Cases reported "Pinealoma"

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1/13. Pineoblastoma showing unusual ventricular extension in a young adult--case report.

    A 19-year-old male presented with a 4-week history of headache. Neurological examination showed bilateral papilledema. Computed tomography revealed a pineal region mass with remarkable obstructive hydrocephalus. magnetic resonance imaging showed a pineal region tumor continuously invading through the tectum into the cerebral aqueduct and the fourth ventricle with the preservation of the adjacent structures. The tumor appeared an iso- to hypointense mass on T1-weighted images, a heterogeneous iso- to hyperintense mass on T2-weighted images, and a heterogeneously enhanced mass after administration of contrast medium. Histological examination after endoscopic biopsy confirmed that the tumor was a pineoblastoma. radiotherapy was given to the whole brain and the spinal cord, and magnetic resonance imaging showed complete remission of the tumor. Pineoblastomas are highly malignant tumors with seeding potential through the neighboring ventricle or along the meninges, and this type of tumor becomes larger with local extension. We found no previous reports of the continuous extension into the fourth ventricle. The present case showed ventricular extension with minimal mass effect to adjacent structures, and did not disturb ventricular configuration. According to the unusual finding of ventricular extension, this rare case of pineoblastoma requires adjuvant chemotherapy.
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2/13. Paroxysmal tonic upgaze: physiopathological considerations in three additional cases.

    Paroxysmal tonic upgaze of childhood has been described as a benign distinctive syndrome of abnormal ocular movement, with or without concomitant ataxia. After the first observation of four children, a further 29 patients have been reported with a wide spectrum of neurologic abnormalities such as ataxia, unsteady of gait, learning disabilities and mental retardation at follow-up. Electroencephalograms were normal in all the subjects and magnetic resonance imaging showed deficient myelination in only one patient. Recently it has been suggested that paroxysmal tonic upgaze could be a heterogeneous syndrome, ranging from a simply age-dependent manifestation to a clinical appearance of a variety of disorders affecting the corticomesencephalic loop of vertical eye movement. Moreover, it also could be an early sign of more widespread neurologic dysfunction. We describe three patients who presented paroxysmal tonic upgaze; in one, ataxia was present; in the second child, ataxia and language disorder also were observed; and in the third patient paroxysmal tonic upgaze was associated with loss of muscle tone (drop-attack-like events). On magnetic resonance imaging, a pinealoma compressing the dorsal mesencephalic region was detected. On the basis of our observations, we suggest that any insult with periaqueductal mesencephalic gray-matter involvement could be considered the basic condition for this peculiar clinical manifestation.
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3/13. Pineal region lesion masquerading choroid plexus papilloma: case report.

    BACKGROUND: choroid plexus papillomas (CPPs) are rare intracranial neoplasms, especially in the third ventricle. We report a patient with a posterior third ventricular CPP extending into the pineal that radiographically and clinically presented as a pineal region lesion. CASE DESCRIPTION: In a 51-year-old female with headache and upward gaze impairment radiological examination showed a mass in the pineal region obstructing the aqueduct of Sylvius and causing hydrocephalus. After ventriculoperitoneal shunting the tumor was approached through the infratentorial-supracerebellar approach and pathological examination revealed a typical CPP. CONCLUSIONS: This case represents an unusual presentation of an intracranial CPP. This entity should be considered an extremely rare cause of a lesion in the pineal region.
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4/13. ventriculostomy in a tumor involving the third ventricular floor.

    Recently, endoscopic management has gotten preference over open surgical treatment in selected cases of intraventricular tumors. Endoscopic third ventriculostomy (ETV) appears unfeasible when tumors extend to the third ventricular floor region due to the risk of perforators and injury to the basilar artery. We report the case of a 12-year-old male with symptoms of acute, chronic, raised intracranial pressure. Computed tomography (CT) and magnetic resonance imaging (MRI) showed a posterior third ventricular tumor involving the aqueductal and floor regions. The ETV was done after clearing the floor by partial tumor resection, keeping the dorsum sellae as the major anatomical landmark. The patient improved satisfactorily and was given adjuvant radiotherapy, and the need for an external shunt was completely eliminated. We conclude that ETV appears worth trying, even in third ventricular tumors involving the floor region if they can be cleared from the tumor keeping the dorsum sellae as the major anatomical landmark.
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5/13. Reversible hearing loss associated with a malignant pineal germ cell tumor. Case report.

    In patients with pineal tumors, clinical symptoms are due to direct compression of adjacent structures. The most common signs include increased intracranial pressure (80%) caused by obstruction of the sylvian aqueduct, and Parinaud syndrome (50%) caused by direct compression of the superior colliculi. hearing loss is rare in patients with tumors in this location. The authors report on the case of a 12-year-old boy in whom a malignant pineal germ cell tumor was found together with the unusual occurrence of severe hearing loss due to direct bilateral compression of the inferior colliculi. This condition resolved completely after tumor regression.
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6/13. Pineal epidermoid coinciding with pineocytoma.

    Tumours of the pineal region are uncommon. We report on a 62-year-old male presenting with Parinaud's syndrome and aqueduct stenosis caused by a cystic tumour in the pineal region. During surgery, adjacent to the cystic tumour, a second smaller tumour was identified, which was clearly separate from the first tumour and from the pineal gland. Histological examination disclosed the cystic tumour as an epidermoid cyst, whereas the second tumour demonstrated histological and immunohistochemical features of a pineocytoma. The unique finding of two different types of tumours in the pineal region is evaluated with regard to the histogenesis of epidermoid cysts and pineocytomas.
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7/13. One stage removal of periaqueductal glioma in adult via infratentorial supracerebellar and transaqueductal approaches.

    Most cases of periaqueductal tumours were found in children and adolescents, so treatment modalities in adults are not evaluated yet. A case of 40 years old woman with tectal and periaqueductal protoplasmatic astrocytoma grade II with history of headache and episodes of syncope is described. MRI showed triventricular hydrocephalus. After a shunt procedure she was doing well for about 15 months. Then she became somnolent, disoriented, and Parinaud syndrome appeared. The solid tumour was resected microsurgically in one stage. A part of it was removed via the supracerebellar infratentorial approach and tectal plate incision. The remainder of the tumour was removed through the fourth ventricle and the aqueduct which was filled by tumour mass. Postoperatively bilateral ptosis, vertical gaze palsy, slight horizontal gaze limitation and pupilloplegia were the main neurological sequelae. They all almost completely resolved within a year. The patient is doing well two and half years after the surgery. MRI showed patency of the aqueduct and no residual tumour. The authors suggest, that direct surgical attempt at total tumour removal should be considered in cases of periaqueductal and tectal gliomas. They also believe it is the first described case, in whom this type of tumour was totally removed by a combined transtectal and transaqueductal route.
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8/13. Aqueduct stenosis-?Benign.

    'Benign' aqueduct stenosis is a common cause of hydrocephalus in the paediatric population and is frequently treated by endoscopic third ventriculostomy. Occasionally, aqueduct stenosis can be a prelude to the development of other pathology, as is seen in these two cases of pineal tumours developing in patients whose hydrocephalus was successfully treated with endoscopic third ventriculostomy. The case histories are presented, along with the recommendation for increased radiological screening of patients with this usually 'benign' presentation.
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9/13. Periaqueductal tumor as a cause of late-onset aqueductal stenosis.

    Localized periaqueductal tumors usually present as hydrocephalus, which is then thought to be late-onset congenital aqueductal narrowing. In the past, radiological investigations, including positive contrast ventriculography, pneumoencephalography and even contrast CT scanning, have frequently failed to show tumors in this region in the early stages. However, recent experience using MRI on patients with unexplained late-onset aqueductal stenosis has in some instances shown the presence of a localized periaqueductal tumor as the cause of obstruction of the aqueduct. Four patients are described with hydrocephalus secondary to presumed late-onset congenital aqueductal stenosis, all of whom were shown to have a periaqueductal tumor. One patient had been investigated with a pneumoencephalogram and positive contrast ventriculogram and CT with an early-generation scanner, but the tumor was diagnosed only at the time of autopsy. In one patient, the tumor was diagnosed by CT and also confirmed with MRI; a histologic diagnosis has been obtained. In two other patients, CT with and without contrast enhancement was negative and in one of these, a positive contrast ventriculogram was also negative, but the tumor was easily identified on MRI scans. In both of these patients, a histologic diagnosis was obtained by stereotactic biopsy. Periaqueductal tumors must be considered in the differential diagnosis of patients who present with late onset aqueductal occlusion and in such patients, MRI would appear to be the investigation of choice.
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10/13. homovanillic acid and 5-hydroxyindoleacetic acid in the ventricular CSF of comatose patients with obstructive hydrocephalus.

    Ventricular cerebrospinal fluid (CSF) levels of homovanillic acid (HVA) and 5-hydroxyindoleacetic acid (5-HIAA) were determined every 2 to 4 hours over a period of 1 to 4 days in 12 patients, consisting of seven cases of brain tumor, two cases of cerebrovascular disease, and three cases of head injury. The concentrations of HVA and 5-HIAA varied with time in all cases, and significant correlations were found between the two values in eight cases. However, the relationship between variations of HVA and 5-HIAA levels and rhythms of sleep and waking could not be clarified. Both HVA and 5-HIAA concentrations varied at high levels in two patients whose CSF flow was completely blocked by tumor at the site of the fourth ventricle and aqueduct, respectively. On the contrary, in a case with craniopharyngioma in the third ventricle which blocked the bilateral foramina of Monro, although the HVA values were high, the 5-HIAA values varied at low levels. Of five comatose patients, two had cerebrovascular lesions and three had sustained head injury, and, in four of the five, the values of either one or both of HVA and 5-HIAA were low, but in the fifth case the 5-HIAA value was high. Estimation of HVA and 5-HIAA concentrations in ventricular CSF may be a valuable tool in the investigation of brain monoamine metabolism. However, many factors must be considered in the interpretation of results of clinical studies.
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