[Systemic hemodynamics in the early period following the removal of tumors from hypothalamo-hypophyseal and pontobulbar locations].
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Lesions that completely destroyed the paraventricular nucleus of the hypothalamus were placed in fetal sheep (n = 4) at 108-110 days of gestational age. These fetuses were then subjected to hypotension (50% of initial mean fetal arterial blood pressure), hypoxaemia (a decrease in fetal PaO2 greater than or equal to 5 torr) and bolus injection of corticotropin releasing factor (CRF-1.0 micrograms iv) in random order on successive days. The lesioned fetuses produced significantly less ACTH after hypotension (+10 min: 35.7 +/- 26.9 vs. 358.0 +/- 99.7 and +30 min: 28.2 +/- 12.2 vs. 238.0 +/- 73.0 pg.ml-1) (P less than 0.05), hypoxaemia (+40 min: 23.5 +/- 9.3 vs. 198.3 +/- 75.8 and +60 min: 32.3 +/- 18.8 vs. 295.3 +/- 99.9 pg.ml-1) (P less than 0.05) and intravenous administration of 1 microgram CRF (+15 min: 32.0 +/- 16.8 vs. 145.7 +/- 25.0 and +60 min: 33.0 +/- 23.3 vs. 161.3 +/- 43.1 pg.ml-1) (P less than 0.05). Our experiments suggest an important role for the fetal paraventricular nucleus in control of ACTH secretion. They also indicate that impairment of paraventricular nucleus function at this stage of fetal life may have a detrimental effect on the ability of the anterior pituitary to secrete ACTH in response to exogenous CRF.
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A 56-year-old man presented with recalcitrant uveitis with anterior chamber and vitreous cells. He developed small scattered lesions in the macular areas of both eyes, which disappeared in the right eye and evolved to multifocal, discrete, punched-out lesions resembling birdshot retinochoroidopathy in the left eye. Ocular reticulum cell sarcoma (RCS) was suspected but was not confirmed by diagnostic vitrectomy. The patient died 3 years later and was found to have RCS with central nervous system and ocular involvement. The occurrence of tumor cells under the retinal pigment was the apparent cause of the multifocal lesions that disappeared in the right eye and that led to discrete punched-out lesions with no scarring in the left eye.
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This study is a multicenter, retrospective report of 10 infants in whom acquired nystagmus was the initial sign of chiasmal/ parachiasmal glioma. Nine patients presented before the age of 10 months. The nystagmus, primarily described as pendular and asymmetric, was difficult to differentiate from and therefore most often diagnosed as spasmus nutans . On average in the ten patients, the intracranial glioma was not recognized for 8.6 months after the onset of nystagmus. In the five diagnosed as spasmus nutans , the mean delay in recognizing the tumor was 14.5 months. Three associated clinical findings were present or developed in these patients to distinguish this entity from spasmus nutans : optic atrophy in all ten patients, poor feeding due to diencephalic syndrome in 5 of 10, and increased intracranial pressure with hydrocephalus in 3 of 10. The acquired nystagmus in these infants was evidence of a life-threatening chiasmal/ parachiasmal glioma.
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Oncocytic transformation is an infrequent event within the central nervous system and is limited to neoplasms of the choroid plexus, meninges, and pituitary gland. Oncocytic modifications in choroid plexus tumors seem to occur predominantly in adult patients and in the fourth ventricle and do not seem to reflect any particular biological behavior. Meningiomas showing oncocytic differentiation have been recently described and this variant probably behaves more aggressively. Pituitary oncocytomas are regarded as a subtype of null cell adenomas. Oncocytic tumors have a significantly higher risk of progression with a higher recurrence rate after radiotherapy. Oncocytic changes in astrocytic neoplasms are rare. More frequently, astrocytomas can show granular changes that result in neoplasms composed of cells with granular cytoplasm and eccentric nuclei resembling foamy macrophages. Granular cell astrocytomas may mimic non-neoplastic lesions such as cerebral infarction and demyelinating disease. Cells with granular bodies are frequent in pilocytic astrocytoma, pleomorphic xanthoastrocytoma, and ganglion cell tumors. Their presence is considered a useful diagnostic finding to distinguish these low-grade lesions from malignant gliomas. A rare neoplasm is the granular cell tumor of the infundibulum that, when symptomatic, has to be differentiated from pituitary adenomas and other more common lesions of the sellar region.
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Hypothalamic/visual pathway astrocytomas in children are usually quiescent, but certain cases contain aggressive neoplasms that cause progressive neurological and visual deterioration. Radiotherapy is not recommended in young children because of its adverse effects later in life. This report describes the efficacy of a chemotherapeutic regimen. Four young children with hypothalamic/visual pathway astrocytoma, with a mean of 18 months of age at diagnosis, were treated with chemotherapy. Three patients had diencephalic syndrome at the disease's onset. Three patients with pilocytic astrocytoma were histologically verified, and another infant was clinically diagnosed. A combination chemotherapy using cisplatin and vincristine was administered in a total of 8 cycles in 3 children and 4 in one child. One patient who demonstrated renal insufficiency after 4 cycles of this regimen was treated with additional 4 cycles using carboplatin instead of cisplatin. The acute and subacute hematologic and otologic toxicities were mild, and a transient renal insufficiency in a child during chemotherapy improved. After chemotherapy, tumor regression was documented in 3 patients, and the disease was observed to be stable in one patient with an evidence of intratumoral necrosis on MRI. Three patients showed neurological and endocrinological improvements. These results suggest that this regimen is feasible in young children and may be useful as a first-line treatment for hypothalamic/visual pathway astrocytomas, which in turn may allow potentially deleterious irradiation on the maturing brain to be deferred until the disease progresses.
AIM: To analyse the long-term results of conservative management with radiotherapy in patients with optic pathway tumours. DESIGN: All 69 patients were symptomatic at diagnosis and most neoplasms involved the optic chiasm and hypothalamus. RESULTS: At 10 years, overall survival and progression free survival were 83% and 65.5%, respectively. After radiotherapy, vision improved in 18 patients and remained stable in 29 other patients. Cerebrovascular complications occurred in nine of 53 patients treated with radiotherapy after a median interval of two and a half years. These complications were five times more frequent in patients with neurofibromatosis type 1 (NF1). Severe intellectual disabilities were present in 18 children, most of whom underwent irradiation at a very young age (median age, 4 years). IMPLICATIONS: Radiotherapy is a valuable treatment in terms of tumour response, visual outcome, and progression free survival. However, in young children and in patients with NF1, major sequelae are encountered and new treatment strategies should be proposed for these patients.
The authors report gigantism in a 16-month-old boy with an extensive optic pathway glioma infiltrating into somatostatinergic pathways, as revealed by magnetic resonance imaging and immunocytochemical studies. Stereotactic biopsies of areas showing hyperintense signal abnormalities on T2-weighted images in and adjacent to the involved visual pathways provided rarely obtained histological correlation of such areas. The patient received chemotherapy, which resulted in reduction of size and signal intensity of the tumor and stabilization of vision and growth velocity.
A 64-year-old man presented with a change in personality and behaviour but with no neurological deficits. He exhibited impaired executive functions, apathy, unsteadiness of gait and falling; he lacked insight and exhibited purposeless behaviour, making loud grunting noises. A CT scan at 6 months revealed no abnormality, but at 9 months CT and MRI showed a bilateral thalamic tumour. The presenting symptoms had similarities to frontal lobe dysfunction progressing to dementia.
Three children with diencephalic tumours are described. In these cases, the main clinical features are extreme emaciation and nystagmus. Ultrasonography and computed tomography demonstrated the tumour. In one case, high levels of growth hormone were observed. In the three cases, the tumour was a glioma.