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Bilateral basal ganglia germinomas are exceptionally rare intracranial germ cell tumors associated with significant diagnostic delays and irreversible neurological impairment. This study addressed this diagnostic challenge by analyzing the largest dedicated cohort of histopathologically confirmed bilateral basal ganglia germinomas (n = 8). We identified alarming diagnostic delays (median, 1.2 years) in 62.5% of cases, culminating in irreversible deficits such as paralysis and cognitive impairment. Key early clinical indicators included adolescent predominance (mean age, 12.4 years), marked male predominance (7:1), cognitive decline (87.5%), and hemiparesis or monoparesis (62.5%). Magnetic resonance imaging detected cerebral atrophy in 87.5% of patients, with 25.0% showing concomitant mass lesions and atrophy, a distinctive radiological pattern not previously emphasized in unilateral basal ganglia germinomas. Cerebrospinal fluid beta-human chorionic gonadotropin analysis demonstrated superior sensitivity to serum testing (57.1% vs 28.6%). To mitigate devastating outcomes, we propose integrated cerebrospinal fluid beta-human chorionic gonadotropin screening combined with magnetic resonance imaging, with an emphasis on atrophy detection. In patients exhibiting progressive deficits or serial radiological progression despite negative biomarker results, stereotactic biopsy should be prioritized to enable early intervention and preserve neurological function.

This study investigated the effectiveness and safety of low-dose salvage craniospinal irradiation (CSI) for recurrent germinoma. We retrospectively reviewed long-term tumor control and late adverse effects in 15 recurrent germinoma patients treated at our hospital between 1983 and 2019. Following the first recurrence of germinoma, seven were treated with 24–30 Gy of salvage CSI, three underwent non-CSI, and five were treated with only chemotherapy. CSI achieved a significantly better recurrence-free survival rate after the first recurrence compared to other strategies (100% vs 33%, p < 0.001: log-rank test). To evaluate the safety of salvage CSI, we assessed the outcomes at the final follow-up of seven patients who received salvage CSI at first recurrence and three patients who received salvage CSI at second recurrence. The median follow-up period was 220 months after initial treatment. Five patients who received 40–50 Gy of radiation therapy or underwent multiple radiation therapy before salvage CSI were classified into Group A, whereas five patients treated with platinum-based chemotherapy and 24–32 Gy of radiation therapy to the primary site, whole ventricle, or whole brain were classified into Group B. In Group A, one had endocrine dysfunction and the other had visual dysfunction. None were socially independent. Meanwhile, in Group B, no endocrine or visual dysfunction was found, and three patients were socially independent. Salvage CSI achieved excellent tumor control in recurrent germinoma and was safe in patients initially treated with low-dose radiation therapy and chemotherapy.

CNS germinomas have an excellent prognosis with radiation therapy alone. However, in children, volume and dose of CNS radiation are associated with neurocognitive and neuroendocrine sequelae. Our objective was to determine long-term outcomes of our cohort who received chemotherapy and reduced radiation. This retrospective cohort study analyzed treatment and outcome of intracranial germinoma patients consecutively treated at Sick Kids, Toronto, Canada, from January 2000 to December 2013. 24 children (13 male, 11 female; median age 13.36 years) were identified. Median follow up was 61 months (range 1–144 months). Tumor location was suprasellar (n = 9), bifocal (8), pineal (6), and basal ganglia (1). Three children showed dissemination on imaging. 2/24 had only elevated serum human chorionic gonadotropin, 3/24 only elevated lumbar cerebrospinal fluid (CSF) hCG, and 2/24 had both elevated serum and lumbar CSF hCG. 23/24 children completed treatment and received multi-agent chemotherapy followed by either ventricular radiation (2340–2400 cGy) (n = 9), ventricular radiation + boost (1600 cGy) (n = 8), whole brain (2340 cGy) (n = 3), focal (4000 cGy) (n = 2) or craniospinal radiation (2340 cGy) (n = 1). Five-year progression free and overall survival was 96 and 100 % respectively. 8/24 patients with ventricular radiation ± boost (2340/4000 cGy) displayed stable full scale intelligence quotient over a mean interval of 3 years following radiation, but showed declined processing speed. In this limited experience, excellent 5-year overall survival rates were achieved with chemotherapy followed by reduced whole ventricular radiation even if ventricular radiation was delivered without boost.

Purpose This study identified the factors affecting cerebral microbleed (CMBs) development. Moreover, their effects on intelligence and memory and association with stroke in patients with germinoma who had long-term follow-up were evaluated. Methods This study included 64 patients with germinoma who were histologically and clinically diagnosed with and treated for germinoma. These patients were evaluated cross-sectionally, with a focus on CMBs on susceptibility-weighted magnetic resonance imaging (SWI), brain atrophy assessed through volumetric analysis, and intelligence and memory. Results The follow-up period was from 32 to 412 (median: 175.5) months. In total, 43 (67%) patients had 509 CMBs and 21 did not have CMBs. Moderate correlations were observed between the number of CMBs and time from initial treatments and recurrence was found to be a risk factor for CMB development. Increased temporal CMBs had a marginal effect on the processing speed and visual memory, whereas brain atrophy had a statistically significant effect on verbal, visual, and general memory and a marginal effect on processing speed. Before SWI acquisition and during the follow-up periods, eight strokes occurred in four patients. All of these patients had ≥ 15 CMBs on SWI before stroke onset. Meanwhile, 33 patients with < 14 CMBs or 21 patients without CMBs did not experience stroke. Conclusion Patients with a longer time from treatment initiation had a higher number of CMBs, and recurrence was a significant risk factor for CMB development. Furthermore, brain atrophy had a stronger effect on memory than CMBs. Increased CMBs predict the stroke onset.

Mature teratoma is a subtype of intracranial germ cell tumors (GCTs), distinguished from malignant GCTs by its benign nature and excellent prognosis. Typically, no adjuvant therapy is recommended following gross total resection (GTR). We report a case of a prepubertal girl with a suprasellar mature teratoma that recurred as a germinoma 6 months post-GTR. A 7-year-old girl presented with headache and polydipsia. Imaging revealed a suprasellar mass. The patient underwent GTR, and pathological diagnosis confirmed a mature teratoma without other GCT components. Six months later, MRI identified a newly developed suprasellar mass adjacent to the optic chiasm. A second surgery confirmed the mass as a germinoma. The patient subsequently underwent adjuvant chemotherapy combined with proton therapy, resulting in complete remission. The diagnosis of mature teratoma must be approached with caution, and thorough follow-up is imperative, particularly in cases involving female patients, prepubertal age, or non-pineal locations.

Introduction Intracranial germinoma has a favorable prognosis with modern therapies, but the long-term outcome of cerebrospinal fluid (CSF) diversion for its associated hydrocephalus has been rarely focused on. Purpose To evaluate the long-term success of CSF diversion methods—endoscopic third ventriculostomy (ETV) versus ventriculoperitoneal (VP) shunt—in intracranial germinoma patients. Methods Only pure intracranial germinomas with obstructive hydrocephalus and a minimum follow-up duration of 24 months were retrospectively reviewed. Their demographics, as well as pre-and postoperative data, were recorded. Patients were stratified into the ETV and the non-ETV groups and subsequently compared to determine the longevity and morbidity related to the procedures. Factors associated with the failure of CSF diversion were examined. Results From 1993 to 2022, eighty-three pathologically confirmed intracranial germinomas were identified. Excluding four cases of mixed pathology, eight with incomplete data, and two with insufficient follow-up, we enrolled 69 eligible patients for analysis. Among them, forty-three cases with obstructive hydrocephalus were classified into the ETV ( n  = 22) and non-ETV ( n  = 21) groups. No intraoperative or immediate postoperative complications occurred. With a median follow-up of 101 months (IQR 77.75–139.75), the ETV group had no failures. In the non-ETV cohort (median follow-up 144 months (IQR 97–210)), two VP shunt cases (9.5%) required revision due to blockage, and two patients (9.5%) experienced transient over-drainage. These 4 patients were without long-term difficulty despite short-term cumbersome events. No significant factors predicting CSF diversion failure were identified. To date, all 43 patients are alive without metastases, maintaining a good quality of life. Conclusion This study highlights ETV as a preferred CSF diversion method in pure intracranial germinoma, achieving 100% success without morbidity. Apart from simultaneous biopsy, avoiding a separate operation, this approach eliminates shunt-related complications, ensuring long-term quality of life in patients with extraordinary prognoses from modern chemo- and radiotherapy.

Central nervous system germ cell tumors (CNSGCTs) are rare neoplasms which usually develop in the midline structures. They are occasionally involved in off-midline structures of the brain. Here, we report an extremely rare case of an intracranial germinoma in the lateral ventricle. The patient was a 10-year-old boy with a 1-year history of polydipsia and polyuria. Brain magnetic resonance imaging (MRI) showed a relatively homogeneously enhancing lesion in the lateral ventricle, and the posterior pituitary gland was not hyperintense on T1-weighted imaging. Subependymoma was suspected, and tumor removal operation was performed; however, because the intraoperative pathological investigation revealed germinoma, we could only perform partial removal of the tumor. Postoperative histology also confirmed germinoma. Then, the patient received chemotherapy, followed by radiation therapy. MRI showed no recurrence for 6 years after treatment. Intracranial germinoma in the lateral ventricle is extremely rare. The diagnosis is occasionally challenging, especially when the tumors are located in atypical locations. This paper presents a literature review of previously described CNSGCTs of the lateral ventricle to improve awareness of CNSGCTs in atypical locations. We also consider the relationship between imaging findings and clinical manifestations.

 Abstract Intracranial germ cell tumors are relatively rare. They can affect the pineal region of the brain or the suprasellar area, or both simultaneously, and can cause various complications as well as endocrinological disorders. These tumors are characterized by their aggressive behavior and the tendency to metastasize. Intracranial germ cell tumors require an interdisciplinary approach in order to determine the most appropriate management. Radiotherapy and chemotherapy form the backbone of treatment strategies. Surgical methods are not the standard of care for this type of pathology, but they can provide histological confirmation in cases where tumor markers are not significant and can also be considered when seeking immediate symptomatic control. This study presents a juvenile case of a bifocal primary brain germinoma in which a liver metastasis was found after initial brain surgery and radiation therapy. The complex treatment continued with liver metastasectomy and chemotherapy, with the result of a complete therapeutic response and no recurrence after more than seven years of follow-up.

The purpose of this study was to investigate the value of C-11 methionine (MET) positron emission tomography (PET)/computed tomography (CT) in patients with intracranial germinoma (IG). We conducted a retrospective analysis of 21 consecutive patients with pathologically confirmed IGs and eight patients with intracranial non-germinomas (INGs) located in a similar region. Clinical characteristics, imaging findings, and tumor markers such as α-fetoprotein (AFP) and β-human chorionic gonadotropin (HCG) were used as clinical variables. Maximum standardized uptake value (SUVmax), tumor-to-normal tissue (T/N) ratio, and visual scoring of tumor were used as MET PET parameters. All IGs were well visualized on MET PET with a three-grade visual scoring system. In addition, SUVmax of IGs was higher than that of INGs (P = 0.005). Pre-treatment (Pre-Tx) T/N ratio was significantly correlated with pre-Tx serum HCG (P = 0.031). Moreover, MET PET parameters showed significant associations with tumor location, sex, KRAS variant, and symptoms. MET PET/CT could be a useful diagnostic tool in patients suspected of having IGs. In addition, the MET avidity of tumor is a potential surrogate biomarker of HCG, which has been used as a diagnostic marker for IGs. Tumor MET parameters also had significant differences according to tumor locations, sex, symptoms, and KRAS mutation. However, MET avidity of tumors had no significant prognostic value.

Pure germinoma typically originates from the midline and is usually found in the pineal and suprasellar regions in 76–90 % of cases. When it is in both regions, it is considered bifocal (10 % at diagnosis). If pure germinoma is located outside of the midline, it is considered ectopic, with a global incidence of about 0.7 %. The study aims to describe the clinical and surgical approach to patients with atypical intracranial ectopic germinoma (IEG) and bifocal germinoma (BG) through a literature review with the goal to delineate the correct diagnostic and therapeutic pathway, to reduce the diagnostic delay and improve the prognosis of these patients. A systematic review of the literature in most common electronic database (PubMed, Ovid MEDLINE and Ovid EMBASE) on IEG and BG, in according with the “PRISMA statement” criteria, from January 1990 to September 2022 was done. In addition, two rare cases of IEG and BG were reported. This systematic review included 16 papers (20 patients) with a final diagnosis of IEG and 30 papers (121 patients) with a final diagnosis of BG. IEGs seems to involve primary basal ganglia (40 %) and corpus callosum (40 %). For IEGs, biopsy (70 %, 14 cases out of 20) was the most common surgical approach: open approach (35 %), stereotactic minimally invasive approach (30 %) or endoscopic trans-sphenoidal approach (5 %). Partial resection was performed in 10 % of cases, whereas a total resection was performed in 20 % of cases. Also for BGs, biopsy was the most common surgical approach in 80 % of patients, whereas surgical resection (partial or total) was performed in 5.3 % of patients. IEG and BG are rare type of primary intracranial germ cell tumor, whose unusual location often can cause delays in diagnosis, which can have a significant impact on the patient's prognosis and requiring a multidisciplinary and timely approach. •Atypical ectopic germinoma is a rare type of primary intracranial germ cell tumor.•Ectopic germinoma for its unusual location often causes delays in diagnosis, which can significantly impact the patient's prognosis.•Bifocal intracranial germinoma is a more common form of germ cell tumor in the central nervous system.•Primary intracranial germinoma is a rare condition for which a multidisciplinary and timely approach is necessary.