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PubMed2006

Craniopharyngiomas.

Craniopharyngiomas are rare, mainly sellar/parasellar, epithelial tumors diagnosed during childhood or adult life. Histologically, two primary subtypes have been recognized (adamantinomatous and papillary) with an as yet, unclarified pathogenesis. They may present with a variety of manifestations (neurological, visual, and hypothalamo-pituitary). Despite their benign histological appearance, they often show an unpredictable growth pattern, which, combined with the lack of randomized studies, poses significant difficulties in the establishment of an optimal therapeutic protocol. This should focus on the prevention of recurrence(s), improvement of survival, reduction of the significant disease and treatment-related morbidity (endocrine, visual, hypothalamic, neurobehavioral, and cognitive), and preservation of the quality of life. Currently, surgical excision followed by external beam irradiation, in cases of residual tumor, is the main treatment option. Intracystic irradiation or bleomycin, stereotactic radiosurgery, or radiotherapy and systemic chemotherapy are alternative approaches; their place in the management plan remains to be assessed in adequately powered long-term trials. Apart from the type of treatment, the identification of clinical and imaging parameters that will predict patients with a better prognosis is difficult. The central registration of patients with these challenging tumors may provide correlates between treatments and outcomes and establish prognostic factors at the pathological or molecular level that may further guide us in the future.

Antibiotics, Antineoplastic↗
PubMed1982

Physiological and psychological mechanisms of cancer anorexia.

Recent research addressing possible causes of the decline in spontaneous food intake which accompanies tumor growth and antitumor therapies is reviewed. Investigations of whether disruptions in brain mechanisms involved in feeding are responsible for cancer anorexia are briefly summarized. Studies evaluating the contributions of learned food aversions to the anorexia induced by tumors and antitumor therapy are discussed in some detail. Evidence is presented, from both the clinic and the laboratory, that learned food aversions occur as a result of the association of foods with the discomfort induced by chemotherapy and/or tumor growth. The possible role these aversions play in the etiology of cancer anorexia is discussed, and studies aimed at developing methods for preventing them are described.

Adaptation, Physiological↗
PubMedDate not supplied

Anaplastic evolution of childhood optico-hypothalamic pilocytic astrocytoma: report of an autopsy case.

A 19-month-old boy was found to have an optico-hypothalamic tumor with obstructive hydrocephalus, and received a ventriculoperitoneal (V-P) shunt followed by irradiation to the hypothalamic area. Although his visual disturbances continued, general conditions improved subsequently and he enjoyed normal life with only a visual disturbance. Four years later, however, he developed vomiting, left hemiparesis and dysuria, and died at the age of 6.5 years. Autopsy revealed a huge tumor occupying the hypothalamus, thalamus, basal ganglia and the right temporal lobe. Histologically, the tumor was composed of pilocytic astrocytes with Rosenthal fiber in the hypothalamus, where the tumor is considered to have developed initially, and poorly differentiated cells with some astrocytic and neuronal differentiation in other areas. Dissemination of the tumor was extensive in the whole CSF spaces, and in the peritoneum through the V-P shunt. We consider this a rare case of a childhood hypothalamic pilocytic astrocytoma undergoing delayed malignant evolution.

Astrocytoma↗
PubMed1997

Hemidystonia as presenting symptom of an optic glioma.

The first case is presented of an exophytic chiasmatic-hypothalamic glioma causing progressive hemidystonia in an 8-year-old boy, and all 24 reported cases of intraaxial cerebral tumours resulting in symptomatic hemidystonia are reviewed.

Astrocytoma↗
PubMed1989

Hypothalamic and basal forebrain germinoma presenting with amnesia and hyperphagia.

Patients harboring primary intracranial germinomas usually have had Parinaud's syndrome and hydrocephalus (with pineal tumors) or hypopituitarism, visual loss, and diabetes insipidus (with suprasellar tumors) at presentation. The few reported cases of verified intraparenchymal hypothalamic germinomas all have been in prepubertal males with progressive neurologic deficits, altered levels of consciousness, or clinically apparent panhypopituitarism. The subject of this report was an endocrinologically normal, primiparous 24-year-old woman who presented with hydrocephalus followed by worsening anterograde amnesia and hyperphagia despite having a functioning ventriculoatrial shunt. Computed tomography scanning and magnetic resonance imaging demonstrated an anterior hypothalamic, thalamic, and basal forebrain mass, which stereotactic biopsy proved to be a germinoma. Intraaxial germinomas restricted to the anterior hypothalamus or thalamus rarely have been reported. The clinical features of the current patient may be unique.

Adult↗
PubMedDate not supplied

Large hypothalamic and optic chiasm gliomas in infants: difficulties in distinction.

Hypothalamic and optic chiasm gliomas may be indistinguishable clinically, radiographically, and pathologically. Ten children with giant gliomas of the hypothalamus and optic pathway, all under age 2 years, had masses greater than 3 cm in diameter. Pathologically all proven cases (seven) were cytologically benign fibrillary astrocytomas. Previous authors have recognized the difficulty in distinguishing these lesions; in this series, using previously suggested criteria, masses of optic chiasm could not be differentiated from hypothalamic origins. Likewise, at surgery and autopsy, the origin of these large masses was indeterminate. These tumors were more aggressive, invasive, and less responsive to therapy than the relatively benign orbital and optic nerve gliomas of older children and adults.

Astrocytoma↗
PubMed2003

Recurrent spinal cord astrocytoma with intraventricular seeding.

PATIENT: We report on an unusual case of a recurrent and progressive spinal pilocytic astrocytoma with metastatic spreading to the hypothalamus in a 14-year-old boy. TREATMENT AND RESULTS: The patient underwent resection of an intramedullary atypical pilocytic astrocytoma classified as WHO grade II at the level of Th11/12 in 1997 and received local photon beam irradiation. Three years later, a second operation was necessary for a recurrent tumour at the same level. Seventeen months later, a second recurrent tumour with spinal seeding as well as an intracranial tumour in the third ventricle and hypothalamus was detected. He was shunted for an occlusive hydrocephalus and a stereotactic biopsy of the hypothalamic lesion was performed. The tumour was classified as anaplastic pilocytic astrocytoma (WHO grade III). He received chemotherapy with ifosfamide, cisplatin and etoposide (HIT-GBM-C-protocol), craniospinal radiation, and is still alive 60 months after the first operative intervention without neurological deficits.

Adolescent↗
PubMed1995

[A clinicopathological study of 16 cases with optico-hypothalamic glioma].

Although gliomas of the optic nerve pathways, optico-hypothalamic gliomas, have been considered to be benign neoplasms, some recurrent, malignant gliomas especially at the optic chiasm and hypothalamus have also been reported. It is crucial to know the factors that influence the prognosis of these tumor entities. In order to address this question, we analyzed 16 cases of optico-hypothalamic gliomas treated in our institute with emphasis especially upon the age, location of tumor, histological subtype and treatment modality. Eleven patients younger than 20 years and five older than 20 years were included in this study. In two patients the gliomas were accompanied by neurofibromatosis 1. The male to female ratio was 9:7. Anatomical locations of the tumors were categorized from T1 to T4, and visual symptoms were V0 to V4. Patients with tumors located within the optic nerve, chiasm (T1-3) mainly presented visual symptoms, but those with hypothalamus (T4) showed neuroendocrine signs but not visual ones. Twelve out of 16 cases represented isodense mass lesions on plain CT scans, which were homogenously enhanced by contrast media. Among 15 cases verified pathologically, 10 cases were pilocytic astrocytomas, 4 were astrocytomas and one was anaplastic astrocytoma. The survival rate was measured by Kaplan-Meier method and overall 5-year survival rate was 70.5%. Patients younger than 20 years could survive longer than those older than 20 years (87.5% and 40.0% respectively).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1993

[Characterization of space occupying masses in the diencephalic-hypothalamic region. MRI vs. TC].

All morphologic aspects of neoplastic and non neoplastic masses, arising from the diencephalic-hypothalamic region are discussed in this paper. We describe the densitometric characteristics on CT scan and the MRI appearance of masses affecting this region, both of benign lesions and malignant neoplastic masses. We describe separately masses arising from within the III ventricle and those of extraventricular origin and all semiotic findings of neuroradiological techniques of computed imaging.

Brain Diseases↗