Intracranial meningiomas in children: Review of 29 cases

Paediatric posterior fossa lesions can have much overlap in their clinical and radiological presentation. There are, however, a number of key imaging features that can help the reading radiologist to distinguish tumours from important tumour mimics which are often inflammatory or metabolic entities. This pictorial review provides a number of important cases that proved challenging on imaging and illustrates some common pitfalls when interpreting lesions in the posterior fossa in children. Not everything that is abnormal will be a tumour, but often other causes are overlooked and misinterpreted as tumours, leading to great morbidity for that child. This article highlights some lesions that were mistaken as tumours and will introduce the reader to less commonly seen pathologies which are important to consider on a differential list for this location.

To evaluate the relationship between meningioma histological subtype and tumor site in under-20 year-olds.

A review of the literature on meningioma during the first 2 decades of life was carried out through a Medline search up to February 2019. To evaluate the adult population, a cross-sectional study was conducted on patients operated on between 2000 and 2014 in a single institution. Exclusion criteria comprised: series reports and papers that lacked detailed description of clinical findings, neuroimaging confirmation of tumor location, and/or at least 5 years’ follow-up.

One hundred and seven manuscripts were included, for 365 under-20 year-old patients: 200 male, and 164 female. Histopathology found 197 cases (53.9%) of WHO grade I meningioma, with predominance of meningothelial (41.1%) and transitional (30.9%) subtypes; 123 (33.7%) grade II, and 45 (12.3%) grade III. For 65 (18.25%) of the 356 cases, recurrence was documented, with only 24 deaths (6.7%).

Meningioma in this population presented 2 differences compared to the adult population: male predominance, and high incidence of atypical meningioma. Surgery was the primary treatment. Adjuvant radiotherapy is controversial in the literature.

More than 70,000 primary central nervous system tumors are diagnosed in the United States each year. Approximately 36% of these are meningiomas, making it the most common primary brain tumor. Because meningioma risk increases dramatically with age, the healthcare burden of meningioma in the developed world will continue to rise as demographics shift toward an older population. In addition to demographic factors associated with increased meningioma risk (i.e., older age, female sex, African American ethnicity), increased body mass index is a strong risk factor. A history of atopic allergies, eczema, and increased serum IgE are all consistently associated with reduced meningioma risk, suggesting a potential role for immunosurveillance. Although ionizing radiation is a strong meningioma risk factor, it accounts for very few cases at the population level. Recent studies suggest that diagnostic radiation (e.g., dental X-rays) increases meningioma risk. Because radiation dosages associated with medical imaging have decreased dramatically, the public health impact of this exposure is likely in decline. Genome-wide association studies have identified common inherited variants in the gene MLLT10 and RIC8A as low-penetrance meningioma risk alleles. To provide further insight into the etiology of meningioma, future studies will need to simultaneously examine genetic and environmental risk factors, while also stratifying analyses by subject sex.

Parasagittal meningiomas (PM) are very common intracranial lesion but their occurrence in young adult is very rare. Most giant PM are characterized with the invasion of the superior sagittal sinus and form very huge collaterals which makes surgical resection very difficult. The origin of meningioma is still a matter of debate which make our case much more puzzling. We therefore reviewed detailed literature on the etiologies of meningioma as well.

We present a case of 24-years old young lady with history of seizures, blurred vision, intermittent tremors and occasional dropping of objects on the right arm. She had dizziness, headaches, tinnitus and paresthesia on the right side with loss of smell. She had a transient amaurosis fugax once before presenting to us. MRI done revealed a huge tumor compression on the left fronto-parietal gyrus and invading the superior sagittal sinus (SSS). The goal of surgery in this young lady was to preserve the SSS completely, stop frequent seizures and also preserve eloquent areas since the lesion was lying directly on the focal motor area. With a good preoperative evaluation and careful resection of the tumor, we achieved total resection without any further neurologic complications.

We believe the etiology of meningioma in our patients started at childhood and progress to this giant status. We therefore suggest that neurosurgeon's screen children early to detect meningiomas before they advance into giant stages. Two years follow-up after surgery showed no tumor recurrence.

Rhabdoid meningiomas are rare World Health Organization grade 3 tumors that tend to follow an aggressive course, with an increased likelihood for local recurrence, remote metastasis, and cerebrospinal fluid dissemination. Genetic testing has found certain genes associated with reduced time to tumor recurrence. BAP1 (BRCA1-associated protein 1) is a tumor suppressor gene that is associated with multiple tumors, including rhabdoid meningiomas.

We present a case of a pediatric patient who presented with a rhabdoid meningioma occurring in the right tentorium and invading multiple venous structures, including the right jugular vein. The patient underwent 5 separate operations for management of this tumor. The first surgery was an intracranial tumor debulking with reconstruction of venous structures. Postoperatively, the patient was unable to have the ventricular catheter removed and underwent placement of a ventriculoperitoneal shunt. Significant recurrence of the intracranial portion of tumor was found during preoperative imaging for her second stage procedure. She underwent a second craniotomy for resection of the tumor. Her postoperative magnetic resonance imaging showed significant residual tumor and the patient therefore underwent a third craniotomy for total tumor resection, which involved reconstruction of the superior sagittal sinus. She did well after this surgery, with no new neurologic deficits. Her final operation involved resection of the residual tumor in the neck and chest by both otolaryngology and cardiothoracic surgery. This surgery involved opening the jugular vein and resecting residual tumor from the intima. Pathologic results from all surgeries were consistent with rhabdoid meningioma; however, the tissue from the biopsy and first craniotomy lacked the high-grade features that were found on subsequent resections. Genetic analysis found loss of both BAP1 tumor suppressor genes. Peripheral blood testing showed that this patient was a germline carrier of a pathogenic BAP1 variant.

Pediatric rhabdoid meningiomas represent a rare disease and are found on recurrent tumors in conjunction with lower-grade meningioma disease. Our patient presented with what was initially believed to be a low-grade meningioma with rhabdoid features, which then transformed into a World Health Organization grade III rhabdoid meningioma on recurrence. This tumor was discovered to have a biallelic loss of BAP-1 mutation and the patient was found to have a germline mutation in 1 of her BAP-1 alleles. Germline mutations in BAP-1 are associated with a cancer syndrome that involves uveal and cutaneous melanoma, malignant mesothelioma, atypical Spitz tumors, and clear-cell renal cell carcinoma. Patients with this mutation are encouraged to undergo annual eye examinations starting at the age of 11 years. The BAP-1 tumor predisposition syndrome is most commonly an inherited mutation associated with incomplete penetrance and variation with nonoverlapping tumor types.

Rhabdoid meningiomas are unlikely to be found in children and have a high rate of local recurrence. Gross total resection has to be balanced with risk of postoperative deficit. Genetic testing of this rare entity should be performed to identify any hereditary germline mutations.

Meningioma is one of the most common primary brain neoplasms with poor outcomes. The present study was aimed to determine clinical and surgical characteristics of intracranial meningiomas associated with tumor recurrence and complications.

A total of 138 patients undergoing surgical resection of intracranial meningiomas between Jan 2003 and Dec 2014 were included and followed for the period of at least 12 months. The demographic and clinical characteristics possibly associated with tumor recurrence were assessed, including age, gender, clinical symptoms, pathology data, tumor parameters, preoperative and postoperative Karnofsky Performance Scale (KPS), complications and recurrence rate.

One hundred and twenty one lesions were benign (classified as Grade I) and 17 were atypical/malignant (classified as Grade II/III). The patients were of a mean age of 60.5 years and a mean follow-up duration of 36.8 months (range, 16.3–62.9 months). The 1, 3, 5-year recurrence/tumor enlargement rates were 3.4%, 7.2%, and 15.7%, respectively. In multivariate analysis, symptoms of disturbance of consciousness and palpable cranial mass were associated with increased recurrence/tumor enlargement. In addition, patients with Simpson grade IV were more likely to have recurrence/tumor enlargement.

The pattern of intracranial meningioma in this series is typical to other studies. Presenting symptoms is suggested to be predictive of recurrence.