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Results for “Hypothalamic Neoplasms”

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PubMed1981

Radiological evaluation of sellar lesions.

The treatment and prognosis of sellar region masses differ according to the type of tumor, therefore, accurate assessment of the characteristics of the lesion as well as extent of the process is important. Evaluation of these tumors by various imaging modalities has revealed certain definable characteristics. Many of these processes, however, share similarities on any given imaging format. For this reason complete evaluation may require conventional skull radiography, complex motion tomography, radionuclide imaging, computed tomography, cerebral air or metrizamide studies, or cerebral angiography. Utilizing a combination of diagnostic modalities, definable characteristics of various types of tumors have been evolved which often may allow distinction of benign and malignant processes.

Adenoma↗
PubMed1995

Multiple cerebral cavernous angiomas. Case report.

This 26-year-old woman was admitted to Neurosurgery Department in 1972 for the first time due to increased intracranial pressure and since observed and treated by ventriculo-peritoneal shunt during next twenty years. First symptoms such as visual disturbances, hemiparesis and hemianesthesia appeared 8 years earlier followed 4 years later by epileptic fits. Ventriculography revealed hydrocephalus and the tumor in the region of the third ventricle. CT scan visualized multiple hyperdense foci. Ventriculo-peritoneal shunt provided good improvement. During the next twenty years condition of the patient gradually worsened. Diagnosis of multiple cavernous angiomas was established only at autopsy.

Adult↗
PubMed2003

Multiple deep-seated cavernomas in the third ventricle, hypothalamus and thalamus.

BACKGROUND: Cavernomas are rarely located in the third ventricle, hypothalamus, or thalamus. In this report, we present our experience managing a patient with three cavernomas, one each in the floor of the third ventricle, hypothalamus, and left thalamus. CASE PRESENTATION: This 62-year-old woman had had an unsteady gait and weakness of both legs for six months. Magnetic resonance imaging (MRI) revealed multiple intracranial tumours in the third ventricle, hypothalamus, and left thalamus. The third ventricle tumour was found to be a cavernoma by intra-operative endoscopic examination and then was excised via a transcortical, transventricular approach. Pathology revealed a cavernoma. The other two tumours were assumed to be cavernomas because of their MRI features. Three days after surgery, the patient developed right hemiparesis and disturbance of consciousness. Computed tomography revealed a left thalamic haemorrhage. After conservative treatment, her conscious level gradually recovered and she could walk with support seven months after surgery. INTERPRETATION: Our experience with this rare case of multiple, deep-seated cavernomas suggests that management of such patients requires specific consideration of the clinical manifestations, location, size, and previous bleeding history.

Brain Neoplasms↗
PubMed2005

Intracranial retrograde dissemination in filum terminale myxopapillary ependymomas.

Myxopapillary ependymomas (ME) are considered benign tumours (WHO grade I) of the central nervous system with long term survival rates and a tendency to local recurrence. However an aggressive course has occasionally been described, leading to CSF dissemination and even systemic metastases. We describe the case of a 23-year-old man diagnosed with intracranial subarachnoid dissemination of a filum terminale ME three years after the initial diagnosis. We have performed a careful review of the literature on CSF dissemination in ME and finally propose treatment of these cases.

Adult↗
PubMed1982

hCG beta producing pineal choriocarcinoma.

A patient is described with tumours in the region of the pineal gland and anterior hypothalamus associated with high levels of hCG beta in the serum and cerebrospinal fluid (CSF). He presented aged 19, with hypopituitarism, but persistent secondary sexual characteristics. LH immunoreactivity in serum was due to the hCG beta which probably caused the elevated level of testosterone. Following cranial irradiation the tumour became undetectable and hCG beta was eradicated from the serum and CSF. The patient later died because of an intramedullary metastasis of choriocarcinoma in the cervical spinal cord. The endocrine details of six previously reported intracranial neoplasms which have been shown to secrete hCG beta are reviewed. Only two of these involved the pineal region. Chorionic gonadotrophin production by this tumour enabled early detection of its recurrence. It is unclear how often precocious puberty is caused by tumours producing hCG beta, but patients presenting either with that problem or a pineal tumour should have circulating hCG beta measured.

Adult↗
PubMed1994

Rare case with metastatic involvement of hypothalamo-pituitary and pineal body presenting as hypopituitarism and diabetes insipidus.

An unusual case with metastatic spread of a small oat cell lung carcinoma to the hypothalamo-pituitary region and pineal body presenting as pituitary insufficiency is reported. Computed tomographic scan revealed an isodense mass in the hypothalamo-pituitary region and pineal body that was strongly enhanced by contrast medium. Magnetic resonance imaging demonstrated a downward displacement of the posterior lobe of the pituitary by the hypothalamo-pituitary mass. Dynamic assessment of hypothalamo-pituitary function showed hypopituitarism and diabetes insipidus; partial recovery was observed with the improvement in the carcinoma by chemotherapy. The brain was subsequently irradiated. The patient survived 10 months.

Aged↗
PubMed2003

Long-term outcome in children with gliomas of the anterior visual pathway.

We performed a retrospective assessment of the long-term visual, neurologic, and systemic outcomes of 47 patients with anterior visual pathway gliomas seen at the Johns Hopkins Hospital. All of the patients had follow-up of at least 10 years or died during the follow-up period. Two patients died before 10 years of follow-up were achieved. The remaining 45 patients (including three patients who subsequently died) had follow-up of 10-28 years (mean, 15.3 years; median, 15 years). Sixteen of the patients in this study, most of whom had neurofibromatosis type 1 (NF1), received no treatment. None of these patients died or developed neurologic morbidity as a result of their tumor. Thirty-one of the patients, most of whom did not have evidence of NF1, received treatment. Many of these patients subsequently developed neurologic, endocrine, or visual morbidity. However, although patients with anterior visual pathway gliomas who were not treated fared better visually, neurologically, and systemically than patients who were treated, patients who required treatment for progression generally had a good overall prognosis, particularly patients with tumors that did not involve the hypothalamus. Most of these patients survived and maintained useful vision in at least one eye. We believe that patients with anterior visual pathway gliomas, particularly those with NF1, should not be treated unless there is clear clinical or neuroimaging evidence of progression.

Adolescent↗
PubMed1995

Optic pathway and hypothalamic/chiasmatic gliomas in children younger than age 5 years with a 6-year follow-up.

BACKGROUND: Gliomas of the hypothalamus and optic pathways (H/OPG) comprise 5% of pediatric intracranial tumors, present most frequently in patients younger than age 5 years, and may have a more aggressive course in younger children. This study examined clinical characteristics and consequences of treatment of young children diagnosed with H/OPG: METHODS: The authors reviewed the course, treatment, and outcomes of 46 children diagnosed with H/OPG younger than age 5 years; the median follow-up was 72 months. The median age at diagnosis was 27 months. RESULTS: Fifteen (33%) of 46 patients had neurofibromatosis-1 (NF-1). Forty children (87%) had tumor progression in the follow-up period, and tumor growth was less common in children with NF-1. Initial therapy was limited to surgical resection in three and radiation in five children. To postpone radiation until after the age of 5 years, initial therapy was limited to chemotherapy in 32 patients. Radiation was not required in 9 of these patients and was postponed for 40 months (mean) in 17. Of the 46 children, 5 died of tumor progression, 4 became blind, and 20 of 34 evaluable patients had endocrine abnormalities. Endocrinopathy did not correlate with therapy. Ten of 17 children evaluated by questionnaire required special education. There was a trend for educational problems to occur in children who were irradiated before the age of 5 years. CONCLUSIONS: Gliomas of the hypothalamus and optic pathways and their treatment cause long term morbidity in young children. Chemotherapy postpones radiation effectively, and this delay may reduce neurologic morbidity; however, 60% of children eventually relapse. By contrast, patients with NF-1 have indolent disease.

Child Behavior Disorders↗
PubMed2002

Hepatocyte growth factor/scatter factor facilitates migration of GN-11 immortalized LHRH neurons.

The molecular cues regulating the migratory process of LHRH neurons from the olfactory placode into the brain are not well known, but gradients of chemotropic and chemorepellent factors secreted by the targets are likely to play a key role in guidance mechanisms. Hepatocyte growth factor/scatter factor (HGF/SF) is a pleiotropic cytokine inducing cell migration. It is involved in a variety of developmental processes through interaction with its receptor c-Met. Here we show that c-Met-antibody labels LHRH migrating neurons in the olfactory mesenchyme of E12 mouse and analyze the potential chemotropic effect of HGF/SF on two immortalized LHRH cell lines, GT1-7 and GN11, isolated from tumors developed in the hypothalamus and in the olfactory bulb, respectively. By RT-PCR analysis, Western blotting, and immunocytochemistry, we provide evidence for a high level of c-Met expression in GN11, but not in GT1-7, cells. In addition, HGF/SF treatment promotes specific migratory activity of GN11 cells, as demonstrated by collagen gel assay, time-lapse video microscopy, and Boyden's chamber experiments. Such promotion is inhibited by the neutralizing antibody. The data reported here represent the first direct evidence of a chemotactic effect of HGF/SF on immortalized LHRH neurons.

Animals↗
PubMed2006

[Childhood diencephalic pilocytic astrocytoma. A review of seven observations].

BACKGROUND AND PURPOSE: Pilocytic astrocytoma (PA) is a WHO grade I tumor of the central nervous system mostly arising in children and young adults. Management of diencephalic PA is a difficult challenge. Surgical treatment has to cope with both the difficulties of deep location and eloquent area tumors. MATERIALS AND METHODS: We retrospectively reviewed seven pediatric cases (female: 4, male: 3) of diencephalic PA. Opto-chiasmatic tumors were excluded from the series. Mean age at diagnosis was 108 months (9 years) (range: 4 month-18 years), median age was 111 months. Median follow-up for the series was 125 months. Tumor locations were as followed: right thalamus: 2, both thalami: 1, hypothalamus: 3, and right basal ganglia: 1. At the onset, the first symptom was mostly raised intracranial pressure. The delay in diagnosis ranged from 48 hours up to 6 years. TREATMENT: a shunting procedure was performed in 3 patients, a direct surgical approach in 5 patients (gross total removal: 2; partial removal: 3) and one patient had only a biopsy. Three children were re-operated. Three patients were treated by radiationtherapy (RT) after surgery. Chemotherapy was delivered for 4 children. RESULTS: The overall survival rate was 71.4 months (almost 6 years) (range: 3-184 months). Median survival rate was 42 months (3.5 years). Three children died, two by tumor progression and one death related to late side-effects of RT. Four patients have a good quality of life with GOS I (n = 3) or II (n = 1). We observed tumor regression in two patients at 1 and 17 years after the beginning of treatment. Correct diagnosis was only made for two cases at the initial pathological examination. CONCLUSION: The course of diencephalic PA is still unpredictable. The tumor can be controlled by a partial surgical removal, and a residual tumor can sometimes decrease in size after surgery. Gross total removal of these tumors, although difficult, may be performed. With cranial navigation systems, the risk is low. Pathological diagnosis is sometimes difficult to assess.

Adolescent↗
PubMed1998

Childhood onset of GH deficiency: reassessment of GH status and effects of substitution.

The final height of 77 patients with growth hormone (GH) insufficiency of childhood onset was analysed. Patients were treated between 1968 and 1996 for 1-15 years. The mean final height in patients with severe GH deficiency was 163.9 cm (males) or 151.1 cm (female patients). In patients with partial GH insufficiency, the mean final heights were 166.2 and 157.7 cm, respectively, and in patients with GH deficiency caused by intracerebral tumours, 175.9 or 160.2 cm, respectively. Late established diagnosis, lack of (pituitary) GH during the time prior to 1980 or low frequency of injections per week were related to the fact that final height was often below the target height range. In four of 51 retested patients with childhood onset GH deficiency, a normal response of the pituitary gland to pharmacological testing was found, whereas all other patients still suffered from GH insufficiency. An increased amount of fat and a decreased amount of lean body mass as well as low bone mineral density (12 out of 15 patients) could be demonstrated at re-evaluation.

Adolescent↗
PubMed1986

[Failure to thrive as a main symptom of intracranial tumors in early childhood].

In the course of only three years we studied three children, who presented for a long time with failure to thrive as the only symptom of an intracranial tumour. Up to 24 months elapsed from the first manifestation at the age of four, ten and eleven months to the final diagnosis. A suprasellar tumour was discovered once, an infratentorial one twice. In spite of lower caloric intake a more rapid weight gain was documented after neurosurgery. The intracranial tumour is an important part of differential diagnosis in infants and young children not thriving satisfactorily.

Astrocytoma↗
PubMed2001

Intracranial hemorrhage from an aneurysm encased in a pilocytic astrocytoma--case report and review of the literature.

The authors present an unusual complication of a recurrent chiasmal/hypothalamic pilocytic astrocytoma. From his second year of life onwards, the patient was repeatedly operated on and also underwent external radiation therapy (54 Gy total dose) 1 month after the first subtotal tumor resection. Nine years after irradiation, the patient was referred to our center with a sudden onset of severe headache, vomiting and neck stiffness. Computed tomography, magnetic resonance imaging, and cerebral angiography demonstrated an intratumoral, intraventricular, and subarachnoidal hemorrhage from an anterior communicating artery aneurysm encased in the pilocytic astrocytoma. The aneurysm was clipped and the patient recovered nicely from the hemorrhage. Three years later, the patient suddenly died of cardiac failure. Autopsy disclosed vessel wall changes compatible with radiation-induced vasculopathy. In light of this finding, the importance of radiation therapy and intracranial neoplasms for aneurysm formation is discussed.

Acute Disease↗