Cases reported "Brain Stem Neoplasms"

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1/38. Histiocytic lesion mimicking intrinsic brainstem neoplasm. Case report.

    This 10-year-old girl presented with a 1-month history of progressive bulbar palsy and a solitary enhancing mass originating within the floor of the fourth ventricle. Results of initial imaging studies and presentation were suggestive of neoplasia. Subtotal resection was performed and pathological examination revealed the mass to be a histiocytic lesion, with no evidence of a glioma. The patient had no other stigmata of histiocytosis and was treated with steroid medications, resulting in prolonged resolution of the lesion. This case demonstrates that for discrete brainstem lesions the differential diagnosis includes entities other than glioma for which treatment is available. biopsy sampling should be considered when technically feasible.
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ranking = 1
keywords = glioma
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2/38. Neuroendoscopic biopsy of tectal glioma: a case report.

    OBJECTIVE AND IMPORTANCE: A type of intrinsic dorsal midbrain glioma known as benign tectal glioma can cause obstructive hydrocephalus. Because of its slow progress and relatively good prognosis, initial treatment should be cerebrospinal fluid diversion and biopsy. CLINICAL PRESENTATION: We report a 24-year-old man with obstructive hydrocephalus from a tectal glioma, who was admitted to the hospital for malfunction of a shunt placed 16 years previously. INTERVENTION: magnetic resonance imaging demonstrated enlargement of the dorsal midbrain associated with increased signal intensity on T2-weighted images and fluid attenuation inversion recovery (FLAIR) images. No enhancement occurred with contrast administration. We performed a neuroendoscopic third ventriculostomy and biopsy of the tumor. Histologic examination of the specimen obtained disclosed a low-grade astrocytoma. No postoperative neurologic complications occurred. CONCLUSION: FLAIR images were superior to T2-weighted images in demonstrating abnormal intensity in the dorsal midbrain. neuroendoscopy confirmed a yellowish tumor inferior to the posterior commissure occluding the entrance of the aqueduct, an observation useful in planning biopsy of the lesion. As the tumor apparently had existed for a number of years, the case supports the notion that the prognosis associated with these tumors is good.
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ranking = 3.5
keywords = glioma
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3/38. Familial brainstem glioma.

    Two young males with a high degree of consanguinity presented with similar brainstem gliomas. As the patients were raised separately, their presentation with similar tumors suggests a genetic basis for the occurrence of these gliomas.
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ranking = 3
keywords = glioma
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4/38. Respiratory control and respiratory sensation in a patient with a ganglioglioma within the dorsocaudal brain stem.

    We encountered a young woman with severe central sleep apnea caused by a medullary glioma located slightly dorsal to and to the right of the midline, a region not generally associated with CO(2) chemosensitivity. The patient had normal spirometric readings, lung volumes, diffusing capacity, maximal inspiratory pressure, and alveolar-arterial oxygen difference. While awake, she displayed marked irregularity in her breathing pattern; her end-tidal CO(2) (FET(CO(2))) ranged from 5.3 to 10.9%. During voluntary hyperpnea, she could quickly reduce her FET(CO(2)) to 4.2%, but her PCO(2) did not change after administration of acetazolamide or progesterone. Like patients with congenital central hypoventilation syndrome (CCHS), our patient had a relatively intact ventilatory response to exercise; her PCO(2) was high at the start of exercise and increased slightly thereafter. In contrast to CCHS patients, however, our patient had an intact hypoxic ventilatory response (DeltaVE/ DeltaSa(O(2)) = -0.37 L/min/Sa(O(2))). In further contrast to CCHS patients, our patient had a very short breathholding time and described a sensation of air hunger as the factor limiting her breathholding ability. Her heart rate and blood pressure responses to the valsalva maneuver were normal.
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ranking = 2.5
keywords = glioma
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5/38. moyamoya disease associated with a brain stem glioma.

    An 8-year-old boy was found to have primary moyamoya disease associated with a brain stem glioma. For over 3 years the child had experienced transient ischemic attacks induced by hyperventilation. One month before referral to our hospital he had presented with progressive left facial nerve palsy. magnetic resonance imaging showed a cystic mass in the lower pons. angiography revealed severe bilateral stenosis of the internal carotid arteries and prominent moyamoya vessels in the basal ganglia. Partial resection of the tumor yielded a histological diagnosis of pilocytic astrocytoma. Local radiation therapy reduced the size of the tumor. Anastomosis of the superficial temporal arteries and middle cerebral arteries on both sides was then performed. After direct bypass surgery, the patient remained in a good condition for a 5-year follow-up period. Clinical investigation of the coincidence of primary moyamoya disease and brain stem glioma led the authors to conclude that these two diseases coexisted independently.
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ranking = 3
keywords = glioma
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6/38. Alveolar hypoventilation syndrome in brainstem glioma with improvement after surgical resection.

    A 3-year-old boy presented with brainstem astrocytoma and central alveolar hypoventilation syndrome. Contrast MRI of the brain showed that the tumor involved the cerebellum, with compression of brainstem, and resolved after surgical resection. polysomnography performed before and after total tumor resection showed significant improvement in nocturnal respiratory rate, respiratory disturbance index, and oxygen desaturation. It is apparent that central alveolar hypoventilation syndrome secondary to brainstem tumor may improve after surgical resection for those with favorable anatomic location and histology. Serial polysomnography and MRI scans are useful for diagnosis and in the management plan, and to monitor progress.
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ranking = 2
keywords = glioma
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7/38. Diffuse brainstem glioma in a patient with Laurence-moon-(Bardet-)Biedl syndrome.

    An autopsy case of a patient with diffuse brainstem glioma associated with Laurence-moon-(Bardet-)Biedl syndrome is described. The subject was a 25-year-old woman who had been suffering from mental retardation, pigmented retinopathy, obesity, hexadactyly, amenorrhea and renal cysts. She developed dizziness, headache and consequent consciousness disturbance. Magnetic resonance images disclosed marked swelling of the pons without contrast enhancement. By means of combined chemotherapy and radiation, she survived for 15 months. Histopathological diagnosis for postmortem specimens obtained from the brainstem was glioblastoma multiforme. No pathogenetic association between the syndrome and brainstem gliomas is known, and the literature contains no cases of patients with this coincidence.
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ranking = 3
keywords = glioma
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8/38. Pontine glioma and cerebellopontine angle epidermoid tumour occurring as collision tumours.

    A 36-year-old male was admitted with a 10-year history of gradually progressive left ear hearing loss, diplopia and right hemiparesis. magnetic resonance imaging showed a left lateral pontine enhancing tumour and an additional cerebellopontine angle epidermoid tumour in close proximity forming a collision tumour. On exploration, the epidermoid tumour and the intraaxial pontine glioma were completely resected. The literature on collision tumours is reviewed.
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ranking = 2.5
keywords = glioma
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9/38. diagnosis and results of treatment with radiation therapy in gliomatosis cerebri patient: case report.

    Gliomatosis cerebri (GC) is a rare disease loosely defined as a diffusely infiltrating glioma involving extensive areas of the brain. The prognosis is poor and no definite treatment has proven effective for GC. Little information exists regarding the role of radiation therapy (RT) for GC, but some researchers have suggested that it is a good choice of treatment from their limited experience. In this report, we present a case with imaging and histological diagnosis of GC and demonstrate the treatment results of RT. The patient was a 39-year-old woman with progressive symptoms of dizziness, unsteady gait, headache, vomiting, and consciousness disturbance for 6 months. She received a series of radiographic examinations and surgical interventions for diagnosis. The definite diagnosis of GC was made by a combination of magnetic resonance imaging (MRI) findings and histological examinations. Forty Gray (Gy) of whole brain irradiation followed by 14 Gy reduced-field boosts were given to her. The MRI, following treatment, showed regressive changes, and clinical symptoms were slightly improved. The patient survived 19 months after the diagnosis, which is longer than the average survival time of patients without treatment.
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ranking = 2.5
keywords = glioma
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10/38. Paroxysmal alternating skew deviation and nystagmus after partial destruction of the uvula.

    A patient with suspected brain stem glioma involving the area of the left vestibular nuclei and cerebellar peduncle, developed paroxysmal alternating skew deviation and direction changing nystagmus after biopsy of the inferior cerebellar vermis resulting in destruction of the uvula. Between attacks she had right over left skew deviation with asymptomatic right beating horizontal nystagmus. Slow phases of the resting nystagmus showed increasing velocity, similar to congenital nystagmus. At intervals of 40-50 seconds, paroxysmal reversal of her skew deviation occurred, accompanied by violent left beating horizontal torsional nystagmus lasting 10-12 seconds and causing severe oscillopsia. It is proposed that this complex paroxysmal eye movement disorder results from (1) a lesion in the left vestibular nuclei causing right over left skew and right beating resting nystagmus and (2) a disruption of cerebellar inhibition of vestibular nuclei, causing alternating activity in the vestibular system with intermittent reversal of the skew deviation and paroxysmal nystagmus towards the side of the lesion.
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ranking = 0.5
keywords = glioma
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