelial tumor that arises in the pineal region. The optimal treatment for PTPR remains controversial, as no definitive treatment strategy exists for this lesion. It is not clear whether aggressive surgical removal is superior to biopsy followed by radiotherapy. The majority of cases in the literature have undergone attempted gross total resection with a supracerebellar-infratentorial or a transcallosal-transventricular approach. In this report, we describe a case of PTPR in a 23 year-old male that presented as a third ventricular mass causing obstructive hydrocephalus. An endoscopic third ventriculostomy was performed followed by an endoscopic biopsy. Postoperative radiotherapy resulted in complete regression of the tumor with no evidence of tumor recurrence at 25 months. This case highlights a minimally invasive strategy for a rare neoplasm that resulted in a favorable response to radiation therapy, thereby avoiding the risks of aggressive surgical removal. We also review the radiographic and histopathologic features of PTPR and discuss various options of treatment reported in the literature.

Purpose: Bifocal germinoma (BFG) is a rare intracranial neoplasm for which the choice of radiation therapy (RT) field is controversial. Some believe that BFG represents disseminated disease requiring craniospinal irradiation (CSI), whereas others believe that BFG represents localized disease and advocate for more limited fields., Methods and Materials: We analyzed 20 BFG patients at our institutions with classic bifocal lesions (pineal gland and suprasellar region). In addition, we identified 60 BFG patients from the literature. The RT fields, use of chemotherapy and extent of disease were recorded and analyzed for each patient., Results: There were 55 patients with bifocal lesions only (Group I), and 25 with bifocal lesions plus ventricular and/or CSF positive disease (Group II). The 5-year progression-free survival was 95% for Group I and 80% for Group II. In Group I, there were no failures in patients receiving CSI (n = 11), two spinal failures in those treated with more limited RT fields without chemotherapy (n = 17), and one spinal failure with chemotherapy (n = 23). In Group II, there were no failures in patients receiving CSI (n = 11), but four spinal failures were observed in patients receiving more limited RT fields with chemotherapy (n = 13); 1 patient who received whole-brain RT without chemotherapy experienced failure in the spine and brain., Conclusions: CSI is associated with excellent PFS in BFG. In Group I BFG patients, omission of spinal irradiation appears to be a reasonable approach, especially when chemotherapy is used. Patients with Group II BFG are best treated with CSI., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Radiotherapy is extensively used in the treatment of malignant tumors of the central nervous system, but may also cause considerable morbidity and mortality, probably through damage to the remarkably vulnerable vascular system. We present two cases of infarction in the posterior cerebral vasculature related to earlier irradiation for a pineal gland tumor. Two patients were irradiated for a pineal gland tumor in young adulthood. Respectively 20 and 35 years later, they presented with a progressive neurological decline, related to early progressive atherosclerosis in the posterior cerebral vasculature. In conclusion, irradiation of the posterior cerebral territory may produce precocious atherosclerosis of posterior circulation vasculature, even decades later. When a progressive clinical decline with accumulating ischemic events is observed, together with signs of atherosclerosis, radiotherapy-related vasculopathy may be considered.

A 13-year-old patient presented with massive intra-abdominal metastasis and spontaneous acute tumour lysis syndrome, 17-months after VP shunt placement for metastatic pineal germinoma treated with cranio-spinal-irradiation. Hyperhydration/rasburicase improved renal function, allowing chemotherapy with subsequent surgery. The patient remains event-free 34-months later. Risk of intra-abdominal metastasis from VP shunts is discussed.

Pineocytoma is a rare tumor; therefore, assimilating data from case reports and small case series to generate definitive treatment guidelines is difficult. The authors recently systematically reviewed the existing literature on outcomes for patients with pineocytoma. Gross total resection is associated with significantly increased tumor control and survival compared with subtotal resection combined with radiotherapy. When gross total resection is not possible, adding radiotherapy to subtotal resection is not associated with increases in either tumor control or survival. Although aggressive surgery in the pineal region carries the risk of neurologic injury, gross total resection should be attempted for pineocytoma., (Copyright © 2011. Published by Elsevier Inc.)

The papillary tumor of the pineal region (PTPR) is a distinct entity that is particularly rare in the pediatric population. The authors document the youngest reported patient with this clinicopathological entity to date. A case of PTPR in a 15-month-old boy is described. Initially thought to be a tectal glioma, the tumor was later identified as a pineal region tumor after demonstrating growth on routine imaging. Diagnosis of PTPR was established by histopathological evaluation of biopsy samples, which revealed papillary, cystic, and solid tumor components. The patient's postoperative course was complicated by tumor growth despite several debulking procedures and chemotherapy, as well as persistent hydrocephalus requiring 2 endoscopic third ventriculostomies and eventual ventriculoperitoneal shunt placement. After a 15-month follow-up period, the patient has received proton-beam therapy and has a stable tumor size. The PTPR is a recently described tumor of the CNS that must be included in the differential diagnosis of pineal region masses. The biological behavior, prognosis, and appropriate treatment of PTPR have yet to be fully defined.

A 65-year-old male who had previously received curative treatment for a pineal tumor presented with an extremely rare case of primary central nervous system (CNS) primitive neuroectodermal tumor (PNET) of the spinal cord manifesting as progressive tetraparesis. Although the histology was not verified, highly radiosensitive tumor was suspected because of the benign clinical course for over 20 years after only radiation therapy. Magnetic resonance imaging demonstrated an intramedullary tumor extending from C5 to T1. He underwent partial resection and histological examination revealed blue tumor with undifferentiated small round cells. Immunohistochemically, c-kit was negative but CD99 was strongly and diffusely positive. Therefore, rearrangement of the Ewing sarcoma gene was examined to determine the presence of peripheral type of PNET. The results were negative and systemic workup revealed no other disease. These findings led to the diagnosis of primary intramedullary CNS PNET of the spinal cord, and suggested that the spinal cord tumor occurred independently of the prior pineal disease. The residual tumor was controlled by postoperative local radiation therapy.

Germ cell tumours (GCT) are a relatively common malignancy in men aged 15-35 years. They occur most frequently in the gonads, but 3-5% have extragonadal origin, mainly in the pineal gland, neurohypophysis, mediastinum and retroperitoneum. Although intracranial germinomas may present with synchronous midline lesions, development of metachronous testicular germ cell primaries seems to be extremely rare, and confirmed dissemination of intracranial GCT to the testes has never been reported. We report the case of a 32-year-old man, with previously treated pineal germinoma at age 16 years, who later developed mixed GCT of the left testis.

Background: To evaluate the effectivity of fractionated radiotherapy in adolescent and adult patients with pineal parenchymal tumors (PPT)., Methods: Between 1982 and 2003, 14 patients with PPTs were treated with fractionated radiotherapy. 4 patients had a pineocytoma (PC), one a PPT with intermediate differentiation (PPTID) and 9 patients a pineoblastoma (PB), 2 of which were recurrences. All patients underwent radiotherapy on the primary tumor site with a median total dose of 54 Gy. In 9 patients with primary PB treatment included whole brain irradiation (3 patients) or irradiation of the craniospinal axis (6 patients) with a median total dose of 35 Gy., Results: Median follow-up was 123 months in the PC patients and 109 months in the patients with primary PB. 7 patients were free from relapse at the end of follow-up. One PC patient died from spinal seeding. Among 5 PB patients treated with radiotherapy without chemotherapy, 3 developed local or spinal tumor recurrence. Both patients treated for PB recurrences died. The patient with PPTID is free of disease 7 years after radiotherapy., Conclusion: Local radiotherapy seems to be effective in patients with PC and some PPTIDs. Diagnosis and treatment of patients with more aggressive variants of PPTIDs as well as treatment of PB needs to be further improved, since local and spinal failure even despite craniospinal irradiation (CSI) is common. As PPT are very rare tumors, treatment within multi-institutional trials remains necessary.

A 17-year-old male was found to have a fourth cranial nerve palsy and chronic papilloedema following his presentation to our institution with a 6-week history of blurred vision in both eyes and vertical binocular diplopia. A diagnosis of pineal germinoma was made following imaging studies and endoscopic neurosurgical biopsy of the tumour. He was diagnosed with Attention Deficit Hyperactivity Disorder (ADHD) 3 years earlier and treated with amphetamines. Since this diagnosis, he continued to suffer from hyperactive behaviour, poor concentration, worsening headaches and insomnia. Pineal pathology has a known association with sleep disturbance through the disturbance of melatonin synthesis and/or metabolism. This case report serves to highlight how the presence of organic brain disease presenting to an eye department can be masked by a diagnosis of ADHD.

Purpose of Review: Pineal tumors are rare in children, with pineoblastoma and germ cell tumors (GCTs) being the most common. Here we discuss recent advances in treatment and controversies in the management of these tumors., Recent Findings: There is significant heterogeneity in the clinical behavior of pineoblastoma in children. We will discuss differences in outcome of children with pineoblastoma who are less than and greater than 3 years of age, and between pineoblastoma and nonpineal supratentorial primitive neuro-ectodermal tumors when treated with multiple different strategies. Significant controversies exist in the treatment of GCTs as well, including the levels of tumor markers in the blood and cerebrospinal fluid that are required to establish without biopsy the diagnosis of a GCT, the role of surgery in GCTs and the optimal treatment for germinomas as well as mixed malignant GCTs., Summary: Although pineoblastoma in infants and very young children still remains a therapeutic challenge, significant progress has been made in the treatment of pineal GCTs with treatment strategies using a combination of chemotherapy and reduced dose and volume irradiation, resulting in increased survival rates and reduced long-term morbidity.

Trilateral retinoblastoma (TRB) is a rare condition characterized by an intracranial neuroblastic tumor associated with bilateral or unilateral retinoblastoma (RB). The outcome is almost always fatal. An 18-month-old patient with familial bilateral RB was referred for a pineal lesion detected on a screening by magnetic resonance imaging. The child, considered inoperable by 2 different neurosurgical teams, was treated with conventional chemotherapy (methotrexate, vincristine, vepeside, cyclophosphamide, and carboplatin) plus tandem transplantation (vepeside/carboplatin and thiotepa/mephalan) followed by local radiotherapy. At 80 months from the diagnosis of TRB, the patient is alive and in complete remission, with no neuropsychologic consequences. An early and aggressive treatment may improve the prognosis of TRB.

Object: Pineocytoma is a rare tumor, and the current literature on these tumors is primarily composed of case reports and small case series. Thus, recommendations on appropriate treatment of these tumors are highly varied. Therefore, the authors performed a systematic review of the literature on tumor control after surgery for pineocytoma to determine the relative benefits of aggressive resection and postoperative adjuvant radiotherapy., Methods: A comprehensive search of the published English-language literature was performed to identify studies citing outcome data of patients undergoing surgery for pineocytoma. Determination of rates of progression-free survival (PFS) was performed using Kaplan-Meier analysis., Results: Sixty-four articles met the criteria of the established search protocol, which combined for a total of 166 patients. Twenty-one percent of these patients had undergone a biopsy procedure, 38% had undergone subtotal resection (STR), 42% had undergone gross-total resection, and 28% were treated with radiation therapy. The 1- and 5-year PFS rates for the resection group versus the biopsy group were 97 and 90% (1 year), and 89 and 75% (5 years), respectively (p < 0.05, log-rank test). The 1- and 5-year PFS rates for the GTR group versus the group undergoing STR combined with radiation therapy were 100 and 94% (1 year), and 100 and 84% (5 years), respectively (p < 0.05, log-rank test). There was no significant difference in PFS for STR only compared with STR in addition to radiation therapy., Conclusions: Gross-total resection is the ideal treatment for pineocytoma and might represent a cure for these lesions. When gross-total resection is not possible, adjuvant radiation therapy after STR is of questionable benefit for these patients.

The paper is the summary of mostly published works of the clinical results of intracavitary Yttrium-90 colloid irradiation of recurrent cystic craniopharyngiomas, and Iodine-125 interstitial irradiation of gliomas, pinealomas, brainstem tumors, recurrent meningeomas, solid craniopharyngiomas and metastases. It concisely demonstrates the usefulness of image fusion in the verification of isotope seeds and catheters, the comparison of 125 Iodine stereotactic brachytherapy and LINAC radiosurgery modalities on physical dose distribution and radiobiological efficacy, and the analysis of volumetric changes after interstitial irradiation of gliomas. Results of the immunohistochemical study deal with the role of microglia/macrophage system in the tissue response to 1-125 interstitial brachytherapy of cerebral gliomas. Due to financial reasons, gamma knife and Linac are not available to many countries and neurosurgical institutes. In the absence of the above mentioned radiosurgical methods, we have shown brachytherapy as an alternative solution in the treatment of different types of inoperable or recurrent brain tumors. The observed results may be noticable at LINAC and gamma knife irradiation too.

Purpose: To compare the sparing potential of cerebral hemispheres with intensity-modulated radiotherapy (IMRT) and three-dimensional conformal radiotherapy (3D-CRT) for whole-ventricular irradiation (WVI) and conventional whole-brain irradiation (WBI) in the management of localized central nervous system germ cell tumors (CNSGCTs)., Methods and Materials: Ten cases of patients with localized CNSGCTs and submitted to WVI by use of IMRT with or without a "boost" to the primary lesion were selected. For comparison purposes, similar treatment plans were produced by use of 3D-CRT (WVI with or without boost) and WBI (opposed lateral fields with or without boost), and cerebral hemisphere sparing was evaluated at dose levels ranging from 2 Gy to 40 Gy., Results: The median prescription dose for WVI was 30.6 Gy (range, 25.2-37.5 Gy), and that for the boost was 16.5 Gy (range, 0-23.4 Gy). Mean irradiated cerebral hemisphere volumes were lower for WVI with IMRT than for 3D-CRT and were lower for WVI with 3D-CRT than for WBI. Intensity-modulated radiotherapy was associated with the lowest irradiated volumes, with reductions of 7.5%, 12.2%, and 9.0% at dose levels of 20, 30, and 40 Gy, respectively, compared with 3D-CRT. Intensity-modulated radiotherapy provided statistically significant reductions of median irradiated volumes at all dose levels (p = 0.002 or less). However, estimated radiation doses to peripheral areas of the body were 1.9 times higher with IMRT than with 3D-CRT., Conclusions: Although IMRT is associated with increased radiation doses to peripheral areas of the body, its use can spare a significant amount of normal central nervous system tissue compared with 3D-CRT or WBI in the setting of CNSGCT treatment., (Copyright 2010 Elsevier Inc. All rights reserved.)

Introduction: Residual radiologically progressive masses following multimodality treatment of malignant mixed intracranial germ cell tumors are described. Often these enlarge, and this is called the growing teratoma syndrome. A similar phenomenon during radiotherapy alone has not been described., Subjects and Methods: A 5-year old boy presented with features of raised intracranial pressure. Imaging revealed a posterior third ventricular mass, which was biopsied endoscopically., Results: A review of the scanty tissue was suggestive of a pineal parenchymal tumor, and hence radiation was planned. After just ten fractions, he developed rapid neurological deterioration. Repeat imaging raised a possibility of a teratomatous tumor. He underwent emergency excision. However, he had a stormy postoperative course and succumbed to deep venous infarction. Histology revealed a purely mature teratoma., Conclusion: Though a growing teratoma syndrome has been described following chemotherapy, no such report while on radiation exists. Ours could be the first such reported case. We discuss the possible mechanisms with a review of the literature.