Cases reported "Brain Stem Neoplasms"

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1/6. 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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2/6. 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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3/6. Dysembryoplastic neuroepithelial tumor of the midbrain tectum: a case report.

    Dysembryoplastic neuroepithelial tumor (DNT) is a relatively new nosologic entity. First described in 1988, it is now included in the "neuronal and mixed neuronal-glial tumours" category in the revised 2000 world health organization (WHO) classification of Tumours of the nervous system. The collective experience of more than 300 reported cases indicates that, with only rare exceptions, DNTs are cerebral cortical lesions. At present, the actual incidence of extracortical DNT is unknown. We describe, the clinicopathologic features of the first tectal DNT. The patient was a 51-year-old man with a 2-month history of pulsatile headaches. On neurologic examination, the only abnormality was gait ataxia. magnetic resonance imaging (MRI) demonstrated a midbrain tumor involving the tectum. It was hypointense on T1-weighted images and featured an iso- to hyperintense nodule at its center. The nodule showed enhancement upon contrast administration. No aqueductal obstruction or intraventricular extension of tumor was detected. The tumor was approached supratentorially and removed completely. The mucoid tumor was well demarcated from neural tissue. Histopathologically, it was a typical DNT, exhibiting a nodular pattern of growth with a "specific glioneuronal component." This case report documents the first DNT to arise in the midbrain tectum and focuses on the problem of diagnosing this uncommon tumor at extracortical sites.
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4/6. Long-term survival and complete response of a patient with recurrent diffuse intrinsic brain stem glioblastoma multiforme.

    Recurrent diffuse intrinsic brain stem glioblastoma multiforme carries an extremely poor prognosis and a median survival of less than 7 months. In this article, the authors report good results in a 40-year-old man diagnosed with glioblastoma multiforme who received antineoplastons. The patient's brain tumor was diagnosed in May 1999, and he subsequently underwent subtotal tumor resection and standard radiation therapy. magnetic resonance imaging and positron emission tomography scans documented his tumor recurrence. Approximately 2 months after completion of radiation therapy, he was admitted for administration of intravenous antineoplastons A10 and AS2-1 through a subclavian venous catheter by intermittent bolus injections 6 times per day using a portable pump. Administration of antineoplastons A10 and AS2-1 was over 655 consecutive days with the exception of a few short interruptions. The maximum dosage of A10 was 8.15 g/kg/d and AS2-1 0.35 g/kg/d. Antineoplastons A10 and AS2-1 administration was very well tolerated with only mild reversible side effects. Follow-up magnetic resonance imaging and positron emission tomography scans revealed decrease and eventually disappearance of the tumor. A complete response was documented after approximately 1 year of antineoplastons A10 and AS2-1 administration. More than 4 years later, off antineoplastons A10 and AS2-1, the patient is tumor free, able to carry on normal activities, and works full-time, and his karnofsky performance status increased from 50 to 100. Extensive phase II trials with antineoplastons A10 and AS2-1 in patients with glioblastoma multiforme are nearing completion. These trials may provide more data regarding the efficacy of antineoplastons A10 and AS2-1 in the treatment of glioblastoma multiforme in untreated patients compared to the results in those patients with tumor recurrence after radiation therapy.
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5/6. Massive reduction of tumour load and normalisation of hyperprolactinaemia after high dose cabergoline in metastasised prolactinoma causing thoracic syringomyelia.

    In 1970 a 20 year old woman presented with a pituitary chromophobe adenoma for which she underwent transfrontal pituitary surgery. In 1978 she had to be reoperated on because of local tumour recurrence, resulting in hypopituitarism. bromocriptine (5 mg/day) was given for 15 years, but the plasma prolactin levels remained elevated. In 2000 the patient presented with signs and symptoms suggestive of a spinal cord lesion at the mid-thoracic level. A magnetic resonance imaging (MRI) scan showed an extensive leptomeningeal mass extending from the brainstem to L5, with a thoracic syringomyelia at the T7-T8 level. The plasma prolactin level was very high (5114 microg/l). A biopsy showed the presence of a metastasised prolactinoma.On administration of high dose cabergoline, 0.5 mg twice a day orally, the plasma prolactin levels decreased within one month and then normalised within 26 months. Tumour load reduced considerably but unfortunately, her signs and symptoms did not improve. This case illustrates that a high dose dopamine agonist might be an important therapeutic option in patients with a metastasised prolactinoma.
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6/6. Transitory, spontaneously recovering, peripheral facial nerve palsy after vinorelbine administration.

    Childhood intrinsic brain-stem gliomas have a dismal prognosis. Different treatment strategies have been adopted over the years without changing the final outcome of this ominous disease. Due to this grim prognosis, experimental therapeutic designs are worthwhile. Vinorelbine is a semi-synthetic vinca alkaloid that has demonstrated a broad spectrum of activity both in in vitro and in vivo experimental systems. By adopting vinorelbine during and after focal radiotherapy in the last two years, we have tried to evocate its known synergistic effect in brain-stem tumour control. Vinorelbine was administered intravenously before, during and after radiotherapy on tumour bed for a total duration of 10 months. All the consecutive patients whose clinical and radiological features corresponded to the diagnosis of an intrinsic brain-stem tumour, i.e., diffuse pontine glioma, have been accrued to this treatment protocol since July 2002. A histological assessment was not required. All patients were treated during hospital stay or in the outpatient clinic at the Istituto Nazionale Tumori of Milan (n=12) and at the Pediatric Clinic of Policlinico in Catania (n=1). Two of the thirteen patients so far treated have developed multiple subsequent, and transitory, episodes of monolateral peripheral facial nerve palsy during vinorelbine administration. The palsy always completely and spontaneously resolved at a short interval-around 30 min-after the end of the drug infusion. Obvious tumour progression was excluded by means of MRI; therefore the drug was administered as scheduled until the end of the treatment. We describe possible neurological and oncological implications of this unusual side effect, until now not reported in any other series dealing with vinorelbine as adjuvant treatment.
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