1/10. Spontaneous remission of chiasmatic/hypothalamic masses in neurofibromatosis type 1: report of two cases.We report two children with neurofibromatosis type 1 showing enhancing masses on MRI suggesting neoplasms in the chiasm and hypothalamic region. In both patients no visual or endocrinal dysfunction was present. On serial MRI spontaneous partial remission was found, implying that a cautious approach to therapeutic management of similar cases should be taken.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/10. 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.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
3/10. Suprasellar monomorphous pilomyxoid neoplasm: an ultastructural analysis.The authors report 3 patients, 2 children and 1 adult, each of whom presented with an unusual myxoid lesion reminiscent of pilocytic astrocytoma but with other features of myxopapillary ependymoma. The magnetic resonance imaging findings in all cases showed a diffusely contrast-enhancing suprasellar mass focally extending into the third ventricle. Involvement of adjacent structures was more extensive in both infants. By light microscopy, all were composed of a monotonous population of cells with delicate piloid-like processes, loosely arranged within a prominent myxoid background. Focally, the neoplastic cells converged upon small blood vessels in pseudorosette-like formations. These histomorphologic features are identical to those of the recently described astrocytoma with monomorphous pilomyxoid features. In addition, the individual tumor cells showed strong cytoplasmic immunoreactivity with antibodies to glial fibrillary acidic protein (GFAP) and vimentin, as well as nuclear and cytoplasmic staining with S-100. All stained positive for synaptophysin and negative for chromogranin. By electron microscopy, the tumor cells were bipolar with elongated processes and apical surfaces displaying microvilli, cytoplasmic blebs and rare cilia. Vesicles and coated pits were seen, as were occasional synaptoid complexes. The current study serves to expand our clincopathologic experience with this rare and enigmatic lesion, with particular attention given to the ultrastructural characteristics.- - - - - - - - - - ranking = 4keywords = neoplasm (Clic here for more details about this article) |
4/10. Surgical management of exophytic chiasmatic-hypothalamic tumors of childhood.Sixteen children underwent 18 operations for radical resection of chiasmatic-hypothalamic tumors. The clinical presentation correlated with age: infants under 1 year of age presented with macrocephaly, failure to thrive, and severe visual failure; children aged 1 to 5 years predominantly had precocious puberty with mild visual deficits; and older children (greater than 5 years old) had slowly progressive loss of vision. All three infants had biologically aggressive tumors in spite of low-grade histology, and died from progressive tumor growth. Eleven of the 13 children aged 1 year or over are alive and well, without clinical or radiographic evidence of disease progression, 4 months to 4 1/2 years following surgery. Six of these patients, with a follow-up period of 10 months to 4 1/2 years (mean 27 months), have had no adjuvant therapy following radical surgical resection. The authors conclude that: 1) radical surgical resection of chiasmatic-hypothalamic tumors can be performed with minimal morbidity; 2) radical resection may delay the time to disease progression in older children and postpone the need for irradiation; 3) resection of postirradiation recurrent tumors may provide neurological improvement and long-lasting clinical remission; and 4) chiasmatic-hypothalamic tumors of infancy are aggressive neoplasms that require multimodality therapy.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
5/10. Combined occurrence of third ventricular germinoma and hypothalamic mixed glioma.A unique combination of occurrence of the 3rd ventricular germinoma and hypothalamic cystic mixed astrocytoma and oligodendroglioma is encountered in a 15-year-old man who presented clinically with stunted growth of the body, diabetes insipidus, and stupor. The germinoma was diagnosed by biopsy but the hypothalamic glioma was discovered at autopsy. Immunohistochemical localization of glial fibrillary acidic protein (GFAP) in the neoplastic cells confirmed the gliomatous nature of the hypothalamic lesion. The probability of multiple tumors should be kept in mind in dealing with intracranial neoplasms in order that the patient will be properly diagnosed and managed.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
6/10. 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.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
7/10. Hypothalamic neuronal hamartoma and adenohypophyseal neuronal choristoma: their association with growth hormone adenoma of the pituitary gland.Hypothalamic neuronal hamartomas and neuronal choristomas of the anterior pituitary are rare lesions; either may be associated with endocrinopathy. We describe a case of each with associated growth hormone-producing pituitary adenomas and clinical acromegaly, both well documented and studied by immunocytochemistry and electron microscopy. That a functional relationship exists between the neuronal malformation and the pituitary neoplasm remains speculative. We suggest that a growth hormone-releasing factor-like substance may have been elaborated by hypothalamic-type neurons, which, by a trophic effect, may have resulted in the production of an adenohypophyseal neoplasm. Our study supports the concept that secretory neurons, either outside or within the sella, may induce adenomas.- - - - - - - - - - ranking = 2keywords = neoplasm (Clic here for more details about this article) |
8/10. Laughing seizures due to a midline intraventricular neoplasm in tuberous sclerosis.Laughing seizures have been described in association with hypothalamic hamartomas and precocious puberty. Laughing seizures due to a neoplasm arising from the floor of the left lateral ventricle extending down towards the hypothalamus in a child with tuberous sclerosis is reported. This combination has not been reported before and emphasises the importance of imaging to diagnose such midline lesions in children with paroxysmal laughing.- - - - - - - - - - ranking = 5keywords = neoplasm (Clic here for more details about this article) |
9/10. Hypothalamic osteolipoma: a case report.The authors present a case of an ossified lipoma at tuber cinerum, an incidental finding when performing an autopsy of a 61-year old male. A tight connection to right communicans posterior artery led first to the diagnosis of a calcified saccular aneurysm, but histologic examination revealed an osteolipoma consisting of mature adipose tissue and formation of bone. Intracranial lipomas are neither hamartomas nor true neoplasms, but they are more likely to be congenital malformations. The recent literature is reviewed, and the two most current embryologic concepts of the development of intracranial lipomas are discussed.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
10/10. cisplatin/vincristine chemotherapy for hypothalamic/visual pathway astrocytomas in young children.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.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
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