<unk>  are tumors that occur in the region of the brain referred to as the brain<unk> , which is the area between the aqueduct of Sylvius and the fourth ventricle. In the era preceding modern imaging, all<unk>  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<unk>  as a distinct subgroup, and reported that these lesions were surgically curable with aggressive resection.<unk>  may be classified into four subtypes: diffuse, focal, dorsal exophytic, and cervicomedullary.<unk>  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<unk> .<unk>  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<unk>  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.<unk>  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<unk>  may progress to develop increased intracranial pressure and cerebral herniation. Common complications of<unk>  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<unk> , and diffuse subtype has the worst prognosis with treatment. The hallmark of<unk>  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<unk>  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<unk> . 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<unk>  depends on the subtype and whether it is newly diagnosed or a recurrent tumor. Patients with diffuse<unk>  are treated with radiotherapy and chemotherapy, whereas patients with focal<unk>  are treated with surgical resection with or without radiation therapy and chemotherapy. Surgical intervention is not recommended for the management of diffuse<unk> . Surgery with or without radiotherapy and chemotherapy is the mainstay of treatment for focal, dorsal exophytic and cervicomedullary<unk> .
Brain Stem Gliomas
Adult brain tumors
Mixed gliomas
Glioblastoma multiforme
Medulloblastoma
High grade astrocytoma
Ameloblastoma
Astrocytoma risk factors