Primary brain tumors are the most frequent solid pediatric tumors, accounting for 40-50% of all cancers in children. Eighty to ninety percent of the 250,000 new cases of pediatric cancer each year are discovered in low and middle-income nations, where nearly 88 percent of the world's children reside.
Das et al. performed a retrospective study of patients who were admitted to the neurosurgery department with unusual pediatric brain tumors between March 1, 2019, and March 1, 2022. The study included pediatric patients up to age 18 years. They included those pediatric brain tumors whose (i) location was uncommon, or (ii) presented with unusual clinical presentation, or (iii) histopathology suggested to be a rare tumor, or (iv) radiological features were atypical.
They included 9 cases of rare unusual pediatric brain tumors. Three out of 9 cases required preoperative tumor embolization due to its hypervascular nature on digital subtraction angiography (DSA). All patients underwent surgical excision within 24-48 h of tumor devascularization. One out of 9 cases died in follow-up period due to pleural effusion and distant metastasis to lungs.
Treatment considerations for unusual pediatric brain tumors include a comprehensive multidisciplinary approach, including community-based screening and proper referral system for early treatment, a variety of treatment modalities, and sophisticated follow-up strategy. Government shall work in coherence with tertiary centers to spread social awareness and provide various financial scheme to prevent treatment dropouts 1).
In a retrospective study of 128 children diagnosed with a CNS tumor under 5 years of age at a single center in the Division of Pediatric Neurosurgery, University Hospital Zurich between 1990 and 2019.
The median age at diagnosis was 1.81 years [IQR, 0.98-3.17]. The Median follow-up time of surviving patients was 8.39 years [range, 0.74-23.65]. The main tumor subtypes were pediatric low-grade glioma (36%), pediatric high-grade glioma (11%), ependymoma (16%), medulloblastoma (11%), other embryonal tumors (7%), germ cell tumors (3%), choroid plexus tumors (6%), and others (9%). The 5-year overall survival (OS) was 78.8% (95% CI, 71.8-86.4%) for the whole cohort. Eighty-seven percent of survivors > 5 years had any tumor- or treatment-related sequelae with 61% neurological complications, 30% endocrine sequelae, 17% hearing impairment, and 56% visual impairment at last follow-up. Most patients (72%) attended regular school or worked in a skilled job at the last follow-up.
Young children diagnosed with a CNS tumor experience a range of complications after treatment, many of which are long-lasting and potentially debilitating. Our findings highlight the vulnerabilities of this population, the need for long-term support, and strategies for rehabilitation, specifically tailored for young children 2).
High-dose chemotherapy (HDC) and autologous stem cell transplantation (auto-SCT) are used to improve the survival of children with high-risk brain tumors who have a poor outcome with the standard treatment.
A study of Choi et al. from Seoul aimed to evaluate the outcome of HDC/auto-SCT with topotecan-thiotepa-carboplatin and melphalan-etoposide-carboplatin (TTC/MEC) regimens in pediatric brain tumors.
They retrospectively analyzed the data of 33 children (median age 6 years) who underwent HDC/auto-SCT (18 tandem and 15 single) with uniform conditioning regimens.
Eleven patients aged < 3 years at diagnosis were eligible for HDC/auto-SCT to avoid or defer radiotherapy. In addition, nine patients with high-risk medulloblastoma (presence of metastasis and/or postoperative residual tumor ≥ 1.5 cm2), eight with other high-risk brain tumor (six CNS primitive neuroectodermal tumor, one CNS atypical teratoid rhabdoid tumor, and one pineoblastoma), and five with relapsed brain tumors were enrolled. There were three toxic deaths, and two of which were due to pulmonary complications. The main reason for not performing tandem auto-SCT was due to toxicities and patient refusal. The event-free survival (EFS) and overall survival (OS) rates of all patients were 59.4% and 80.0% at a median follow-up with 49.1 months from the first HDC/auto-SCT, respectively. The EFS/OS rates of patients aged < 3 years at diagnosis, high-risk medulloblastoma, other high-risk brain tumors, and relapsed tumors were 50.0/81.8%, 87.5/85.7%, 66.7/88.9%, and 20.0/60.0%, respectively.
Although tandem HDC/auto-SCT with TTC/MEC regimens showed promising survival rates, treatment modifications are warranted to reduce toxicities. The survival rates with relapsed brain tumors were unsatisfactory despite HDC/auto-SCT, and further study is needed 3).
Gopalakrishna et al., retrospectively reviewed the chart of infants undergoing craniotomy for brain tumour removal from 2000 to 2017. The data related to preoperative variables, intraoperative management details and postoperative factors were collected and analysed. The primary outcome measure was occurrence of new postoperative neurological deficit (POND) and secondary outcome measure was hospital length of stay (LOHS).
The complete data was available for 40 infants undergoing craniotomy for excision of intracranial tumour. The new onset POND were found in 14 (35%) of infants. Based on logistic regression analysis, POND was associated with use of mannitol and massive blood transfusion (MBT) was found trending towards significance. Based on linear regression analysis, the risk factors associated with prolonged LOHS was reintubation and POND was found trending towards significance.
In this study, factors associated with new POND were mannitol use and to certain extent MBT. The variables associated with prolonged LOHS were reintubation and to certain extent POND. The anaesthetic technique, location of tumour, tumour histology and extent of tumour resection did not influence the occurrence of new POND or prolonged LOHS in infantile intracranial tumour surgery. Further prospective studies with larger sample size are required for confirmation of these findings and identification of new peri-operative risk factors 4).
From 2001 to 2008, 49 pediatric patients (mean age, 12.16 years) with tumors located in the intraventricular or paraventricular areas underwent neuroendoscopic biopsy, with or without simultaneous endoscopic third ventriculostomy. Neuroendoscopic biopsies were performed to verify the histological diagnosis of neoplasms and to establish pathological diagnoses necessary for planning appropriate treatment strategies.
In 45 of 49 patients (91.8%) neuroendoscopic biopsy specimens were appropriate for diagnosis and revealed 27 germinomas, 11 astrocytomas, and one ependymoma, etc. The tumor location included the pineal gland (n = 28), thalamus (n = 7), intraventricle (n = 3), hypothalamus (n = 3), suprasellar area (n = 2), and diffuse multifocal area (n = 3). In two patients (4.1%) biopsy specimens were informative but not diagnostic. Tumor tissue specimens were undiagnostic in two patients (4.1%). There were eight transient morbidities, including four EOM limitations, two central DI, one EVD infection, and one Cerebrospinal fluid fistula. One patient experienced postoperative tumor bleeding requiring emergent operation. There was no case of operative mortality.
Neuroendoscopic biopsy can be considered as the first choice for tissue sampling of periventricular and intraventricular tumors with acceptable risks 5).
Di Rocco et al., report on 51 infants with intracranial tumours treated in an eleven-year period; these infants represent 13% of the total population of children with intracranial tumours who have been operated on in the same institution during the same period of time. Males (28 cases) were slightly more frequent. Astrocytomas (17 cases), medulloblastomas (12 cases), and ependymal tumours (5 cases) were the commonest histologic types. Signs and symptoms of increased intracranial pressure were by far the most frequent clinical manifestations, followed by seizure disorders. Thirty tumours were localized within the supratentorial, and 21 within the subtentorial compartment. The parasellar region (10 cases) and the lateral cerebral ventricles (8 cases) for the supratentorial tumours, the inferior cerebellar vermis and fourth ventricle (13 cases) for the infratentorial tumours appeared to be the preferred topographic locations. Craniotomies were carried out in 44 infants, with a total or radical removal of the tumour in 19 cases, a subtotal removal in 6 cases, and a partial removal in 17 cases. In 3 cases only a biopsy procedure was performed. Twenty-nine of these patients required an ancillary procedure such as CSF shunting. Three subjects underwent a biopsy procedure and 1 infant the insertion of a CSF shunting device only. Surgery was not performed in 5 cases. Overall, there were two surgical deaths. Two infants died before any surgical treatment could be performed. Radiation therapy was administered to 9 patients when they had reached three years of age. Chemotherapy was given to 21 infants, according to various chemotherapeutic protocols. During the postoperative period 20 deaths (39%) were recorded. Two patients were lost to follow-up. From 1 to 10 years after the operation, 29 patients are still alive, 14 of them (28%) with a normal psychomotor development, 10 (20%) with some neurological or mental deficits, and 5 (10%) with severe psychomotor retardation. There was no apparent correlation in this series between late outcomes and the histological type of the tumour 6).
Between 1975 and 1989, 98 children with brain tumours under the age of three at time of diagnosis were entered into a retrospective study. Twenty of them are alive and free of tumour more than five years after treatment and were evaluated in this study. Thirteen tumour localizations were infratentorial and 7 were supratentorial. A histological examination was performed in 15 patients: 5 ependymomas, 6 medulloblastomas and 4 astrocytomas were identified. Fifteen patients underwent surgical removal of tumour, all but one received radiotherapy and 8 were given chemotherapy. Only two children have not late effects. Analysis of long-term sequelae in survivors showed central endocrinopathies in 14 (70%), a neurological handicap in 13 (65%) and impaired cognitive functions in 17 (85%). Irradiation was clearly responsible for mental sequelae in 7 patients and endocrinopathies in 6 patients. The other possible causes are tumour injury, hydrocephalus or surgery. The risks incurred with radiotherapy and advances in infant brain tumour therapy are discussed 7).