Brain stem gliomas
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Shanshan Cen, M.D. [2]Sujit Routray, M.D. [3]
Synonyms and keywords: Brainstem gliomata; brain stem glioma; brainstem glioma; brain stem gliomas; diffuse brainstem glioma; diffuse brainstem gliomas; diffuse brain stem glioma; diffuse brain stem gliomas; focal brainstem glioma; focal brain stem glioma; focal brainstem gliomas; focal brain stem gliomas; dorsal exophytic gliomas; dorsal exophytic glioma; cervicomedullary glioma; cervicomedullary gliomas
Overview
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Brainstem gliomas are tumors that occur in the region of the brain referred to as the brain stem, which is the area between the aqueduct of Sylvius and the fourth ventricle. In the era preceding modern imaging, all brainstem gliomas were regarded as a solitary pathological entity with poor prognosis. In the late 1960s, Matson suggested that all brainstem tumors were malignant and were deemed inoperable regardless of their histopathological characteristics or location.This assertion was questioned shortly thereafter by Pool, who was one of the first to report tumor resection in the brainstem, which in the case described was inside the aqueduct. In 1980, Hoffman et. described the dorsally exophytic group of brainstem gliomas as a distinct subgroup, and reported that these lesions were surgically curable with aggressive resection. Brainstem gliomas may be classified into four subtypes: diffuse, focal, dorsal exophytic, and cervicomedullary. Brainstem gliomas may arise from glial cells of the brainstem, a majority of these tumors are found in the pons. Other areas include tectal area and medulla. Genetic mutations in histone genes, activin A receptor gene, tyrosine kinase mutations and TP53 mutations have been implicated in the development of brain stem gliomas. Brainstem gliomas must be differentiated from other brain tumors presenting as headache, visual disturbances, dizziness, paralysis, paresis, pyramidal signs, nausea, vomiting and weight loss. The differentials include medulloblastomas, craniopharyngiomas, ependymoma, pinealoma, meningioma, hemangioblastoma, tuberculous infection, toxoplasmosis and brain metastases. The incidence of brainstem gliomas is estimated to be 0.05 – 0.1 cases per 100,000 individuals in USA. A bimodal distribution by age is noted with peak incidences rates in children and older adults. Brainstem gliomas are commonly found in individuals suffering from Li-Fraumeni syndrome, neurofibromatosis type 1 (NF1), nevoid basal cell carcinoma syndrome, tuberous sclerosis and Turcot syndrome. If left untreated, patients with brainstem gliomas may progress to develop increased intracranial pressure and cerebral herniation. Common complications of brainstem gliomas include loss of motor and sensory functions and loss of regulation of basic body functions like blood pressure, swallowing and respiration. Prognosis is generally good for dorsal exophytic and cervicomedullary brainstem gliomas, and diffuse subtype has the worst prognosis with treatment. The hallmark of brainstem gliomas is the classic triad of ataxia, long tract signs and cranial nerve palsies. Common symptoms include hemiparesis or hemiplegia, unilateral facial nerve palsy, ataxia, vision defect, hearing loss, morning headache or headache that goes away after vomiting, nausea and vomiting, drowsiness, fatigue, and behavioral changes. Less common symptoms include seizure, trouble learning in school, and deterioration of handwriting and speech. Common physical examination findings of diffuse brainstem gliomas include cranial nerve deficit, pyramidal tract signs, and ataxia whereas that of focal gliomas are diplopia, ophthalmoplegia, Parinaud syndrome, loss of accommodation, and light-near dissociation. The presence of facial sensory loss, dysphagia, and dysphonia on physical examination is diagnostic of cervicomedullary brainstem gliomas. On MRI brain, diffuse brainstem glioma is characterized by decreased intensity on T1, heterogenously increased on T2. Focal brainstem glioma is characterized by iso- to hypointense to grey matter on T1, and hyperintense to grey matter on T2. The optimal therapy for brainstem gliomas depends on the subtype and whether it is newly diagnosed or a recurrent tumor. Patients with diffuse brainstem gliomas are treated with radiotherapy and chemotherapy, whereas patients with focal brainstem gliomas are treated with surgical resection with or without radiation therapy and chemotherapy. Surgical intervention is not recommended for the management of diffuse brainstem gliomas. Surgery with or without radiotherapy and chemotherapy is the mainstay of treatment for focal, dorsal exophytic and cervicomedullary brainstem gliomas.
Historical prospective
In the era preceding modern imaging, all brainstem gliomas were regarded as a solitary pathological entity with poor prognosis. In the late 1960s, Matson suggested that all brainstem tumors were malignant and were deemed inoperable regardless of their histopathological characteristics or location.This assertion was questioned shortly thereafter by Pool, who was one of the first to report tumor resection in the brainstem, which in the case described was inside the aqueduct. In 1980, Hoffman et. described the dorsally exophytic group of brainstem gliomas as a distinct subgroup, and reported that these lesions were surgically curable with aggressive resection. Over the past 3 decades, the treatment of brainstem gliomas has notably progressed as a result of the gradual advancements in microsurgical techniques, sophisticated imaging technology and, most importantly, the availability of MRI. These modalities have revealed that brainstem gliomas are a heterogeneous group of tumors.
Classification
Brainstem gliomas may be classified into four subtypes: diffuse, focal, dorsal exophytic, and cervicomedullary.
Pathophysiology
Brainstem gliomas may arise from glial cells of the brainstem, a majority of these tumors are found in the pons. Other areas include tectal area and medulla. Genetic mutations in histone genes, activin A receptor gene, tyrosine kinase mutations and TP53 mutations have been implicated in the development of brain stem gliomas. On gross pathology, brainstem gliomas can be classified into four subtypes: diffuse, focal, dorsal exophytic and cervicomedullary. Each of the four subtypes has its distinct microscopic pathology.
Differentiating Brain Stem Gliomas From Other Diseases
Brainstem gliomas must be differentiated from other brain tumors presenting as headache, visual disturbances, dizziness, paralysis, paresis, pyramidal signs, nausea, vomiting and weight loss. The differentials include medulloblastomas, craniopharyngiomas, ependymoma, pinealoma, meningioma, hemangioblastoma, tuberculous infection, toxoplasmosis and brain metastases.
Epidemiology and Demographics
The incidence of brainstem gliomas is estimated to be 0.05 – 0.1 cases per 100,000 individuals in USA. A bimodal distribution by age is noted with peak incidences rates in children and older adults. The prevalence and incidence of brainstem gliomas do not vary by either race or gender
Risk Factors
Brainstem gliomas are commonly found in individuals suffering from Li-Fraumeni syndrome, neurofibromatosis type 1 (NF1), nevoid basal cell carcinoma syndrome, tuberous sclerosis and Turcot syndrome.
Screening
Screening for brainstem gliomas is not recommended.
Natural history, complications and prognosis
If left untreated, patients with brainstem gliomas may progress to develop increased intracranial pressure and cerebral herniation. Common complications of brainstem gliomas include loss of motor and sensory functions and loss of regulation of basic body functions like blood pressure, swallowing and respiration. Prognosis is generally good for dorsal exophytic and cervicomedullary brainstem gliomas, and diffuse subtype has the worst prognosis with treatment.
Diagnosis
History and symptoms
The hallmark of brainstem gliomas is the classic triad of ataxia, long tract signs and cranial nerve palsies. Common symptoms include hemiparesis or hemiplegia, unilateral facial nerve palsy, ataxia, vision defect, hearing loss, morning headache or headache that goes away after vomiting, nausea and vomiting, drowsiness, fatigue, and behavioral changes. Less common symptoms include seizure, trouble learning in school, and deterioration of handwriting and speech.
Physical exam
Common physical examination findings of diffuse brainstem gliomas include cranial nerve deficit, pyramidal tract signs, and ataxia whereas that of focal gliomas are diplopia, ophthalmoplegia, Parinaud syndrome, loss of accommodation, and light-near dissociation. The presence of facial sensory loss, dysphagia, and dysphonia on physical examination is diagnostic of cervicomedullary brainstem gliomas.
Laboratory findings
There are no diagnostic lab findings associated with brainstem gliomas.
X-ray
There are no x-ray findings associated with brainstem gliomas.
CT scan
Head CT scan may be helpful in the diagnosis of brainstem gliomas. CT scan findings of brainstem gliomas vary according to the different subtypes.
MRI
On MRI brain, diffuse brainstem glioma is characterized by decreased intensity on T1, heterogenously increased on T2. Focal brainstem glioma is characterized by iso- to hypointense to grey matter on T1, and hyperintense to grey matter on T2.
Echocardiography or ultrasound
There are no echocardiography or ultrasound findings associated with brainstem gliomas.
Other imaging findings
On angiography, brainstem gliomas is characterized by anterior displacement of basilar artery.
Other diagnostic studies
Biopsy may be indicated for brain stem tumors that are not diffuse and intrinsic or when there is diagnostic uncertainty based on imaging findings.
Treatment
Medical therapy
The optimal therapy for brainstem gliomas depends on the subtype and whether it is newly diagnosed or a recurrent tumor. Patients with diffuse brainstem gliomas are treated with radiotherapy and chemotherapy, whereas patients with focal brainstem gliomas are treated with surgical resection with or without radiation therapy and chemotherapy.
Surgery
Surgical intervention is not recommended for the management of diffuse brainstem gliomas. Surgery with or without radiotherapy and chemotherapy is the mainstay of treatment for focal, dorsal exophytic and cervicomedullary brainstem gliomas.
References
Historical Perspective
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Umair Hamid, M.D[2]
Overview
In the era preceding modern imaging, all brainstem gliomas were regarded as a solitary pathological entity with poor prognosis.[1] In the late 1960s, Matson suggested that all brainstem tumors were malignant and were deemed inoperable regardless of their histopathological characteristics or location. [2]This assertion was questioned shortly thereafter by Pool, who was one of the first to report tumor resection in the brainstem, which in the case described was inside the aqueduct. [3] In 1980, Hoffman et. al [4] described the dorsally exophytic group of brainstem gliomas as a distinct subgroup, and reported that these lesions were surgically curable with aggressive resection. Over the past 3 decades, the treatment of brainstem gliomas has notably progressed as a result of the gradual advancements in microsurgical techniques, sophisticated imaging technology and, most importantly, the availability of MRI. These modalities have revealed that brainstem gliomas are a heterogeneous group of tumors. [5]
References
- ↑ Alaqeel AM, Sabbagh AJ (2014). “Pediatric brainstem tumors. Classifications, investigations, and growth patterns”. Neurosciences (Riyadh). 19 (2): 93–9. PMID 24739404.
- ↑ Matson DD. Tumors of the posterior fossa. In: Matson DD, Ingraham FD, editors. Neurosurgery of Infancy and Childhood.2nd ed. Springfield (IL): Charles C. Thomas; 1969. p. 469-477
- ↑ Pool JL (1968). “Gliomas in the region of the brain stem”. J Neurosurg. 29 (2): 164–7. doi:10.3171/jns.1968.29.2.0164. PMID 5673314.
- ↑ Hoffman HJ, Becker L, Craven MA (1980). “A clinically and pathologically distinct group of benign brain stem gliomas”. Neurosurgery. 7 (3): 243–8. doi:10.1227/00006123-198009000-00007. PMID 7207742.
- ↑ Epstein F, Wisoff JH (1988). “Intrinsic brainstem tumors in childhood: surgical indications”. J Neurooncol. 6 (4): 309–17. PMID 3221258.
Classification
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]
Overview
Brainstem gliomas may be classified into four subtypes: diffuse, focal, dorsal exophytic, and cervicomedullary.
Classification
| Brainstem gliomas | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Diffuse brainstem glioma | Focal brainstem glioma | exophytic | Cervicomedullary | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Brainstem gliomas may be classified into 4 subtypes:
- Diffuse brainstem glioma
- Focal brainstem glioma
- Tectal plate glioma
- Other focal gliomas
- (Dorsally) exophytic
- Cervicomedullary
- Probably an artificial group made up of the downward extension of true brainstem gliomas or upward extension of upper cervical cord intramedullary spinal cord tumors.
| Brainstem gliomas | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Diffuse brainstem glioma | Focal brainstem glioma | exophytic | Cervicomedullary | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
References
Pathophysiology
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]Syed Hassan A. Kazmi BSc, MD [3]
Overview
Brainstem gliomas may arise from glial cells of the brainstem, a majority of these tumors are found in the pons. Other areas include tectal area and medulla. Genetic mutations in histone genes, activin A receptor gene, tyrosine kinase mutations and TP53 mutations have been implicated in the development of brain stem gliomas. On gross pathology, brainstem gliomas can be classified into four subtypes: diffuse, focal, dorsal exophytic and cervicomedullary. Each of the four subtypes has its distinct microscopic pathology.
Pathophysiology
Cytogenetic Characteristics of Diffuse Intrinsic Pontine Gliomas (DIPGs)
Diffuse brainstem gliomas also known as diffuse intrinsic pontine gliomas (DIPGs) as the name suggests are predominantly present in the pons. DIPGs exhibit genotypic modifications that differ from other high grade gliomas, both adult and pediatric. The gene expression profile of DIPG also varies from that of non–brain stem pediatric high-grade gliomas, further supporting a distinctive biology for this subset of pediatric gliomas. DIPG patirents may have the following genomic abnormalities:[1]
- Histone H3 genes: Approximately 80% of DIPG tumors have a mutation in a specific amino acid in the histone H3.1 (H3F3A) or H3.3 (HIST1H3B) genes. These same mutations are observed in pediatric high-grade gliomas at other midline locations but are uncommon in cortical pediatric high-grade gliomas and in adult high-grade gliomas.[2]
- Activin A receptor, type I (ACVR1) gene: Approximately 20% of DIPG cases have activating mutations in the ACVR1 gene, with most occurring concurrently with H3.3 mutations. Germline mutations in ACVR1 cause the autosomal dominant syndrome fibrodysplasia ossificans progressiva (FOP), although there is no cancer predisposition in FOP.
- Receptor tyrosine kinase amplification: PDGFRA amplification occurs in approximately 30% of cases, with lower rates of amplification observed for some other receptor tyrosine kinases (e.g., MET and IGF1R).
- TP53 deletion: DIPG tumors commonly show deletion of the TP53 gene on chromosome 17p. Additionally, TP53 is commonly mutated in DIPG tumors, particularly those with histone H3 gene mutations. Aneuploidy is commonly observed in cases with TP53 mutations.
Gross pathology
Brain stem gliomas may occur in the pons, midbrain, tectum, dorsum of the medulla at the cervicomedullary junction, or in multiple regions of the brain stem. As a general rule, mesencephalic tumors tend to be of a lower grade than those in the pons and medulla.
- Pontine[3]
- Most common location
- Classic location for the childhood ‘brainstem glioma’ which tends to refer to a diffuse pontine glioma
- Focal dorsally exophytic brainstem glioma is an uncommon variant accounting for only 10% of pontine tumours, and has a much better prognosis, as it usually represents a pilocytic astrocytoma
- Overall survival of pontine gliomas is 10% at 5 years
- Mesencephalic[4]
- Includes diffuse, focal, exophytic and tectal variants.
- Focal brainstem gliomas are more common here than elsewhere in the brainstem
- Tectal plate gliomas are typically indolent
- Medullary
- Least common location
- Includes focal dorsally exophytic, focal, diffuse and cervicomedullary junction variants
- Cervicomedullary junction tumours usually represent upper cervical tumours extending superiorly
- Most common location for NF1-associated tumors
Microscopic pathology
The brain stem gliomas are classified into 4 subtypes, and each has its distinct histological features:[5]
- Diffuse brainstem gliomas
- Also called as diffuse intrinsic pontine gliomas (DIPGs)
- Usually fibrillary astrocytomas
- WHO grades II-IV
- Grade does not impact on prognosis, and thus biopsy is usually not necessary
- 75% of brain stem gliomas
- Focal glioma
- Fibrillary astrocytoma (grade II): most common histology
- Pilocytic astrocytoma
- Ganglioglioma
- (Dorsally) exophytic glioma
- NF1-associated brainstem glioma[6]
- Seen in up to 9% of neurofibromatosis-1 (NF-1) patients
- Most frequently seen in the medulla
- Appears similar to a sporadic focal brainstem glioma but has an even better prognosis, with little if any progression
References
- ↑ Pathophysiology of brainstem gliomas. NIH National cancer institute. http://www.cancer.gov/types/brain/hp/child-glioma-treatment-pdq#section/_35
- ↑ Uekawa K, Nakamura H, Shinojima N, Takezaki T, Yano S, Kuratsu JI (April 2016). “Adult Diffuse Astrocytoma in the Medulla Oblongata: Molecular Biological Analyses Including H3F3A Mutation of Histone H3.3”. NMC Case Rep J. 3 (2): 29–33. doi:10.2176/nmccrj.cr.2015-0012. PMC 5386147. PMID 28663993.
- ↑ Warren KE (2012). “Diffuse intrinsic pontine glioma: poised for progress”. Front Oncol. 2: 205. doi:10.3389/fonc.2012.00205. PMC 3531714. PMID 23293772.
- ↑ Boydston WR, Sanford RA, Muhlbauer MS, Kun LE, Kirk E, Dohan FC, Schweitzer JB (1991). “Gliomas of the tectum and periaqueductal region of the mesencephalon”. Pediatr Neurosurg. 17 (5): 234–8. doi:10.1159/000120603. PMID 1822688.
- ↑ Pathophysiology of Brainstem gliomas. Dr Yuranga Weerakkody and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/brainstem-glioma
- ↑ Uekawa K, Nakamura H, Shinojima N, Takezaki T, Yano S, Kuratsu JI (April 2016). “Adult Diffuse Astrocytoma in the Medulla Oblongata: Molecular Biological Analyses Including H3F3A Mutation of Histone H3.3”. NMC Case Rep J. 3 (2): 29–33. doi:10.2176/nmccrj.cr.2015-0012. PMC 5386147. PMID 28663993.
Causes
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
The cause of brain stem gliomas has not been identified.
Causes
The cause of brain stem gliomas has not been identified.
References
Differentiating Brain Stem Gliomas from other Diseases
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]
Overview
Brainstem gliomas must be differentiated from other brain tumors presenting as headache, visual disturbances, dizziness, paralysis, paresis, pyramidal signs, nausea, vomiting and weight loss. The differentials include medulloblastomas, craniopharyngiomas, ependymoma, pinealoma, meningioma, hemangioblastoma, tuberculous infection, toxoplasmosis and brain metastases.
Differentiating brain stem gliomas from other diseases
Brain stem gliomas must be differentiated from:[1]
| Diseases | Clinical manifestations | Para-clinical findings | Gold standard |
Additional findings | ||||||
|---|---|---|---|---|---|---|---|---|---|---|
| Symptoms | Physical examination | |||||||||
| Lab Findings | MRI | Immunohistopathology | ||||||||
| Head- ache |
Seizure | Visual disturbance | Constitutional | Focal neurological deficit | ||||||
| Childhood primary brain tumors | ||||||||||
| Craniopharyngioma [2][3][4][5] |
+ | +/− | + Bitemporal hemianopia | − | + |
|
|
|
| |
| Pilocytic astrocytoma [6][7][8] |
+ | +/− | +/− | − | + | − |
|
|
| |
| Medulloblastoma [9][10][11] |
+ | +/− | +/− | − | + | − |
|
|
| |
| Ependymoma [12][5] |
+ | +/− | +/− | − | + | − |
|
|
| |
| Pinealoma [13][14][15] |
+ | +/− | +/− | − | + vertical gaze palsy |
|
|
|
| |
| Adult primary brain tumors | ||||||||||
| Glioblastoma multiforme [16][17][5] |
+ | +/− | +/− | − | + | − |
|
|
| |
| Oligodendroglioma [18][19][20] |
+ | + | +/− | − | + | − |
|
|
| |
| Meningioma [21][22][23] |
+ | +/− | +/− | − | + | − |
|
|
| |
| Hemangioblastoma [24][25][26][27] |
+ | +/− | +/− | − | + | − |
|
| ||
| Pituitary adenoma [28][29][5] |
− | − | + Bitemporal hemianopia | − | − |
|
|
|
| |
| Schwannoma [30][31][32][33] |
− | − | − | − | + | − |
|
|
| |
| Primary CNS lymphoma [34][35] |
+ | +/− | +/− | − | + | − |
|
|
| |
| Vascular | ||||||||||
| AV malformation [36][37][5] |
+ | + | +/− | − | +/− | − |
|
| ||
| Brain aneurysm [38][39][40][41][42] |
+ | +/− | +/− | − | +/− | − |
|
|
|
|
| Infectious | ||||||||||
| Bacterial brain abscess [43][44] |
+ | +/− | +/− | + | + |
|
|
|
|
|
| Tuberculosis [45][5][46] |
+ | +/− | +/− | + | + |
|
|
|
|
|
| Toxoplasmosis [47][48] |
+ | +/− | +/− | − | + |
|
|
|
|
|
| Hydatid cyst [49][5] |
+ | +/− | +/− | +/− | + |
|
|
|
|
|
| CNS cryptococcosis [50] |
+ | +/− | +/− | + | + |
|
|
|
|
|
| CNS aspergillosis [51] |
+ | +/− | +/− | + | + |
|
|
|
|
|
| Other | ||||||||||
| Brain metastasis [52][5] |
+ | +/− | +/− | + | + | − |
|
|
|
|
Reference
- ↑ D.Dx of Brainstem gliomas. Dr Yuranga Weerakkody and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/brainstem-glioma
- ↑ Brunel H, Raybaud C, Peretti-Viton P, Lena G, Girard N, Paz-Paredes A, Levrier O, Farnarier P, Manera L, Choux M (September 2002). “[Craniopharyngioma in children: MRI study of 43 cases]”. Neurochirurgie (in French). 48 (4): 309–18. PMID 12407316.
- ↑ Prabhu, Vikram C.; Brown, Henry G. (2005). “The pathogenesis of craniopharyngiomas”. Child’s Nervous System. 21 (8–9): 622–627. doi:10.1007/s00381-005-1190-9. ISSN 0256-7040.
- ↑ Kennedy HB, Smith RJ (December 1975). “Eye signs in craniopharyngioma”. Br J Ophthalmol. 59 (12): 689–95. PMC 1017436. PMID 766825.
- ↑ 5.0 5.1 5.2 5.3 5.4 5.5 5.6 5.7 Mattle, Heinrich (2017). Fundamentals of neurology : an illustrated guide. Stuttgart New York: Thieme. ISBN 9783131364524.
- ↑ Sathornsumetee S, Rich JN, Reardon DA (November 2007). “Diagnosis and treatment of high-grade astrocytoma”. Neurol Clin. 25 (4): 1111–39, x. doi:10.1016/j.ncl.2007.07.004. PMID 17964028.
- ↑ Pedersen CL, Romner B (January 2013). “Current treatment of low grade astrocytoma: a review”. Clin Neurol Neurosurg. 115 (1): 1–8. doi:10.1016/j.clineuro.2012.07.002. PMID 22819718.
- ↑ Mattle, Heinrich (2017). Fundamentals of neurology : an illustrated guide. Stuttgart New York: Thieme. ISBN 9783131364524.
- ↑ Dorwart, R H; Wara, W M; Norman, D; Levin, V A (1981). “Complete myelographic evaluation of spinal metastases from medulloblastoma”. Radiology. 139 (2): 403–408. doi:10.1148/radiology.139.2.7220886. ISSN 0033-8419.
- ↑ Fruehwald-Pallamar, Julia; Puchner, Stefan B.; Rossi, Andrea; Garre, Maria L.; Cama, Armando; Koelblinger, Claus; Osborn, Anne G.; Thurnher, Majda M. (2011). “Magnetic resonance imaging spectrum of medulloblastoma”. Neuroradiology. 53 (6): 387–396. doi:10.1007/s00234-010-0829-8. ISSN 0028-3940.
- ↑ Burger, P. C.; Grahmann, F. C.; Bliestle, A.; Kleihues, P. (1987). “Differentiation in the medulloblastoma”. Acta Neuropathologica. 73 (2): 115–123. doi:10.1007/BF00693776. ISSN 0001-6322.
- ↑ Yuh, E. L.; Barkovich, A. J.; Gupta, N. (2009). “Imaging of ependymomas: MRI and CT”. Child’s Nervous System. 25 (10): 1203–1213. doi:10.1007/s00381-009-0878-7. ISSN 0256-7040.
- ↑ Ahmed SR, Shalet SM, Price DA, Pearson D (September 1983). “Human chorionic gonadotrophin secreting pineal germinoma and precocious puberty”. Arch. Dis. Child. 58 (9): 743–5. PMID 6625640.
- ↑ Sano, Keiji (1976). “Pinealoma in Children”. Pediatric Neurosurgery. 2 (1): 67–72. doi:10.1159/000119602. ISSN 1016-2291.
- ↑ Baggenstoss, Archie H. (1939). “PINEALOMAS”. Archives of Neurology And Psychiatry. 41 (6): 1187. doi:10.1001/archneurpsyc.1939.02270180115011. ISSN 0096-6754.
- ↑ Sathornsumetee S, Rich JN, Reardon DA (November 2007). “Diagnosis and treatment of high-grade astrocytoma”. Neurol Clin. 25 (4): 1111–39, x. doi:10.1016/j.ncl.2007.07.004. PMID 17964028.
- ↑ Pedersen CL, Romner B (January 2013). “Current treatment of low grade astrocytoma: a review”. Clin Neurol Neurosurg. 115 (1): 1–8. doi:10.1016/j.clineuro.2012.07.002. PMID 22819718.
- ↑ Smits M (2016). “Imaging of oligodendroglioma”. Br J Radiol. 89 (1060): 20150857. doi:10.1259/bjr.20150857. PMC 4846213. PMID 26849038.
- ↑ Wesseling P, van den Bent M, Perry A (June 2015). “Oligodendroglioma: pathology, molecular mechanisms and markers”. Acta Neuropathol. 129 (6): 809–27. doi:10.1007/s00401-015-1424-1. PMC 4436696. PMID 25943885.
- ↑ Kerkhof M, Benit C, Duran-Pena A, Vecht CJ (2015). “Seizures in oligodendroglial tumors”. CNS Oncol. 4 (5): 347–56. doi:10.2217/cns.15.29. PMC 6082346. PMID 26478444.
- ↑ Zee CS, Chin T, Segall HD, Destian S, Ahmadi J (June 1992). “Magnetic resonance imaging of meningiomas”. Semin. Ultrasound CT MR. 13 (3): 154–69. PMID 1642904.
- ↑ Shibuya M (2015). “Pathology and molecular genetics of meningioma: recent advances”. Neurol. Med. Chir. (Tokyo). 55 (1): 14–27. doi:10.2176/nmc.ra.2014-0233. PMID 25744347.
- ↑ Begnami MD, Palau M, Rushing EJ, Santi M, Quezado M (September 2007). “Evaluation of NF2 gene deletion in sporadic schwannomas, meningiomas, and ependymomas by chromogenic in situ hybridization”. Hum. Pathol. 38 (9): 1345–50. doi:10.1016/j.humpath.2007.01.027. PMC 2094208. PMID 17509660.
- ↑ Lonser RR, Butman JA, Huntoon K, Asthagiri AR, Wu T, Bakhtian KD, Chew EY, Zhuang Z, Linehan WM, Oldfield EH (May 2014). “Prospective natural history study of central nervous system hemangioblastomas in von Hippel-Lindau disease”. J. Neurosurg. 120 (5): 1055–62. doi:10.3171/2014.1.JNS131431. PMC 4762041. PMID 24579662.
- ↑ Hussein MR (October 2007). “Central nervous system capillary haemangioblastoma: the pathologist’s viewpoint”. Int J Exp Pathol. 88 (5): 311–24. doi:10.1111/j.1365-2613.2007.00535.x. PMC 2517334. PMID 17877533.
- ↑ Lee SR, Sanches J, Mark AS, Dillon WP, Norman D, Newton TH (May 1989). “Posterior fossa hemangioblastomas: MR imaging”. Radiology. 171 (2): 463–8. doi:10.1148/radiology.171.2.2704812. PMID 2704812.
- ↑ Perks WH, Cross JN, Sivapragasam S, Johnson P (March 1976). “Supratentorial haemangioblastoma with polycythaemia”. J. Neurol. Neurosurg. Psychiatry. 39 (3): 218–20. PMID 945331.
- ↑ Kucharczyk W, Davis DO, Kelly WM, Sze G, Norman D, Newton TH (December 1986). “Pituitary adenomas: high-resolution MR imaging at 1.5 T”. Radiology. 161 (3): 761–5. doi:10.1148/radiology.161.3.3786729. PMID 3786729.
- ↑ Syro LV, Scheithauer BW, Kovacs K, Toledo RA, Londoño FJ, Ortiz LD, Rotondo F, Horvath E, Uribe H (2012). “Pituitary tumors in patients with MEN1 syndrome”. Clinics (Sao Paulo). 67 Suppl 1: 43–8. PMC 3328811. PMID 22584705.
- ↑ Donnelly, Martin J.; Daly, Carmel A.; Briggs, Robert J. S. (2007). “MR imaging features of an intracochlear acoustic schwannoma”. The Journal of Laryngology & Otology. 108 (12). doi:10.1017/S0022215100129056. ISSN 0022-2151.
- ↑ Feany MB, Anthony DC, Fletcher CD (May 1998). “Nerve sheath tumours with hybrid features of neurofibroma and schwannoma: a conceptual challenge”. Histopathology. 32 (5): 405–10. PMID 9639114.
- ↑ Chen H, Xue L, Wang H, Wang Z, Wu H (July 2017). “Differential NF2 Gene Status in Sporadic Vestibular Schwannomas and its Prognostic Impact on Tumour Growth Patterns”. Sci Rep. 7 (1): 5470. doi:10.1038/s41598-017-05769-0. PMID 28710469.
- ↑ Hardell, Lennart; Hansson Mild, Kjell; Sandström, Monica; Carlberg, Michael; Hallquist, Arne; Påhlson, Anneli (2003). “Vestibular Schwannoma, Tinnitus and Cellular Telephones”. Neuroepidemiology. 22 (2): 124–129. doi:10.1159/000068745. ISSN 0251-5350.
- ↑ Chinn RJ, Wilkinson ID, Hall-Craggs MA, Paley MN, Miller RF, Kendall BE, Newman SP, Harrison MJ (December 1995). “Toxoplasmosis and primary central nervous system lymphoma in HIV infection: diagnosis with MR spectroscopy”. Radiology. 197 (3): 649–54. doi:10.1148/radiology.197.3.7480733. PMID 7480733.
- ↑ Paulus, Werner (1999). “Classification, Pathogenesis and Molecular Pathology of Primary CNS Lymphomas”. Journal of Neuro-Oncology. 43 (3): 203–208. doi:10.1023/A:1006242116122. ISSN 0167-594X.
- ↑ Kucharczyk, W; Lemme-Pleghos, L; Uske, A; Brant-Zawadzki, M; Dooms, G; Norman, D (1985). “Intracranial vascular malformations: MR and CT imaging”. Radiology. 156 (2): 383–389. doi:10.1148/radiology.156.2.4011900. ISSN 0033-8419.
- ↑ Fleetwood, Ian G; Steinberg, Gary K (2002). “Arteriovenous malformations”. The Lancet. 359 (9309): 863–873. doi:10.1016/S0140-6736(02)07946-1. ISSN 0140-6736.
- ↑ Chapman, Arlene B.; Rubinstein, David; Hughes, Richard; Stears, John C.; Earnest, Michael P.; Johnson, Ann M.; Gabow, Patricia A.; Kaehny, William D. (1992). “Intracranial Aneurysms in Autosomal Dominant Polycystic Kidney Disease”. New England Journal of Medicine. 327 (13): 916–920. doi:10.1056/NEJM199209243271303. ISSN 0028-4793.
- ↑ Castori M, Voermans NC (October 2014). “Neurological manifestations of Ehlers-Danlos syndrome(s): A review”. Iran J Neurol. 13 (4): 190–208. PMC 4300794. PMID 25632331.
- ↑ Schievink, W. I.; Raissi, S. S.; Maya, M. M.; Velebir, A. (2010). “Screening for intracranial aneurysms in patients with bicuspid aortic valve”. Neurology. 74 (18): 1430–1433. doi:10.1212/WNL.0b013e3181dc1acf. ISSN 0028-3878.
- ↑ Germain DP (May 2017). “Pseudoxanthoma elasticum”. Orphanet J Rare Dis. 12 (1): 85. doi:10.1186/s13023-017-0639-8. PMC 5424392. PMID 28486967.
- ↑ Farahmand M, Farahangiz S, Yadollahi M (October 2013). “Diagnostic Accuracy of Magnetic Resonance Angiography for Detection of Intracranial Aneurysms in Patients with Acute Subarachnoid Hemorrhage; A Comparison to Digital Subtraction Angiography”. Bull Emerg Trauma. 1 (4): 147–51. PMC 4789449. PMID 27162847.
- ↑ Haimes, AB; Zimmerman, RD; Morgello, S; Weingarten, K; Becker, RD; Jennis, R; Deck, MD (1989). “MR imaging of brain abscesses”. American Journal of Roentgenology. 152 (5): 1073–1085. doi:10.2214/ajr.152.5.1073. ISSN 0361-803X.
- ↑ Brouwer, Matthijs C.; Tunkel, Allan R.; McKhann, Guy M.; van de Beek, Diederik (2014). “Brain Abscess”. New England Journal of Medicine. 371 (5): 447–456. doi:10.1056/NEJMra1301635. ISSN 0028-4793.
- ↑ Morgado, Carlos; Ruivo, Nuno (2005). “Imaging meningo-encephalic tuberculosis”. European Journal of Radiology. 55 (2): 188–192. doi:10.1016/j.ejrad.2005.04.017. ISSN 0720-048X.
- ↑ Be NA, Kim KS, Bishai WR, Jain SK (March 2009). “Pathogenesis of central nervous system tuberculosis”. Curr. Mol. Med. 9 (2): 94–9. PMC 4486069. PMID 19275620.
- ↑ Chinn RJ, Wilkinson ID, Hall-Craggs MA, Paley MN, Miller RF, Kendall BE, Newman SP, Harrison MJ (December 1995). “Toxoplasmosis and primary central nervous system lymphoma in HIV infection: diagnosis with MR spectroscopy”. Radiology. 197 (3): 649–54. doi:10.1148/radiology.197.3.7480733. PMID 7480733.
- ↑ Helton KJ, Maron G, Mamcarz E, Leventaki V, Patay Z, Sadighi Z (November 2016). “Unusual magnetic resonance imaging presentation of post-BMT cerebral toxoplasmosis masquerading as meningoencephalitis and ventriculitis”. Bone Marrow Transplant. 51 (11): 1533–1536. doi:10.1038/bmt.2016.168. PMID 27348541.
- ↑ Taslakian B, Darwish H (September 2016). “Intracranial hydatid cyst: imaging findings of a rare disease”. BMJ Case Rep. 2016. doi:10.1136/bcr-2016-216570. PMC 5030532. PMID 27620198.
- ↑ McCarthy M, Rosengart A, Schuetz AN, Kontoyiannis DP, Walsh TJ (July 2014). “Mold infections of the central nervous system”. N. Engl. J. Med. 371 (2): 150–60. doi:10.1056/NEJMra1216008. PMC 4840461. PMID 25006721.
- ↑ McCarthy M, Rosengart A, Schuetz AN, Kontoyiannis DP, Walsh TJ (July 2014). “Mold infections of the central nervous system”. N. Engl. J. Med. 371 (2): 150–60. doi:10.1056/NEJMra1216008. PMC 4840461. PMID 25006721.
- ↑ Pope WB (2018). “Brain metastases: neuroimaging”. Handb Clin Neurol. 149: 89–112. doi:10.1016/B978-0-12-811161-1.00007-4. PMC 6118134. PMID 29307364.
Epidemiology and Demographics
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]
Overview
The incidence of brainstem gliomas is estimated to be 0.05 – 0.1 cases per 100,000 individuals in USA. A bimodal distribution by age is noted with peak incidences rates in children and older adults. The prevalence and incidence of brainstem gliomas do not vary by either race or gender.
Epidemiology and Demographics
Incidence
The incidence of brainstem gliomas is 0.05 – 0.1 per 100,000 individuals per year.
Age
A bimodal distribution by age is noted with peak incidence rates in children (aged 7-9 years; most common) and among older adults (aged 30-40; rare) is seen.[1] The diffuse infiltrating type occur most often in school-age children where they are responsible for the greatest number of childhood deaths from primary brain tumors. [3]
Gender
There is no gender predilection to the development of brainstem glioma.[1]
Race
There is no racial predilection to the development of brainstem glioma.
References
- ↑ 1.0 1.1 Epidemiology of Brainstem gliomas. Dr Yuranga Weerakkody and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/brainstem-glioma
Risk Factors
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2] Syed Hassan A. Kazmi BSc, MD [3]
Overview
Brainstem gliomas are commonly found in individuals suffering from Li-Fraumeni syndrome, neurofibromatosis type 1 (NF1), nevoid basal cell carcinoma syndrome, tuberous sclerosis and Turcot syndrome.
Risk factors
Brainstem gliomas are commonly found in individuals suffering from:[1][2][3][4][5][6]
- Li-Fraumeni syndrome
- Neurofibromatosis type 1 (NF1)
- Nevoid basal cell carcinoma syndrome
- Tuberous sclerosis
- Turcot syndrome
References
- ↑ “Childhood Brain Stem Glioma Treatment (PDQ®)–Health Professional Version – National Cancer Institute”.
- ↑ Classification of Brainstem gliomas. Dr Yuranga Weerakkody and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/brainstem-glioma
- ↑ Eisele, Sylvia C.; Reardon, David A. (2016). “Adult brainstem gliomas”. Cancer. 122 (18): 2799–2809. doi:10.1002/cncr.29920. ISSN 0008-543X.
- ↑ Michaeli O, Tabori U (May 2018). “Pediatric High Grade Gliomas in the Context of Cancer Predisposition Syndromes”. J Korean Neurosurg Soc. 61 (3): 319–332. doi:10.3340/jkns.2018.0031. PMC 5957320. PMID 29742882.
- ↑ Dipro S, Al-Otaibi F, Alzahrani A, Ulhaq A, Al Shail E (2012). “Turcot syndrome: a synchronous clinical presentation of glioblastoma multiforme and adenocarcinoma of the colon”. Case Rep Oncol Med. 2012: 720273. doi:10.1155/2012/720273. PMC 3479943. PMID 23119205.
- ↑ “pubs.rsna.org”.
Screening
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Screening for brainstem gliomas is not recommended.
Screening
Screening for brainstem gliomas is not recommended.
References
Natural History, Complications and Prognosis
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]
Overview
If left untreated, patients with brainstem gliomas may progress to develop increased intracranial pressure and cerebral herniation. Common complications of brainstem gliomas include loss of motor and sensory functions and loss of regulation of basic body functions like blood pressure, swallowing and respiration. Prognosis is generally good for dorsal exophytic and cervicomedullary brainstem gliomas, and diffuse subtype has the worst prognosis with treatment.
Natural history
- If left untreated, patients with brainstem gliomas may progress to develop increased intracranial pressure and cerebral herniation.
- Radiotherapy-induced neoplasms tend to be more aggressive in their natural history than their de novo counterparts.
- The duration of symptoms is usually much shorter in diffuse gliomas, in which the history is typically very short (a few days).
- FBSG usually present with greater than 3 months of symptoms, while DIPG are usually diagnosed within 3 months of symptom onset.[1]
- Diffuse gliomas are associated with multiple cranial nerve palsies.
- Clinical manifestations of brainstem glioma depend on the following:[2]
- Location of the tumor
- Size of the tumor
- Growth rate of the tumor
- Patient’s age
Complications
Complications of brainstem gliomas include the following:
- Altered blood pressure
- Hypopnea
- Dysphagia
- Loss of motor and sensory functions
Sudden death can result from increased intracranial pressure and subsequent cerebral herniation. This may be a consequence either of edema induced by the tumor or of hemorrhage into the neoplasm.
Prognosis
Factors associated with worse prognosis include the following:[3]
- The type of brain stem glioma
- Where the tumor is found in the brain and if it has spread within the brain stem
- Age of the child when diagnosed
- Children younger than 3 years may have a more favorable prognosis
- Whether or not the child has a condition called neurofibromatosis type 1
- Children with NF1 and brain stem gliomas may have a better prognosis than other patients who have intrinsic lesions
- Whether the tumor has been newly diagnosed or has recurred
As a general rule, dorsal exophytic tumors and cervicomedullary tumors tend to have a good prognosis with treatment, and diffuse type has the worst prognosis with treatment.[4] The median survival for children with diffuse brainstem glioma is less than 1 year. In contrast, focal brainstem gliomas have a markedly improved prognosis, with 5-year overall survival exceeding 90%. Adults tend to have a better prognosis than childhood brainstem gliomas.
- Diffuse brainstem glioma
- Terrible prognosis
- 90-100% patients die within 2 years of diagnosis
- Focal (tectal glioma)
- Excellent long term survival with CSF shunting (essentially benign lesions)
- Focal (other)
- Good long-term prognosis with surgery
- (Dorsally) exophytic tumors
- Good long-term prognosis with surgery
References
- ↑ Green AL, Kieran MW (2015). “Pediatric brainstem gliomas: new understanding leads to potential new treatments for two very different tumors”. Curr Oncol Rep. 17 (3): 436. doi:10.1007/s11912-014-0436-7. PMID 25702179.
- ↑ Symptoms of brainstem tumors. Cancer gov. http://www.cancer.gov/types/brain/patient/child-glioma-treatment-pdq#link/stoc_h2_2
- ↑ Prognosis of brainstem tumors. Cancer gov. http://www.cancer.gov/types/brain/patient/child-glioma-treatment-pdq#link/stoc_h2_2
- ↑ Prognosis of Brainstem gliomas. Dr Yuranga Weerakkody and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/brainstem-glioma
Diagnosis
Diagnosis
History and Symptoms | Physical Examination | Laboratory Findings | X Ray | CT | MRI | Echocardiography or Ultrasound | Other Imaging Findings | Other Diagnostic Studies
Treatment
Treatment
Medical Therapy | Surgery | Cost-Effectiveness of Therapy | Future or Investigational Therapies
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