• Chemotherapy
• Intracranial germinoma
• Meta-analysis
• Radiotherapy
• Surgical resection

• Humans
• Germinoma/therapy
• Germinoma/pathology
• Brain Neoplasms/therapy
• Combined Modality Therapy
• Neoplasm Recurrence, Local/therapy
• Prognosis
• Treatment Outcome
• Craniospinal Irradiation/methods
• Cranial Irradiation/methods

• 82003231 National Natural Science Foundation of China

• endocrinologist
• intracranial germ cell tumors
• neuroendocrine function
• pediatric
• precocious puberty

• Child
• Adolescent
• Humans
• Brain Neoplasms/diagnosis
• Brain Neoplasms/pathology
• Brain Neoplasms/therapy
• Endocrinologists
• Neoplasm Recurrence, Local
• Neoplasms, Germ Cell and Embryonal/diagnosis
• Neoplasms, Germ Cell and Embryonal/therapy
• Neoplasms, Germ Cell and Embryonal/complications
• Diabetes Insipidus/complications

• Case report
• Germ cell tumor
• Immunotherapy
• Lymphadenectasis
• Testicular tumor

• clinical manifestation
• delayed diagnosis
• germ cell tumour
• intracranial germinoma

• case report
• intracranial germ cell tumor
• mature teratoma
• pathogenesis
• therapeutic strategy
• tumor recurrence

• children and adolescents
• hypothalamic dysfunction
• hypothalamic obesity
• metabolic diseases
• nonalcoholic fatty liver disease

• Child
• Humans
• Adolescent
• Retrospective Studies
• China/epidemiology
• Cholesterol

• Children
• Diencephalic syndrome
• Failure to thrive
• Germinoma
• Intracranial tumor

• Astrocytoma/surgery
• Brain Neoplasms/complications
• Brain Neoplasms/diagnostic imaging
• Brain Neoplasms/therapy
• Child
• Failure to Thrive/complications
• Female
• Germinoma/complications
• Germinoma/diagnostic imaging
• Germinoma/pathology
• Humans
• Pituitary Diseases
• Syndrome

Primary central nervous system germinomas of the medulla oblongata are extremely rare and usually have been found in young female Asian patients. The authors present an illustrative case of a patient who presented with severe medullary and posterior cord syndrome, the first South American case published to date, to the authors’ knowledge.

Initially, the radiological differential diagnosis did not include this entity. The lesion was located at the obex and exhibited a well-delineated contrast enhancement without hydrocephalus. An emergency decompressive partial resection following functional limits was performed. After histological confirmation, radiotherapy was indicated, with complete remission achieved at a 6-month follow-up. The patient, however, continued to have a severe proprioceptive disorder. The literature review identified 21 other such patients. The mean age for this location was 23 years, with a strong female and Asian origin predilection. All tumors exhibited contrast enhancement, and only one presented with hydrocephalus.

In the absence of elevated tumor markers, radiological clues such as a well-delineated, contrast-enhanced lesion arising from the obex, without hydrocephalus, associated with demographic features such as young age, female sex, and Asian heritage, should evoke a high level of suspicion for this diagnosis. Gross total resection must not be attempted, because this tumor is potentially curable with high-dose radiotherapy.

Intracranial germinoma (IG) rarely occurs in adults. Its optimal treatment strategy is unclear. We evaluated the outcomes of radiotherapy in adults with intracranial germinoma. Data of 29 adult patients (age, 18–52 years; median age, 24.3 years) with IG treated with radiotherapy at Taipei Veterans General Hospital were retrospectively reviewed. They were followed up for a median time of 5.9 years (range, 1.0–12.8 years). We used the Kaplan–Meier method to estimate the progression-free survival (PFS) and overall survival (OS), and univariate and multivariate Cox proportional hazards regression models to identify the factors affecting PFS. PFS and OS were compared between adult and pediatric patients with IG. The 1-, 3-, and 5-year PFS rates were 96.6%, 85.8%, and 77.8%, respectively, in the adult patients, and the OS rate were all 100%. Seven patients (24.1%) experienced recurrence, and in six of them, salvage therapy successfully controlled the disease. Two patients (6.9%) died after 5 years of follow-up due to disease progression and central pontine myelinolysis. In the univariate and multivariate Cox analysis, bifocal lesions had a significantly lower PFS than those with single lesions (p = 0.008). Kaplan–Meier survival analysis showed that adult patients had a poorer PFS (p = 0.06) and OS (p = 0.025) than pediatric patients. Our study showed bifocal lesions were associated with lower PFS than a single lesion among adult IG patients, and adult IG patients tended to have poorer PFS and OS compared to pediatric IG patients. For adult patients with bifocal IG, we recommend treatment with craniospinal irradiation, whole ventricle irradiation (WVI) with chemotherapy, or frequent spine images follow-up for patients who received only WVI.

• Adolescent
• Adult
• Brain Neoplasms/drug therapy
• Brain Neoplasms/radiotherapy
• Child
• Cranial Irradiation/adverse effects
• Disease-Free Survival
• Germinoma/radiotherapy
• Humans
• Kaplan-Meier Estimate
• Middle Aged
• Radiotherapy Dosage
• Retrospective Studies
• Treatment Outcome
• Young Adult

Little is known about depression and anxiety among paediatric intracranial germ cell tumour (iGCT) survivors. We aimed to evaluate the risk factors associated with depression, anxiety and health-related quality of life (HRQoL) in paediatric iGCT survivors.

We recruited 200 iGCT patients (and their parents) from Beijing Tiantan Hospital and assessed their HRQoL using the Paediatric Quality of Life Inventory (PedsQL) 4.0 Generic Core Scales. The Children’s Depression Inventory, Screen for Child Anxiety Related Emotional Disorders, and Symptom Checklist 90 were used to evaluate depression and anxiety. The results were analysed based on disease recurrence, tumour location and treatment strategies.

Survivors with recurrent tumours had worse HRQoL scores than those with non-recurrent tumours. Patients with tumours involving both the suprasellar and basal ganglia regions had the worst HRQoL scores. A large proportion of survivors had depression or anxiety. Both depression and anxiety scores were highly correlated with the HRQoL emotional functioning scores. The parent proxy-reports (PPR) and child self-reports were highly correlated in all domains.

This study demonstrated the clinical factors affecting paediatric iGCT survivors’ depression, anxiety, and HRQoL. Therefore, psychological interventions should be implemented. It also suggests that the PedsQL PPR would be helpful for routine screening.

Intracranial germ cell tumours (iGCT) are relatively rare brain tumours that predominantly occur in paediatric patients. Since the survival rate of patients with iGCT has been improving and the burden of long-term morbidity is substantial, promoting the health-related quality of life (HRQoL) and reducing depression and anxiety among its survivors is of critical concern. Although many studies have been published on factors related to the HRQoL of iGCT survivors, little is known about their long-term emotional function. This study demonstrated the clinical factors that affect depression, anxiety, and the HRQoL and the associations between the HRQoL and depression/anxiety in 200 paediatric iGCT patients. We found that patients with recurrent tumours had a worse HRQoL. Tumour locations also played a critical role in that tumour involving the basal ganglia were associated with worse outcomes. A large proportion of paediatric iGCT survivors had depression or anxiety in all the groups. Depression/anxiety scores were highly correlated with the HRQoL emotional functioning scores. In addition, we found that the PedsQL parent proxy report is appropriate in most cases when it is not possible to obtain child self-reports. These findings provide evidence to support the optimization of treatment strategies for iGCTs, such as the provision of psychological interventions.

• Anxiety
• Depression
• HRQoL
• Intracranial germ cell tumours
• Tumour survivors

• biopsy
• central nervous system
• diagnosis
• germinoma
• germinoma with elevated markers
• mixed germ cell tumor
• primary intracranial germ cell tumors

• Germinoma
• Growth height
• Hypothalamic-pituitary axis
• Radiation

• Germinoma
• Pineoblastoma
• Pineocytoma
• Tectal glioma
• Teratoma

Central nervous system germ cell tumors (CNS GCTs) are a heterogeneous group of brain tumors, which are more common in Asian countries. There have been different therapeutic strategies in treating germinoma and non-germinomatous germ cell tumors (NGGCT), depending on prognosis. Moreover, long-term follow up should be emphasized due to higher late complication rates. Here, we investigated long-term outcomes and complication profiles of 127 CNS GCT patients who received uniform upfront chemotherapy.

We retrospectively evaluated outcomes of CNS GCT patients treated in Seoul National University Children’s Hospital from August 2004 to April 2019. Patients were classified as low risk (LR) or high risk (HR) based on pathologic diagnosis and tumor markers. Most patients received upfront systemic chemotherapy with carboplatin, cyclophosphamide, etoposide, and/or bleomycin, followed by either proton or photon radiation therapy according to patients’ choice.

The median age at diagnosis was 11.9 (range, 3.8–25.1) years, and 54.3% of patients were LR. Photon and proton radiation therapy were administered to 73.2 and 25.2% of patients, respectively. In both LR and HR groups, there were no significant differences in survival between photon and proton radiation therapy. The 10-year relapse incidences were 9.3 and 5.6% in the LR and HR groups, respectively. All recurrences, except one, were local relapse. Six secondary malignancies occurred; the 10-year incidences of secondary malignancy were 2.2 and 7.6% in the LR and HR groups, respectively. The 10-year overall survival rates were 98.3 ± 1.7 and 91.8 ± 3.9% in the LR and HR groups, respectively. In a subgroup analysis of HR group, pathologically diagnosed NGGCT patients (n = 20) showed worse 10-year EFS (65.9 ± 11.9%, p < 0.001) and OS (77.9 ± 9.8%, p = 0.024) rates compared to other HR patients who were not pathologically diagnosed or were confirmed as germinoma with elevated tumor markers. All mortalities were related to disease progression or secondary malignancy.

The strategy of treating CNS GCTs with upfront chemotherapy according to risk groups resulted in good clinical outcomes and acceptable relapse incidence. However, further modification in the definition of the HR group is needed to reduce long-term complications.

• Central nervous system
• Germ cell tumor
• Intracranial
• Proton therapy
• Secondary malignant neoplasm

• bifocal germ cell tumor
• dual germ cell tumor
• germinoma
• intracranial germ cell tumor
• magnetic resonance imaging
• non-germinoma germ cell tumor
• overall survival
• progression free survival
• radiation
• suprasellar tumor

• Child
• Humans
• Brain Neoplasms/radiotherapy
• Central Nervous System
• Central Nervous System Neoplasms/prevention & control
• Germinoma/radiotherapy
• Retrospective Studies
• Treatment Outcome

• central diabetes insipidus
• craniopharyngioma
• germ cell tumor
• langerhans cell histiocytosis
• sellar and suprasellar region

• Adolescent
• Child
• Child, Preschool
• China
• Diabetes Insipidus, Neurogenic/complications
• Diabetes Insipidus, Neurogenic/pathology
• Diabetes Insipidus, Neurogenic/surgery
• Female
• Follow-Up Studies
• Humans
• Infant
• Infant, Newborn
• Male
• Pituitary Neoplasms/complications
• Pituitary Neoplasms/pathology
• Pituitary Neoplasms/surgery
• Prognosis
• Prospective Studies
• Sella Turcica/pathology
• Sella Turcica/surgery

• Brain tumor
• Children
• Complication
• Endoscopic endonasal surgery
• Outcome

• Adolescent
• Adult
• Child
• Combined Modality Therapy
• Female
• Germinoma/diagnosis
• Germinoma/metabolism
• Germinoma/radiotherapy
• Humans
• Magnetic Resonance Imaging
• Male
• Recurrence
• Treatment Outcome
• Tumor Burden
• Young Adult

• Ministry of Science, Korea