Brain stem neoplasm comprises a very small fraction of all cranial tumors and gliomas are the predominant form. Depending on the location of the neoplasm, patients might present with various symptoms, including headaches, cranial nerve palsies, weakness of the limbs, gait disturbances, and visual deficits. The diagnosis is made through a comprehensive clinical assessment followed by imaging studies such as magnetic resonance imaging (MRI) and MR spectroscopy, whereas a biopsy can sometimes be used to confirm the exact type of tumor.
Presentation
Because a brain stem neoplasm is defined as a lesion occurring anywhere in the medulla oblongata, the pons, or in the midbrain [1], the clinical presentation of cases with this condition can significantly vary. These tumors seem to be more common in infants than in adults, with a mean onset at 6-7 years of age and a very poor prognosis [2]. In adults, the majority of cases are discovered in the fourth decade of life [1] [3] [4] [5]. A headache is regarded as one of the most common complaints, presumably as a consequence of growing intracranial pressure and hydrocephalus [6]. Other common findings include palsies of various cranial nerves (whose nuclei originate from the brainstem, such as the trigeminal, abducens, facial, and vestibulocochlear nerve), but the facial nerve is most frequently affected [1] [5] [6]. Myokymia, hemifacial spasms, or a long history of facial palsy is not uncommon in the affected population [1]. Deficits of gait and limb function, as well as diplopia, are important manifestations of a brain stem neoplasm [1] [5] [6]. Some studies emphasize that patients typically report with unilateral facial symptoms and contralateral limb deficits [6]. The prognosis is generally poor, with overall survival being about 12 and 32 months for the pediatric and adult gliomas, respectively [2] [3].
Entire Body System
- Surgical Procedure
This confirms that all or as much of the tumor as possible is removed, to ensure surgical success. It also reduces the time and number of procedures a patient has to endure. [dukehealth.org]
This procedure is done in an attempt to reduce the raised intracranial pressure and relieve the patient’s symptoms, to normalise the altered cerebral blood flow and to ‘relax’ the brain, which would assist the surgeon in the definitive surgical procedure [jpgmonline.com]
Biopsy, a tissue sample from the tumor taken through a needle during a simple surgical procedure. There may be other diagnostic tests that your doctor will discuss with you depending on your child's individual situation. [danafarberbostonchildrens.org]
However, in this location they are not curable due to the fact that large surgical procedures in the pons usually produce intolerable deficits. [dartmouth.edu]
If a tumor recurs, the doctor will evaluate it for a second surgical procedure, radiation, and/or chemotherapy. Gene expression studies are used to classify gliomas based on certain characteristics, or genetic profiles. [braintumor.org]
Respiratoric
- Sleep Apnea
Int J Pediatr Otorhinolaryngol. 1996; 37(1):53-64 [ PubMed ] Related Publications Two children complaining of sleep apnea presented with brain stem gliomas. [cancerindex.org]
Cardiovascular
- Hypertension
India Source of Support: None, Conflict of Interest: None PMID: 12215704 Keywords: Brain Edema, diagnosis,etiology,therapy,Brain Neoplasms, complications,diagnosis,Drainage, methods,Female, Human, Hydrocephalus, diagnosis,etiology,therapy,Intracranial Hypertension [jpgmonline.com]
Ears
- Tinnitus
Symptoms include loss of balance, tinnitus, total hearing loss, facial pain or numbness, and headache. Surgery is the standard treatment. Pseudotumor Cerebri This condition is not a brain tumor, but its symptoms mimic a brain tumor. [braintumor.org]
Tinnitus, a buzzing or ringing sound, may also be present; other symptoms are also possible. The tumor is benign, grows slowly and is treated with surgery or radiosurgery. It is also known as an acoustic neuroma. [aans.org]
28.8%), impaired coordination (28.8%), paresis (21.2%), pain (21.2%), gait ataxia (18.3%), dysarthria and dysphagia (13.5%), signs of raised intracranial pressure (12.5%), organic psycho-syndrome (7.7%), nausea and vomiting (6.7%), myoclonus (2.9%), tinnitus [bmccancer.biomedcentral.com]
Psychiatrical
- Anger
Attention and Concentration: Confusion, easy distraction, difficulty multitasking and planning Executive Functioning/General Intellectual Abilities: Decreased reasoning ability, impaired judgment, inability to connect cause and effect Personality Changes: Anger [epidermoidbraintumorsociety.org]
Neurologic
- Headache
A headache is regarded as one of the most common complaints, presumably as a consequence of growing intracranial pressure and hydrocephalus. [symptoma.com]
Headaches related to brainstem tumors may be worse shortly after waking up in the morning. [1] Diagnosis [ edit ] An MRI is better than a CT scan when a brainstem tumor is in the differential diagnosis. [en.wikipedia.org]
Headaches are often the most common symptom. But only very rarely do children with headaches have a tumor. [medlineplus.gov]
The headache may be most intense when people awaken from sleep, then lessen later in the day. If headaches with these characteristics start in people who have not had headaches before, a brain tumor may be the cause. [merckmanuals.com]
Based on its location in the brain, a cyst can cause symptoms such as headache, pain, seizures, or a neurological deficit. Cysts can be surgically removed or drained. [braintumor.org]
- Stroke
If you think it's a stroke, dial 999 immediately and ask for an ambulance. [nhs.uk]
The Neuro-ICU cares for patients with all types of neurosurgical and neurological injuries, including stroke, brain hemorrhage, trauma and tumors. [neurosurgery.ucla.edu]
Neural stem cells are being investigated as potential therapies for neurodegenerative diseases, stroke, and trauma [ 1 – 4, 6 – 9 ]. [journals.plos.org]
Although there have been no major studies of gender bias and brain tumors, a 2014 study in the journal Diagnosis found that women with strokes were 30 percent more likely to be misdiagnosed in emergency rooms than men. Dr. David E. [well.blogs.nytimes.com]
National Institute of Neurological Disorders and Stroke, part of National Institutes of Health, is conducting a trial of an experimental drug, IL13-PE38QQR, for the treatment of DIPG and other high grade gliomas using a technique termed convection enhanced [smhs.gwu.edu]
- Cranial Nerve Involvement
Diplopia (due to involvement of the medial longitudinal fasciculus). Nystagmus, oculomotor paresis, and eyelid retraction are also symptoms associated with these tumors (involvement of cranial nerves III and IV). [pedsoncologyeducation.com]
Intra-axial schwannoma is difficult to distinguish from the main differential diagnosis of glioma a priori, particularly in the absence of cranial nerve involvement. [ajnr.org]
Sixth and seventh cranial nerves are involved commonly. Facial sensory loss and a primary-position, upbeating nystagmus may be seen. [emedicine.medscape.com]
- Stupor
As herniation progresses there is a pattern of rostro-caudal deterioration of neural functions, which is discussed further in the chapter on stupor and coma ( Chapter 17 ). [dartmouth.edu]
- Trigeminal Neuralgia
Petroclival tumors can compress the trigeminal nerve, resulting in sharp pain in the face (trigeminal neuralgia) or spasms of the facial muscles. [brighamandwomens.org]
Workup
The diagnostic workup starts with a meticulous clinical evaluation that focuses on the onset of signs, their duration, as well as progression and severity. A detailed patient history may reveal sufficient information to localize the area from where symptoms develop and with a full neurological examination, a preliminary diagnosis can be made. To confirm a brain stem neoplasm, however, imaging studies need to be employed. MRI of the endocranium is the most valuable study for the assessment of brain tumors [1]. Crucial findings on MRI are diffuse enlargement, hyperintense signals on T2-weighted images and fluid attenuation inversion recovery (FLAIR) studies, and hypointense signals on T1-weighted images [1]. The use of gadolinium as a contrast is of limited efficacy due to the widely infiltrative nature of the tumors that develop in this part of the brain [1]. The pons seems to be the main location where brain stem neoplasms are found [1] [3] [5]. Fluorodeoxyglucose-positron emission tomography (FDG-PET) and MR spectroscopy have been described as possibly useful modalities for discriminating different tumor types solely on radiographic findings [4] [6]. When the lesion exhibits inconclusive findings, a biopsy may be performed as a definite diagnostic tool, although significant risks exist when carrying out this procedure [1] [3] [6].
Treatment
Treatment and prognosis [ edit ] Treatment typically consists of radiotherapy and steroids for palliation of symptoms. [ citation needed ] (Proton Beam Therapy should be included here by someone with good knowledge of it; it is more accurate, and very [en.wikipedia.org]
Treatment Options for Childhood Brain Stem Glioma For information about the treatments listed below, see the Treatment Option Overview section. [cancer.gov]
Surgery is the standard treatment. [braintumor.org]
Many people get a combination of treatments. [rxlist.com]
Gliomas in children Find out about gliomas in children and their treatment. Ependymoma Learn about ependymoma and its treatment. Oligodendroglioma Find out about oligodendroglioma and its treatment. [cancerresearchuk.org]
Prognosis
The prognosis is generally poor, with overall survival being about 12 and 32 months for the pediatric and adult gliomas, respectively. [symptoma.com]
Prognosis diffuse terrible prognosis 90-100% patients die within 2 years of diagnosis 6 focal (tectal glioma) excellent long-term survival with CSF shunting (essentially benign lesions) focal (other) good long-term prognosis with surgery (dorsally) exophytic [radiopaedia.org]
Treatment and prognosis [ edit ] Treatment typically consists of radiotherapy and steroids for palliation of symptoms. [ citation needed ] (Proton Beam Therapy should be included here by someone with good knowledge of it; it is more accurate, and very [en.wikipedia.org]
Focal brain stem glioma, however, can carry an exceptional prognosis, with long-term survivals frequently reported. [childhoodbraintumor.org]
Increasing tumor grade is associated with poorer prognosis. Molecular profiling is now an important part of glioma classification. An IDH1 mutation carries a significantly improved prognosis over the IDH1 wildtype, no matter the tumor grade. [emedicine.medscape.com]
Etiology
India Source of Support: None, Conflict of Interest: None PMID: 12215704 Keywords: Brain Edema, diagnosis,etiology,therapy,Brain Neoplasms, complications,diagnosis,Drainage, methods,Female, Human, Hydrocephalus, diagnosis,etiology,therapy,Intracranial [jpgmonline.com]
Clinical Clues to Nonmalignant Brain Lesions Clinical clues Suggested etiology Abnormalities found on more extensive imaging that are suggestive of an etiology Cysticercosis, fungal infections, sarcoidosis, tuberculosis Chronic fever; recent dental procedure [aafp.org]
How much of this variation is due to artifactual influences or etiologic differences has been the subject of many debates. Some reports have suggested a slight male preponderance, whereas others have failed to observe any sex predilection. [emedicine.medscape.com]
In some instances, however, the symptoms are specific to the etiology. Hemorrhage, which can be parenchymal or extra-axial is the most common cause of intracranial mass lesions and is discussed in Chapter 27. [dartmouth.edu]
Neuroradiological findings of the preoperative MRI were analyzed for etiological classification. Central neuroradiological review was not performed to depict daily clinical practice. [bmccancer.biomedcentral.com]
Epidemiology
Other factors that have been shown to negatively influence survival include contrast enhancement within the tumor on MRI, location within the brainstem (pons > medulla > midbrain), and advanced age (> 60 years). [2, 7] Epidemiology Race-, sex-, and age-related [emedicine.medscape.com]
Evaluation of epidemiologic evidence for primary adult brain tumor risk factors using evidence-based medicine. Prog Neurol Surg. 2006;19:54–79. 6. Ostrom QT, et al. [aafp.org]
Authors’ Affiliations (1) Department of Neurosurgery, Schwabing Academic Teaching Hospital, Munich, Germany (2) Service de Neurochirurgie, Hopital Louis Pasteur, Colmar, France (3) Institute for Medical Informatics, Statistics and Epidemiology, University [bmccancer.biomedcentral.com]
Pathophysiology
Anatomic location determines the pathophysiological manifestation of the tumor. With tectal lesions, hydrocephalus may occur as a result of fourth ventricular compression. [emedicine.medscape.com]
As befits a diagnosis that encompasses an array of pathophysiologic alterations, the radiographic appearance of adult brainstem glioma varies widely, with approximately 40% demonstrating enhancement ( 3, 5 ). [frontiersin.org]
Prevention
As the tumour increases in size, the supratentorial pressure build-up increases and prevents as much as possible the encroachment of the brainstem and hypothalamus. [jpgmonline.com]
[…] shrink the tumor or prevent the tumor from growing back Comfort measures, safety measures, physical therapy, occupational therapy, and other such steps may be required to improve quality of life. [medlineplus.gov]
Our state-of-the-art stem cell transplant facility is designed specifically for children and teens undergoing stem cell transplant — minimizing their exposure to harmful toxins and germs without preventing their freedom to leave their room or interact [dana-farber.org]
Nothing specific can be done to prevent the development of cysts. Treatment The type, size, and location of the cyst determine how it will be addressed. [abta.org]
[…] and prevent seizures associated with brain tumors) ventriculoperitoneal shunt (Also called a VP shunt.)- a VP shunt may be placed in the head to drain excess fluid from inside the brain to the abdomen. [urmc.rochester.edu]
References
- Reyes-Botero G, Mokhtari K, Martin-Duverneuil N, Delattre J-Y, Laigle-Donadey F. Adult Brainstem Gliomas. Oncologist. 2012;17(3):388-397.
- Hu J, Western S, Kesari S. Brainstem Glioma in Adults. Front Oncol. 2016;6:180..
- Theeler BJ, Ellezam B, Melguizo-Gavilanes I, et al. Adult brainstem gliomas: Correlation of clinical and molecular features. J Neurol Sci. 2015;353(1-2):92-97.
- Treglia G, Muoio B, Del Ciello A, Bertagna F. Usefulness of 18F-FDG-PET/CT in Evaluating a Brainstem Glioma in an Adult Patient with Neurofibromatosis Type 1. Nucl Med Mol Imaging. 2013;47(3):212-213.
- Guillamo JS, Monjour A, Taillandier L, et al. Brainstem gliomas in adults: prognostic factors and classification. Brain. 2001;124:2528–2539.
- Grimm SA, Chamberlain MC. Brainstem glioma: a review. Curr Neurol Neurosci Rep. 2013;13(5):346.