Rare Intracranial Tumor Research Articles

Large suprasellar mass: answer

Teratomas are rare intracranial neoplasms that make up approximately 0.4% of all primary intracranial tumours [1]. They can be divided into extra-axial and intra-axial teratomas [2]. Extra-axial tumours usually arise in the suprasellar or pineal regions and typically present in childhood with a recent case series demonstrating a mean age of 14.5 years [3]. In contrast, intra-axial teratomas are the most common intracranial foetal tumour and present in the antenatal period with a mean gestational age at diagnosis of 27 weeks [4,5]. Intra-axial teratomas are more commonly supratentorial with a predilection for the frontal lobes [5]. Regardless of location, extragonadal teratomas are believed to develop from ectopic, totipotent primordial germ cells that have either been misplaced or undergone aberrant migration during early embryogenesis [2,6]. Because of this cellular origin, teratomas can contain diverse mixed tissues with up to 90% of childhood teratomas containing elements derived from all three embryonic layers (ectodermal, mesodermal and endodermal) [2]. This is in contrast to epidermoid and dermoid cysts which are both derived from ectodermal origin only [2]. Importantly, although dermoid cysts have sebaceous glands which produce liquid oily sebum appearing as fat density on CT scan and on MRI demonstrating T1 shortening, chemical shift artefact and signal attenuation on fat suppressed sequences, they do not contain solid adipose tissue as lipocytes are mesodermal in origin [2,7]. Similarly, dermoid cysts may contain semi-formed teeth but do not contain cartilage or bone [8]. It should be noted, however, that the absence of detectable fat or bone on imaging does not exclude the diagnosis of teratoma [9]. In this patient, the detection on CT scan of fatty tissue and bone within the mass is the key for diagnosis (Question: Fig. 1a–c). The presence of these tissues within a mixed cystic and solid enhancing mass is strongly suggestive of a pre-operative diagnosis of teratoma, as contrast enhancement is rarely seen in dermoid cysts [10]. MRI confirmed the presence of fat adjacent, but separate to, the right posterior clinoid process identified on CT scan as an area of intrinsic high T1 signal with surrounding chemical shift artefact on the T2 sequence (Question: Fig. 1d–f). Histology confirmed the radiological diagnosis of intracranial teratoma (Fig. 1).

P019 - Intracranial Germinoma: Very rare mimicker of multiple sclerosis

Background Multiple sclerosis (MS) mimickers are neurological conditions that may share clinical, biologic and radiologic features similar to MS. Standard MS treatments often fail. Objectives To report a very rare case of intracranial Germinoma with clinical, biological and radiological features similar to MS. Methods We report 26 year old Emirati male patient who presented with history of recurrent attacks of neurological dysfunction and recurrent brain stem lesions since 2010. Results MRIs of the brain revealed recurrent brain stem T2 hyperintense lesions changing in size and location but remained localized in the brain stem. Initially CSF was positive for OCB so he was diagnosed as clinically isolated syndrome and was started on Rebif injections. In 2011 he developed another relapse and new lesion appeared in the left side of the brain stem like mirror image of the old right side lesion which has disappeared. The symptoms included episodic diplopia, facial paresthesias with subsequent brainstem symptoms that improve with a high dose of corticosteroids. He developed oral ulceration in 2011. Diagnostic work up excluded all other possibilities and Neuro Behcet's disease (NBD) was considered, he responded well to Azathioprine and steroid. He remained well but in 2013 he developed relapse and MRI revealed that left sided midbrain has expanded. CSF revealed lymphocytic pleocytosis without OCB. Patient refused brain biopsy. He received three cycles of Infliximab but he deteriorated clinically and radiologically. The behavior of the disease and the lesion was suggestive of MS in the first year and suggestive of NBD in the second and third year, but in the fourth year it was not typical of MS or NBD. Brain biopsy was performed at John Hopkins hospital in USA in April 2014 and the lesion was reported Germinoma responsive to radiotherapy. The tumor disappeared after radiotherapy. Conclusion Intracranial Germinoma is very rare intracranial tumor that occurs at 0.7 per million, it is even more rare to be a mimicker for MS. This patient was treated successfully with radiotherapy. Multiple sclerosis (MS) mimickers are neurological conditions that may share clinical, biologic and radiologic features similar to MS. Standard MS treatments often fail. To report a very rare case of intracranial Germinoma with clinical, biological and radiological features similar to MS. We report 26 year old Emirati male patient who presented with history of recurrent attacks of neurological dysfunction and recurrent brain stem lesions since 2010. MRIs of the brain revealed recurrent brain stem T2 hyperintense lesions changing in size and location but remained localized in the brain stem. Initially CSF was positive for OCB so he was diagnosed as clinically isolated syndrome and was started on Rebif injections. In 2011 he developed another relapse and new lesion appeared in the left side of the brain stem like mirror image of the old right side lesion which has disappeared. The symptoms included episodic diplopia, facial paresthesias with subsequent brainstem symptoms that improve with a high dose of corticosteroids. He developed oral ulceration in 2011. Diagnostic work up excluded all other possibilities and Neuro Behcet's disease (NBD) was considered, he responded well to Azathioprine and steroid. He remained well but in 2013 he developed relapse and MRI revealed that left sided midbrain has expanded. CSF revealed lymphocytic pleocytosis without OCB. Patient refused brain biopsy. He received three cycles of Infliximab but he deteriorated clinically and radiologically. The behavior of the disease and the lesion was suggestive of MS in the first year and suggestive of NBD in the second and third year, but in the fourth year it was not typical of MS or NBD. Brain biopsy was performed at John Hopkins hospital in USA in April 2014 and the lesion was reported Germinoma responsive to radiotherapy. The tumor disappeared after radiotherapy. Intracranial Germinoma is very rare intracranial tumor that occurs at 0.7 per million, it is even more rare to be a mimicker for MS. This patient was treated successfully with radiotherapy.

Imaging characteristics of primary intracranial teratoma

Primary intracranial teratomas are rare intracranial neoplasms, and are subdivided into mature, immature, and those with malignant transformation. To date, only a few studies of teratoma imaging have been reported. To describe and characterize the magnetic resonance imaging (MRI) findings in a series of 18 patients (16 men/boys and 2 women/girls; mean age, 14.5 years) with pathologically proven teratomas. Findings from medical records and imaging examinations in 18 patients with pathologically confirmed intracranial teratomas from 2001 to 2011 were retrospectively reviewed at our two institutions. Two radiologists evaluated the lesion location, shape, size, number, edge, homogeneous or heterogeneous appearance, attenuation, signal intensity, and degree of enhancement. All tumors were located within the pineal (n = 13), parasellar (n = 2), or suprasellar (n = 3) regions. The lesions appeared of mixed intensity on MRI, reflecting the histologic heterogeneity, including fibrosis, fatty tissue, calcification, cysts, and keratinocytes. In mature teratomas (n = 9), seven of nine tumors showed non-enhanced multilocularity or heterogeneous enhancement of the cyst wall on contrast-enhanced T1-weighted (T1W) images. Two of nine tumors showed moderate, heterogeneous enhancement in the solid portion of the lesion; whereas in immature (n = 7) or malignant transformation (n = 2) teratomas, heterogeneous, ring-like, intratumoral patchy enhancement was noted on T1W images with contrast. Primary intracranial teratomas are usually localized in the pineal and the suprasellar regions, and often present an ovoid or lobulated mass with or without multilocularity on MRI. Marked enhancement of the solid portion or the thick wall of the tumor was the key feature for distinguishing mature teratoma and malignant teratoma.

Pituitary macroadenoma presenting as a nasal tumor: case report

Pituitary macroadenomas are rare intracranial tumors. In a few cases, they may present aggressive behavior and invade the sphenoid sinus and nasal cavity, causing unusual symptoms. In this paper, we report an atypical case of pituitary adenoma presenting as a nasal mass. The patient was a 44-year-old woman who had had amenorrhea and galactorrhea for ten months, with associated nasal obstruction, macroglossia and acromegaly. Both growth hormone and prolactin levels were increased. Magnetic resonance imaging showed a large mass originating from the lower surface of the pituitary gland, associated with sella turcica erosion and tumor extension through the sphenoid sinus and nasal cavity. Histopathological analysis demonstrated a chromophobe pituitary adenoma with densely packed rounded epithelial cells, with some atypias and rare mitotic figures. There was no evidence of metastases. Macroadenoma invading the nasal cavity is a rare condition and few similar cases have been reported in the literature. This study contributes towards showing that tumor extension to the sphenoid sinus and nasopharynx needs to be considered and investigated in order to make an early diagnosis when atypical symptoms like nasal obstruction are present.

Management of hypoglossal schwannomas: single institutional experience of 14 cases.

Background Hypoglossal schwannomas are rare intracranial neoplasms. Microsurgical resection with the goal of cure is the aim of management but is associated with a high rate of postoperative morbidity. Objective The objective of the study was to outline the clinical presentation, radiologic characteristics, surgical techniques, postoperative morbidity, and long-term follow-up results for hypoglossal schwannomas. Methods Patients treated for hypoglossal schwannoma at the Department of Neurosurgery of a tertiary-level referral institution from January 2001 until December 2010 were analyzed retrospectively using hospital records. Results There were 14 patients who were treated in the study period. Tongue atrophy and swallowing difficulties were the most common presenting symptoms. Surgery done in 12 patients using a variety of approaches (retromastoid retrosigmoid suboccipital in 9, midline suboccipital in 2, and far lateral in 1). Five patients having small residual tumors received gamma knife (GK) subsequently. two patients received primary GK stereotactic radiosurgery. Three patients had permanent morbidity in the form of cranial nerve paresis. Immediate postoperative complications like cerebrospinal fluid leak and pneumonia were present in three patients. Conclusion Complete microsurgical resection is often associated with a high rate of morbidity. Subtotal and near-total resection followed by stereotactic radiosurgery or observation now offers an alternative approach.

Neuro-Ophthalmologic Features of Chordoid Glioma

Chordoid glioma is a rare intracranial tumor typically arising in the third ventricle, particularly along the anterior aspect of the hypothalamic wall. We describe the clinical, neuroimaging, and pathologic factors of this neoplasm in a patient presenting with a chiasmal syndrome.

Pituitary macroadenoma presenting as a nasal tumor: case report Macroadenoma hipofisário, apresentando-se como um tumor nasal: relato de caso

CONTEXT: Pituitary macroadenomas are rare intracranial tumors. In a few cases, they may present aggressive behavior and invade the sphenoid sinus and nasal cavity, causing unusual symptoms. In this paper, we report an atypical case of pituitary adenoma presenting as a nasal mass. CASE REPORT: The patient was a 44-year-old woman who had had amenorrhea and galactorrhea for ten months, with associated nasal obstruction, macroglossia and acromegaly. Both growth hormone and prolactin levels were increased. Magnetic resonance imaging showed a large mass originating from the lower surface of the pituitary gland, associated with sella turcica erosion and tumor extension through the sphenoid sinus and nasal cavity. Histopathological analysis demonstrated a chromophobe pituitary adenoma with densely packed rounded epithelial cells, with some atypias and rare mitotic figures. There was no evidence of metastases. CONCLUSION: Macroadenoma invading the nasal cavity is a rare condition and few similar cases have been reported in the literature. This study contributes towards showing that tumor extension to the sphenoid sinus and nasopharynx needs to be considered and investigated in order to make an early diagnosis when atypical symptoms like nasal obstruction are present.

Intradural clival chordoma: a case report.

Clival chordoma is a rare intracranial neoplasm located in the clivus with bony extension and destruction. It is difficult to resect completely and generally has a poor prognosis. However, intradural clival chordomas have been reported with good surgical outcomes. We present a rare case of intradural chordoma and a review of the literature.

Myoepithelioma of the cerebellopontine angle: a previously not documented benign salivary gland-type neoplasm within the cranium

Myoepithelioma is a dimorphic neoplasm with contractile-epithelial phenotype, originally interpreted as deriving from, but not actually restricted to the salivary glands. As a novel addition to the list of exquisitely rare intracranial salivary gland-type tumors and tumor-like lesions, we report on an example of myoepithelioma encountered in the left cerebellopontine angle of a 32-year-old male. Clinically presenting with ataxia and dizziness, this extraaxial mass of 4 × 3.5 × 3 cm was surgically resected, and the patient is alive 6 years postoperatively. Histologically, the tumor exhibited a continuum ranging from compact fascicles of spindle cells to epithelial nests and trabeculae partitioned by hyalinized septa, while lacking tubular differentiation. Regardless of architectural variations, there was robust immunoexpression of S100 protein, smooth muscle actin, GFAP, cytokeratin, and vimentin. Cytologic atypia tended to be modest throughout, and the MIB1 labeling index averaged less than 1%. Fluorescent in situ hybridization indicated no rearrangement of the EWSR1 locus. We interpret these results to suggest that myoepithelioma of the posterior fossa - along with related salivary epithelial tumors in this ostensibly incongruous locale - may possibly represent analogous neoplasms to their orthotopic counterparts, ones arising within aberrant salivary anlagen. The presence of the latter lends itself to being mechanistically accounted for by either postulating placodal remnants in the wake of branchial arch development, or linking them to exocrine glandular nests within endodermal cysts. Alternatively, myoepithelioma at this site could be regarded as a non tissue-specific lesion similar to its relatives ubiquitously occurring in the soft parts.

Surgical strategy and seizure outcome in dysembryoplastic neuroepithelial tumor

Objective Dysembryoplastic neuroepithelial tumor (DNET) is a rare intracranial tumor with drug-resistant epilepsy.The authors present a review of 18 patients who underwent surgical removal of a DNET to discuss the surgical strategy epilepsy outcome.Methods There are 18 DNET patients from Aprial 2004 to Aprial 2010,4 patients were lost to follow up.Medical records,neuroradiological images of all 14 patients were investigated.There were 10 male and 4 female patients.Age of patients ranged from 4 to 25 years,mean 10.3.We preferred lesionectomy and intraoperative electrocorticography concomitantly.After tumor removal,we would coagulate surrounding cortex with bipolar if there were still spikes or sharp waves on electrocorticography.Engel prognosis was recorded for every patient.Results Time of follow-up ranged from 24 to 96 months,mean 46.6 months.Tumor was removed totally in 12 cases,partially in 2 cases.Intraoperative ultrasound was used in five patients and found tumor omission in 1 among the 5 patients.Engel prognosis was Ⅰ grade in 12 patients whose tumor was removed totally,and complete antiepileptic drugs withdrawn was achieved in 11 out of 12 patients.The other two patients got Engel Ⅱ and Ⅳ prognosis respectively.Tumor residue didn't regrow in a follow-up time of 54,63 months respectively.Conclusion Lesionectomy and bipolar coagulation are effective for seizure control.Residual tumor is a significant risk factor for poor seizure outcome.Intraoperative ultrasound are recommended to make sure total removal.Electrocorticography should be routinely used before and after tumor removal for a better seizure outcome. Key words: Dysembryoplastic neuroepithelial tumor; Operation; Epilepsy

Atypical Presentation of Atypical Teratoid Rhabdoid Tumor in a Child

Atypical Teratoid Rhabdoid Tumor (ATRT) is a rare malignant intracranial neoplasm more commonly diagnosed in young children. The authors report the case of an 11-year-old boy with a long standing history of slowly progressive weight loss, fatigue, and weakness over 1.5 years whose magnetic resonance imaging revealed a large heterogeneous enhancing dorsally exophytic lower brainstem mass. Examination revealed extreme cachexia, gaze-evoked nystagmus, dysphagia, dysarthria, bilateral dysmetria, and global weakness without ambulation. The protracted history and neuroimaging features were most suggestive of a low grade glioma. However, pathology revealed a hypercellular tumor with large hyperchromatic nucleoli and loss of INI-1 staining on immunohistochemistry consistent with a diagnosis of an ATRT. The child died shortly after surgery due to complications from his brainstem infiltrative disease. This case illustrates the diverse presentation of ATRT in childhood that can clinically and radiographically mimic that of low grade glioma.

Early Treatment Response of a Rare Papillary Tumor of the Pineal Region after Primary Proton-Beam Therapy using the Raster-Scanning Technique at HIT

Pineocytomas are rare intracranial tumors occurring in the pineal gland region. The curative therapy of choice is gross total resection, which cannot be performed in all patients because of the frequent eloquent location of these tumors. Percutaneous fractionated radiotherapy is an alternative treatment approach that may result in high local control rates. Nevertheless, our knowledge of this tumor entity is limited and based on retrospective case series only. We present a patient with a papillary tumor of the pineal region who was treated with highly conformal proton-beam therapy at the Heidelberg Ion Therapy Center (HIT) using the raster-scanning technique.

Treatment of choroid plexus tumors: a 20-year single institutional experience

Choroid plexus tumors (CPTs) are rare intracranial neoplasms that constitute approximately 2%-5% of all pediatric brain tumors. Most of these tumors present with severe hydrocephalus. The optimal perioperative management and oncological care remain a matter of debate. The authors present the epidemiological and clinical features of CPTs from a 20-year single-institutional experience. A total of 39 consecutive patients with pathologically proven CPTs (31 choroid plexus papillomas [CPPs] and 8 choroid plexus carcinomas [CPCs]) were included in this series. Patient demographics, clinical presentation, comorbidities, indications for surgery, radiological studies, tumor location, and all operative variables were reviewed for each case. Multivariate regression analysis was performed to identify independent predictors of tumor recurrence and survival. The overall mean age (± SD) was 13.13 ± 19.59 years (15.27 ± 21.10 years in the CPP group and 3.66 ± 3.59 years in the CPC group). Hydrocephalus was noted at presentation in 34% of patients. The most common presenting symptoms were headache (32%) and nausea/vomiting (26%). Gross-total resection (GTR) was achieved in 86% of CPPs and in 71% of CPCs (p = 0.57). There was 100% survival in patients with CPPs observed at the 5- and 10-year follow-up and 71% survival in patients with CPCs at the 5-year follow-up. In a multivariate regression analysis, a diagnosis of papilloma, preoperative vision changes, or hydrocephalus; right ventricle tumor location; and GTR were all independently associated with a decreased likelihood of tumor recurrence at last follow-up. The authors' study suggests that patients with CPCs are more likely to experience local recurrence and metastasis; hence, GTR with chemotherapy and radiotherapy, particularly for CPCs, is pivotal in preventing recurrence and prolonging survival. While GTR was important for local control following resection of CPPs, it had a minimal effect on prolonging survival in this patient cohort.

Primary intracranial epithelioid hemangioendothelioma: a low-proliferation tumor exhibiting clinically malignant behavior

Epithelioid hemangioendothelioma is an extremely rare intracranial tumor and is regarded as a low-proliferation tumor. We present two cases of primary intracranial epithelioid hemangioendothelioma and give an overview of the English literature pertaining to this disease. We described two new cases of primary intracranial epithelioid hemangioendothelioma and performed a search of MEDLINE (PubMed) using the words "epithelioid hemangioendothelioma". Only cases in the English language that were intracranially located and contained clinical information pertinent to the analysis were included. The tumor in case 1 originated from the right temporal bone and invaded the surrounding cranium, dura and temple muscles. The tumor in case 2 was located in the petroclival bone and had also invaded the surrounding cranium. Both tumors were well vascularized. The tumors were totally (case 1) or subtotally (case 2) removed with moderate blood loss. A total of 36 cases of intracranial epithelioid hemangioendothelioma were found in the literature. The tumor was typically diagnosed in young adults and infants. There was no sex predominance in adult patients, while in children, males were more frequently affected (M/F ratio, 3.5:1). Surgical removal was the main therapeutic protocol, and adjuvant therapy included radiotherapy or chemotherapy. Including the 2 patients presented here, a total of 38 patients were analyzed: 32 % showed local invasion, 24 % suffered a recurrence, 15 % had metastases, and the mortality rate was 15 %. Intracranial epithelioid hemangioendothelioma is a rare, low-proliferation tumor, but it exhibits some clinically malignant behaviors, such as local invasion, recurrence and metastasis. Total resection is mandatory where possible, and radiotherapy and/or chemotherapy are otherwise required. Preoperative feeding-artery embolization is recommended.

Hypoglossal Schwannomas: Single Institutional Experience of 14 Cases

Background: Hypoglossal schwannomas are very rare intracranial neoplasms. Microsurgical resection with the goal for cure is the aim of management, but is associated with a high rate of postoperative morbidity.

The transylvian trans-insular approach to lateral thalamic lesions

Thalamic tumors are rare intracranial tumors. The most common approaches to the thalamus have been directed through the ventricular system, which surrounds it. The transsylvian trans-insular approach to the lateral thalamus has been infrequently described probably because of the vulnerability of the internal capsule, which skirts this part of the thalamus. To describe the approach emphasizing its anatomical basis and also to evaluate its safety and efficacy. Retrospective study conducted at a tertiary hospital. Patient population included all the patients who underwent surgery for the lesions in lateral thalamus using the transylvian trans-insular approach between 2005 and 2011. A trephine craniotomy was made, centered over posterior sylvian fissure and the surgical corridor was developed through the insular cortex. During the study period 10 patients (7 tumors and inflammatory lesions and 3 hypertensive bleeds) were treated using this approach. One peri-operative mortality was noted. In patients with lesions other than hypertensive thalamic hemorrhage, there was no postoperative worsening of neurological deficit as comparative to preoperative deficits. Total excision/evacuation of the lesion could be accomplished in all the patients. The transylvian trans-insular approach is safe, effective, anatomical procedure, and can be performed at a peripheral center without the need for navigation and intra-operative monitoring.

Intracerebellar hemispheres schwannoma: case report

Brain parenchyma schwannoma is a rare intracranial tumor, and especially rare in cerebellar hemisphere. In this report, a case of cerebellar schwannoma in a 52-year-old woman, was studied by computed tomography (CT), magnetic resonance image (MRI) and PET-CT. This tumor was totally removed by surgery. The histological diagnosis of schwannoma was confirmed by histological, HE and immunohistological staining examination (positivity for the S-100 protein and vimentin, and partly positivity for P53 (70 %+), and negative for GFAP). The patient has been followed-up for more than one year, and she lives in good condition and brain MRI shows no recurrence. Surgery is the most effective treatment for cerebellar schwannoma.

A clinicopathological and neuroradiological study of paediatric meningioma from a single centre

Paediatric meningiomas are rare intracranial neoplasms representing 0.4% to 4.1% of tumours in paediatric patients and 1.5% to 1.8% of all intracranial meningiomas. The goal of this study was to determine the epidemiology, clinical presentation, radiological features, morphological spectrum and treatment outcome of paediatric meningiomas. All evaluable reports of meningioma in children from 1 January 1999 to 31 December 2009 were retrieved from the database of our Department of Pathology and were assessed retrospectively. This study describes 12 patients (nine males, three females; age range, 4–18 years; mean, 12.8 years). Their age and sex distribution, presenting symptoms, neurological findings, location, neuroradiological and histopathological findings were reviewed and the results were compared with published reports. Atypical and malignant meningiomas seem to be more common in childhood and adolescence than in adulthood.

Bifocal Intracranial Germinoma: A Retrospective Analysis of Treatment Outcomes in 20 Patients and Review of the Literature

Bifocal germinoma (BFG) is a rare intracranial neoplasm for which the choice of radiation therapy (RT) field is controversial. Some believe that BFG represents disseminated disease requiring craniospinal irradiation (CSI), whereas others believe that BFG represents localized disease and advocate for more limited fields. We analyzed 20 BFG patients at our institutions with classic bifocal lesions (pineal gland and suprasellar region). In addition, we identified 60 BFG patients from the literature. The RT fields, use of chemotherapy and extent of disease were recorded and analyzed for each patient. There were 55 patients with bifocal lesions only (Group I), and 25 with bifocal lesions plus ventricular and/or CSF positive disease (Group II). The 5-year progression-free survival was 95% for Group I and 80% for Group II. In Group I, there were no failures in patients receiving CSI (n= 11), two spinal failures in those treated with more limited RT fields without chemotherapy (n= 17), and one spinal failure with chemotherapy (n= 23). In Group II, there were no failures in patients receiving CSI (n= 11), but four spinal failures were observed in patients receiving more limited RT fields with chemotherapy (n= 13); 1 patient who received whole-brain RT without chemotherapy experienced failure in the spine and brain. CSI is associated with excellent PFS in BFG. In Group I BFG patients, omission of spinal irradiation appears to be a reasonable approach, especially when chemotherapy is used. Patients with Group II BFG are best treated with CSI.

Pediatric meningioma: current approaches and future direction

With improvement in leukemia therapy, central nervous system (CNS) tumors are the leading cause of cancer mortality in children and the most expensive of all human neoplasms to treat. Meningiomas are rare intracranial tumors in childhood and adolescence arising from arachnoid cell clonal outgrowth in the meninges. There have been no collaborative prospective therapeutic trials for pediatric meningioma because of its rarity, and the best evidence for management comes from retrospective case analyses and extrapolation from the treatment of adult meningioma. However this may not be ideal, because the underlying biology of adult and pediatric meningiomas seems to be different, as is the case for other CNS tumors. In addition, treatment of pediatric brain tumors requires consideration of long-term quality of life. This review reflects on what is currently known about pediatric meningiomas and opportunities for future directions.