Childhood Meningiomas Research Articles

XRT-04. THE ROLE OF STEREOTACTIC RADIOSURGERY FOR MENINGIOMAS IN PEDIATRIC PATIENTS

Abstract Despite the generally benign and slow-growing nature, meningiomas in pediatric populations often exhibit more aggressive behavior. Stereotactic radiosurgery (SRS) is a non-invasive modality that is widely used as an upfront or adjuvant treatment for patients with meningiomas. Due to the rarity of meningiomas in pediatric patients, the outcomes of SRS for childhood meningiomas have never been described. From 1987 to 2022, a total of 2,030 patients with meningiomas underwent SRS at the University of Pittsburgh Medical Center. Nine patients (2 female; incidence rate = 0.44%) with a median age of 14.5 years (range, 9—17 years) at SRS were identified. Three patients (33.3%) had a history of total body irradiation. Seven patients (77.8%) were asymptomatic at the time of SRS presentation. Seven patients (77.8%) had a pre-SRS craniotomy; two patients (22.2%) had pre-SRS fractionated radiotherapy. The median cumulative tumor volume was 3.65 cc (range, 0.01—11.84 cc). The median margin dose prescribed was 14 Gy (range, 11—25 Gy). The median follow-up time was 107 months (range, 24—194 months). Five patients (55.6%) experienced tumor progression at a median of 25 months (range, 13—161 months). Local tumor control at 1-, 5-, and 10 years was 100%, 55.56%, and 55.56%, respectively. After local tumor progression, 2 patients received repeat SRS, 2 patients had surgical resection and 1 was placed on hydroxyurea for an unresectable tumor. At the date of the last clinical follow-up, 5 patients (55.6%) were still alive while 4 patients (44.4%) expired due to the progression of intracranial disease. 1-, 5-, and 10-year survival was 100%, 88.89%, and 64.81%, respectively. Childhood meningioma is a rare tumor that exhibits aggressive behaviors. SRS should be considered as an upfront or adjuvant treatment option in the multi-modal management of childhood meningiomas for optimal tumor control and maximal quality of life preservation.

RARE-34. UK CHILDREN’S CANCER AND LEUKAEMIA GROUP (CCLG): GUIDELINES FOR THE MANAGEMENT OF MENINGIOMA IN CHILDREN, TEENAGERS AND YOUNG ADULTS

Primary tumours of the meninges are rare accounting for only 0.4–4.6% of all paediatric tumours of the central nervous system. Due to the rarity of these tumours in children, and the consequent absence of collaborative prospective trials, there is no clear consensus on how the unique characteristics of paediatric meningiomas impact clinical status, management approach, and survival. Much of the evidence and treatment recommendations for paediatric meningiomas are extrapolated from adult data. Translating and adapting adult treatment recommendations into paediatric practice can be challenging and might inadvertently lead to inappropriate management. In 2009 Traunecker et al. published guidelines for the management of intracranial meningioma in children and young people on behalf of UK Children’s Cancer and Leukaemia Group (CCLG). Ten years later we have developed the updated guidelines following a comprehensive appraisal of the literature. Complete surgical resection is the treatment of choice for symptomatic meningiomas, while radiotherapy remains the only available adjuvant therapy and may be necessary for those tumours that cannot be completely removed. However, significant advances have been made in the identification of the genetic and molecular alterations of meningioma, which has not only a potential value in development of therapeutic agents but in surveillance of childhood meningioma survivors. This guideline builds upon the CCLG 2009 guideline. We summarise recommendations for the diagnosis, treatment, surveillance and long-term follow up of children and adolescents with meningioma.

Variations in screening and management practices for subsequent asymptomatic meningiomas in childhood, adolescent and young adult cancer survivors

Childhood, adolescent and young adult (CAYA) cancer survivors treated with cranial radiotherapy are at risk for developing subsequent meningiomas. There is insufficient evidence concerning the benefits and harms of screening for subsequent meningiomas, and uncertainty about the most appropriate clinical management of asymptomatic meningiomas. Data describing current clinical decision-making is essential to formulate surveillance recommendations. We created an online survey to identify the current international clinical practice regarding screening for and management of subsequent asymptomatic meningiomas among CAYA cancer survivors. Fifty-nine physicians from North America and Europe with expertise relevant to meningiomas were invited to participate. Thirty-four physicians (58%) completed the survey. The reported number of CAYA cancer survivors that physicians are willing to screen to detect one meningioma varied widely from 0 to 750 (median 50). Physicians expressed concerns regarding harms from MRI screening, including risks of unnecessary interventions (n = 25, 73%) and overdiagnosis (n = 19, 56%). Growth pattern (n = 33, 97%), location (n = 31, 91%) and size (n = 29, 85%) were endorsed as the most important factors influencing the decision to treat asymptomatic meningiomas. A challenging location (n = 14, 52%), indolent tumor growth pattern (n = 13, 48%), and absence of symptoms (n = 12, 44%) were endorsed as the main reasons to monitor without intervention. There is international variation in opinions and clinical practice regarding screening for subsequent asymptomatic meningiomas among at risk CAYA cancer survivors. Decision-making regarding interventions of asymptomatic meningiomas are largely driven by clinical characteristics. These valuable insights into current clinical practice will inform surveillance guidelines for CAYA cancer survivors.

Nasopharyngeal tumor developing in a 36-year-old man following radiation for a childhood meningioma.

Nasopharyngeal tumor developing in a 36-year-old man following radiation for a childhood meningioma.

Anaplastic Childhood Meningioma in a 16-Year-Old Boy

Meningiomas account for 0.4-4.6% of all primary brain tumors in children. Childhood meningiomas have several different characteristics than those occurring in adults. Case: Here we report a 16 year old male presented with long-standing headache and incipient weakness on the left side. Magnetic resonance images revealed a 65x47x37 mm sized, right sided frontoparietal parasagittal extra-axial mass. Histopathologic examination showed monotonous, small cells with oval/round nucleus forming whorl-like structures. The tumor showed high mitotic index and Ki67 proliferation rate around 30%. Immunohistochemically tumor cells were positive with EMA, vimentin, BCL-2, progesterone receptor. The final diagnosis according to histopathological, radiological and immunohistochemical findings was anaplastic meningioma. Conclusion: High-grade meningiomas are rare in children. Appropriate grading is important, as high grade tumors with high proliferation index are associated with less favorable clinical outcomes and warrant more radical treatment.

Cervico-occipital meningioma in a 5-year-old child: a case report.

Childhood meningiomas are scarce in clinical practice with an incidence ranging from 0.4 to 4.6% of all pediatric central nervous system (CNS) tumors. Cervico-occipital meningiomas account for 3.7% of childhood meningiomas and are slightly more frequent in male. A 5-year-old female presented with febrile posterior cervico-occipital pain for 3 weeks. She was diagnosed with meningitis and treated for a similar period with adapted antibiotics. The pain persisted even after treatment. Magnetic resonance imaging revealed an enhancing subdural extra medullary mass of the cervico occipital junction, developing around the left vertebral artery. The characteristics of the lesion were strongly suggestive of a neuroma. Surgical removal of the tumor aiming the decompression of the spinal cord and nerve roots was performed with a surprising discovery: The tumor was tightly attached to the dura at the entry of the left vertebral artery. The resection was total and only a thin part close to the artery was left. The pathological findings confirmed the diagnosis of meningothelial meningioma. Meningioma should be considered in the differential diagnosis of contrast enhancing subdural extra medullary lesions of the cervico-occipital junction in children.

The Founding of the International Neuro-Ophthalmology Society

The Founding of the International Neuro-Ophthalmology Society

Management of pediatric intracranial meningiomas: an analysis of 31 cases and review of literature

The purpose of this study is to understand the epidemiology, clinical features, behavior, and the prognostic factors that influence the outcome of intracranial meningiomas in children. Thirty-one patients with age less than 18 years who underwent surgery for intracranial meningiomas during the period of at 1988-2012 at Voluntary Health Services Hospital, Chennai, India were studied. These formed 3.7 % of the total 831 cases of intracranial meningiomas operated at the institute. The study group included 22 (71 %) males and 9 (29 %) females. The mean age of presentation was 15 years, with the youngest being 9 months old. Two (6 %) had evidence of neurofibromatosis type 2 (NF2). The most common symptoms at presentation were seizures in 11 (35.5 %), weakness of limbs in 11 (35.5 %), and raised intracranial pressure in 10 (32 %). Convexity meningiomas were seen in 16 (51 %) and skull base in 15(49 %). Multiple meningiomas was seen in two (6 %) of the patients and intraventricular location was found in one patient (3 %). Two (6 %) had evidence of NF2. Gross total excision was achieved in 26 (83 %) and subtotal excision in 5 (17 %). WHO grade I was found in 20 (64 %) and higher grade was seen in 11 (36 %). The mean follow-up was 46.2 months. Recurrence was seen in 20 patients (64 %). In patients with higher grade or with recurrence resurgery and radiotherapy was given. Three (9 %) had multiple recurrences. On a mean follow-up of 46.2 months, 25 patients (81 %) were neurologically intact, 5 (16 %) were having moderated disability, and 1 (3 %) patient died. Childhood meningiomas are uncommon lesions with a slight male predominance. They can have a varied clinical presentation. Higher grade is found more frequently compared with adults. Gross total resection is the goal and in higher grade meningiomas radiotherapy helps as a good adjuvant. Though the rate of recurrence is high, resurgery and radiotherapy gives a good outcome.

Clinical features and treatment of World Health Organization grade II and III meningiomas in childhood: report of 23 cases.

High-grade meningiomas in childhood are rare, and their clinical features are unknown. The objective of this study was to determine the clinical characteristics and prognosis of childhood high-grade meningiomas. Twenty-three patients with childhood high-grade meningiomas were treated at the Huashan Hospital. Clinical data were collected, tumor samples were reexamined, and prognoses were attained through follow-up visits and telephone interviews. Survival probability was calculated using the Kaplan-Meier method. A 2-sided probability level of 0.05 was chosen for statistical significance. The series included 18 males and 5 females (mean age 12.1 years). The most common symptoms were headache and vomiting (43%). Three patients had accompanying neurofibromatosis type II (NF2). The high-grade meningioma cases with NF2 had larger tumor diameters than those without NF2 (p = 0.010). The skull base was the most common tumor site (39%). Complete resections were achieved in 11 patients after their initial operations. Adjuvant radiation therapy was performed in 9 cases. Follow-up evaluations were performed for 20 patients (mean follow-up 70 months). Ten patients experienced recurrences, 2 patients had lung metastases, and 7 patients died of the recurrence. The extent of surgery was significantly related to progression-free survival (PFS; p = 0.038). A negative progesterone receptor combined with strongly positive Bcl-2 immunoreactivity was significantly related to PFS (p = 0.001) and overall survival (p = 0.002). The MIB-1 labeling index was significantly related to overall survival (p = 0.018), whereas postoperative radiation therapy was not significantly related to PFS (p = 0.087) and overall survival (p = 0.40). Childhood high-grade meningioma is a rare tumor type. Childhood high-grade meningioma has a male predominance and the basilar region is the most common tumor location. Patients with these tumors have high recurrence and mortality rates. The extent of resection is an important prognosis factor. A negative progesterone receptor combined with a strongly positive Bcl-2 immunoreaction might predict cancer recurrence. The MIB-1 labeling index correlates with the prognosis, and an MIB-1 labeling index > 3% increases the risk of recurrence in childhood high-grade meningioma. More cases should be collected, and longer follow-up periods should be obtained, to evaluate the effects of postoperative radiation therapy in childhood high-grade meningioma.

Intracranial meningiomas managed at Memfys hospital for neurosurgery in Enugu, Nigeria

Introduction:The epidemiology and pathology of meningioma in Nigeria are still evolving and little has been published about this tumor in Nigeria, especially in the southeast region. The aim of this paper is to compare the characteristics of intracranial meningioma managed in our center with the pattern reported in the literature worldwide.Materials and Methods:Retrospective analysis of prospectively recorded data of patients managed for intracranial meningioma between January 2002 and December 2010 at a Private neurosurgery Hospital in Enugu, Nigeria. We excluded patients whose histology results were inconclusive.Results:Meningiomas constituted 23.8% of all intracranial tumors seen in the period. The male to female ratio was 1:1.1. The peak age range for males and females were in the fifth and sixth decades, respectively. The most common location is the Olfactory groove in 26.5% of patients followed by convexity in 23.5%. Presentation varied with anatomical location of tumor. Patients with olfactory groove meningioma (OGM) mostly presented late with personality changes and evidence of raised ICP. Tuberculum sellar and sphenoid region tumors presented earlier with visual impairment with or without hormonal abnormalities. Seizures occurred in 30.9% of all patients and in 45% of those with convexity meningiomas. Only 57.4% of the patients were managed surgically and there was no gender difference in this group. WHO grade1 tumors were the most common histological types occurring in 84.6%. One patient had atypical meningioma and two had anaplastic tumors.Conclusion:The pattern of meningioma in our area may have geographical differences in location and histology. Childhood meningioma was rare.

Pediatric meningiomas: a single-center experience with 15 consecutive cases and review of the literature

The goal of this study was to determine the epidemiology, clinical presentation, associated factors, pathological features, and treatment outcome of pediatric meningiomas in a single-center institution. Clinical data of 15 patients under 18 years of age operated on for meningiomas from January 1994 to December 2010 were reviewed. The study group included nine males and six females (mean age of 13 years at surgery). The most common symptoms at presentation were headaches in 6 out of 15 (40 %), raised intracranial pressure in 3 out of 15 (20 %), and seizures in 3 out of 15 (20 %). Sole operated tumors were found in 12 out of 15 (80 %), whose location is as follows: parasagittal in 4 out of 12 (33.3 %), 2 in the convexity (16.6 %), 2 at the skull base (16.6 %), and 4 in other sites (33.3 %). Six children presented with radiation-induced (RT) meningiomas and five had evidence of neurofibromatosis type 2 (NF2). Three patients had multiple meningiomas (all of them had NF2). Simpson's grade I excision was achieved in 12 out of 15 (80 %). On histopathology, 11 out of 15 (73.3 %) were grade I and 4 out of 15 (26.6 %) were grade II (all of them atypical). Five tumors (33.3 %) recurred, four of which had RT or NF2. During the mean follow-up period of 5 years, 12 out of 15 (80 %) had a good outcome (GOS=5). Childhood meningiomas are uncommon lesions with a slight male predominance. Absence of large series with long follow-up precludes any definite conclusions on the clinical course and outcome of these tumors. Associated factors (such as RT and NF2), location, and extent of excision appear to be more important than histopathological grade in predicting outcome.

Meningioma as second malignant neoplasm after oncological treatment during childhood

A total of 38patients (18female/20male) with childhood meningioma were recruited from the German registry HIT-Endo (1989-2009). In 5cases meningioma occurred as second malignant neoplasm (SMN). Histologies were confirmed by reference assessment in all cases (SMN: 2 WHO I, 1 WHO II, 2 WHO III). The SMNs were diagnosed at a median age of 12.4years with a median latency of 10.2years after primary malignancy (PMN; 4brain tumors, 1lymphoblastic leukemia; median age at diagnosis 2.7years). Meningioma occurred as SMN in the irradiated field of PMN (range 12-54Gy). The outcome after treatment of SMN meningioma (surgery/irradiation) was favorable in terms of psychosocial status and functional capacity in 4 of 5patients (1death). We conclude that survivors of childhood cancer who were exposed to radiation therapy at young age harbor the risk of developing meningioma as a SMN at a particularly short latency period in case of high dose exposure.

Spinal meningioma in childhood: clinical features and treatment

The purpose of this retrospective study is to determine the clinical characteristics and the prognosis of the spinal meningioma in childhood (under 18years of age) based on the treatment at our institution. Ten spinal meningioma cases in children were treated during the last 9years. The clinical data was retrospectively analyzed and the results were compared with those in the literature. The series included eight males and two females and the mean age was 13.2years. The most common initial symptoms were pain (6/10) and the common signs were limb weakness (4/10) and gait disturbance (2/10) and distal paresthesia (1/10) and bladder disturbance (1/10). Four patients had other clinical signs of neurofibromatosis type II (NF-2) such as tumors elsewhere. All the tumors were located in cervical and thoracic vertebrae. Resection according to Simpson Grade I (6/10), II (2/10), III (1/10), and IV (1/10) were performed. Grade II meningiomas accounted for 3/10 in this series. All patients were followed up with mean follow-up period of 43months. Seven patients had recurrence of the tumor in that period and one had died. Spinal meningioma is an uncommon pediatric neoplasm and has a poor prognosis. It has a male predominance and is inclined to be associated with NF-2, and those that are associated with higher pathologic subtypes and NF-2 have more unfavorable outcome. Every effort should be made to achieve total removal which may decrease the incidence of recurrence.

16 MONTH‐OLD FEMALE WITH INTRAVENTRICULAR MASS

Pediatric meningiomas are rare and account for about 1.5% of all intracranial tumors. When compared to adults, intraventricular location of childhood meningiomas is four to ten times as high. Atypical pathology of these lesions is very uncommon and indicates an aggressive nature. They are usually associated with Neurofibromatosis 2 (NF2) or previous cranial irradiation. Here, we present an interesting case of an unusually large, congenital intraventricular meningioma of atypical pathology in a 16 month old child with subsequently diagnosed NF2. A brief review of literature is also presented with this case illustration.

小児の嚢胞性髄膜腫の2症例

小児の嚢胞性髄膜腫の2症例

Intraventricular metaplastic meningioma in a child: case report and review of the literature

Childhood meningiomas are rare and display important differences from adult forms. We report the first case of an intraventricular metaplastic meningioma arising in a child. A 7-year-old female underwent resection of an enhancing tumor arising within the left lateral ventricle. It was composed of monomorphic cells embedded within an abundant myxoid stroma. The cells demonstrated epithelial membrane antigen and vimentin immunoreactivity. Ultrastructural analysis demonstrated intermediate filaments, complex intercellular interdigitations and desmosomes, and a diagnosis of myxoid (metaplastic) meningioma was rendered. This case reflects the higher incidence of intraventricular meningiomas in childhood and greater incidence of intraventricular meningiomas in the left lateral ventricle. Recognition of the grade I myxoid meningioma in this case is paramount since chordoid meningiomas, which share similar histologic features, are of a higher grade and worse prognosis.

Childhood and juvenile meningiomas

The purpose of this paper was to study the clinical characteristic, treatment, and prognosis of childhood meningioma. Fifty-four meningioma cases below the age of 18 have been treated in Huashan Hospital in the last 15 years (from 1993 to 2008), their sex and age distribution, clinical manifestation, radiological finding, pathological subtype, treatment, and prognosis are retrospectively analyzed, and the results are compared with those reported in the literature. The ratio of male to female was 1.16:1 (29:25) and mean age was 13.14 years (ranging from 2.75 to 18 years). Five patients in this series were associated with neurofibromatosis-2. The most common symptoms and signs were the signs of increased intracranial pressure including headache, papilledema, and vomiting. The most common radiological finding was homogeneous enhancement with contrast. The most common locations of meningiomas in this series were intraventricular and anterior/middle fossa. All of these patients were surgically treated; resection both in Simpson grades I and II could be achieved in 39 out of 54 patients. Perioperative mortality was 3.7% (2 out of 54); the most common postoperative complications were intracranial infection and injury of cranial nerve. Fibroblastic meningiomas were the most common pathological subtype, and malignant and atypical meningiomas (both of grades II and III according to WHO classification) accounted for 18.5% of the whole series. Childhood meningiomas are scarce in clinical practice, which are characterized with its male predominance, large size, distinctive radiological features, and high rate of atypical/malignant histological subtypes; favorable outcome is accessible with total removal.

Intracranial meningiomas of childhood and adolescence: report of 34 cases with follow-up

The objective of this study is to investigate the characteristics of rare meningiomas occurring during childhood and adolescence specially demography, locations, radiological findings, pathological features, and outcome; 34 patients can be followed up and analyzed. Fifty-nine patients with meningiomas were treated during the period of 2000-2007 at the Department of Neurosurgery, Beijing Tiantan Hospital. Thirty-four cases which can be followed up were analyzed by retrospective method. Clinical presentation, anatomical distribution, radiological findings, clinical behavior, outcome, and follow-up are summarized. All cases had been performed microsurgical treatment including 20 cases totally resection. Eleven cases have been achieved subtotal resection. Two patients have been performed biopsy because of giant tumor volume. One patient has been partially resected for internal carotid artery involved. The operative mortality is zero. The average follow-up period is 4 years long. There was recurrence in seven and metastasis in two. Adjuvant radiotherapy has been performed on residual, malignant, and recurrent tumors. Six patients are dead. The sex predominance is not similar to that in adults. Increased ICP, decreased visual acuity, and seizure are the common presentation. There is a higher incidence of intraventricle and skull base region. Larger size and frequent abnormal tumor calcification and cyst appear not relative to pathological subtypes. The higher incidence of atypical histopathological features than adult is affirmative. The prognosis is good if the tumor can be totally removed and adjuvant radiotherapy not to be significant beneficial after incomplete excision.

Childhood and adolescent meningiomas: a report of 38 cases and review of literature

The aim is to study the clinical, radiological and pathological features of childhood and adolescent meningiomas and analyse outcome prognosticators. A retrospective analysis of the case records of patients less than 20 years of age operated for a meningioma in our institute since 1982 was performed. The variables analysed included age, sex, presentation, associated neurofibromatosis (NF), imaging characteristics, extent of resection and histopathology. The study group included 20 males and 18 females with a mean age of 15.53 years. Eleven children (28.9%) had evidence of NF of whom three had NF2 with bilateral vestibular schwannomas. The common presenting symptoms were seizures (76.3%), raised intracranial tension (71%), and focal neurological deficits (39.4%). The location of the operated tumours were as follows: ten skull base (24.4%), ten falx/parasagittal (24.4%), eight spinal (19.5%), five convexity (12.2%), three posterior fossa (7.3%), three intraventricular (7.3%) and two optic nerve sheath (4.9%). Two children (4.9%) had cystic meningiomas. Grade I excision was achieved only in twenty tumours (48.8%). On histopathology, thirty (73.2%) were grade I, nine (21.9%) were grade II and two (4.9%) were grade III meningiomas. Seven tumours recurred of which six were located at the skull base. During the mean follow up period of 4.74 years, the majority, 32 (84.2%) had a good outcome and five (13.2%) had a poor outcome. One child (2.6%) expired due to post-operative sepsis. Childhood meningiomas are uncommon but not rare lesions with a marginal male predominance. Absence of large series with long follow up precludes any definite conclusions on the clinical course and outcome. Uniform observations made in different series including ours, include a higher incidence of the skull base location and tumours with atypical histopathology. Favourable prognostic factors include younger age (< than 10 years), superficial location, total excision and absence of neurofibromatosis. Location and extent of excision appear to be more important than histopathology grade in predicting outcome.

Meningiomas in childhood and adolescence – Multicenter cross-sectional study on 42 patients

Meningiomas in childhood and adolescence – Multicenter cross-sectional study on 42 patients