Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0017638 (glioma)
 30,880 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Tumors of the hypothalamus present with a variety of clinical syndromes. Such syndromes appear to be both age and histology dependent. The diencephalic syndrome, an entity classically seen in infancy, presents as failure to thrive. In essentially all cases the pathologic substrate is a tumor with a predominance of gliomas. The traditional approach to treatment of the diencephalic syndrome has been surgery both for decompression and pathologic diagnosis followed by irradiation. Because of the tumor location, surgery is often confined to biopsy rather than radical extirpation. Furthermore, in infancy focal brain irradiation is not without significant morbidity. Within this context, we would like to present a case discussion regarding a 27-month-old boy with a hypothalamic-midbrain protoplasmic glioma treated with primary chemotherapy after surgical biopsy and pathologic documentation. The schedule of drugs utilized was based on the hypothesis of biochemical modulation of nitrosourea chemotherapy. Successful response to therapy was documented by sequential computed tomography (CT) studies and serial neurologic examinations beginning at age 5 months and every 2 months thereafter. The response of a single patient with the diencephalic syndrome treated primarily with chemotherapy makes extrapolation premature. Rather, the authors suggest chemotherapy as an alternative to focal brain irradiation. Such therapy could be utilized either in the event of recurrent tumor or as late consolidation after primary treatment with chemotherapy. The latter approach would allow a patient to have radiation therapy deferred until a later age at which time morbidity attending brain irradiation may be minimized.
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PMID:Chemotherapeutic treatment of the diencephalic syndrome. A case report. 270 79

During the period from 1966 to 1996 the authors analyzed the clinicopathological characteristics of 46 cases of histologically verified primary brain tumors with symptomatic onset during the first 3 years of life. The patient group included 27 males and 19 females. There were 14 patients during the first year, 13 during the second year, and 19 during the third year. Supratentorial tumors (60.9%) were more common than infratentorial tumors. Histologically, neuroepithelial tumors predominated. The incidence of ependymal tumors, particularly malignant ones, and of neuronal/mixed neuronal-glial tumors was higher than in previous reports. Congenital brain tumors, those occurring within 2 months after birth, or tumors of dysplastic origin comprised 42.9% of the tumors that developed within 1 year of birth. At the onset, macrocephaly, failure to thrive, and seizures were prominent symptoms or signs in the younger patients. Focal neurological deficits and increased intracranial pressure predominated in the older patients. All but one patient underwent surgical treatment, and 17 patients received adjuvant therapy after surgery. The prognosis was mainly related to the histology of the malignancy. The outcome of medulloblastomas was poor. The quality of life of surviving patients was relatively good, 77.8% having better performance status (PS) than the Eastern Cooperative Oncology Group PS 2.
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PMID:Primary brain tumors in children under age 3 years. 987 57

Diencephalic syndrome (DS) is a complex of signs and symptoms related to hypothalamic dysfunction. Its main features are emaciation despite normal energy intake and an alert appearance. This syndrome has been described in association with space-occupying lesions of the hypothalamic-optic chiasm region, mainly low-grade glioma, and less often with tumors in the proximity of the IV ventricle. Two patients with DS are reported. The first patient was an 8-month old boy with classical features of DS. Extensive investigation of malabsorption revealed no abnormalities and the first neurologic sign was a rotatory nystagmus. Magnetic resonance imaging (MRI) showed evidence of a hypothalamic tumor, which was identified after surgical resection as a pilocytic astrocytoma. The second patient was an adolescent boy who presented a 20-kg weight loss over a 6-month period and psychological disturbances. Gastrointestinal disease was ruled out as a cause of malnutrition. Because of a polydipsia-polyuria syndrome, manifest while the patient was hospitalized with a suspected diagnosis of anorexia nervosa, MRI of the brain was performed. Multiple intracranial lesions were revealed, mainly in the hypothalamus, and were identified as a disseminated disgerminoma. No neurologic signs or symptoms were present. The patients died 2 years and 1 year after diagnosis, respectively. Although DS is rare, it should be considered in severe failure to thrive or emaciation despite adequate food intake and normal absorptive function of the small bowel.
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PMID:[Diencephalic syndrome: An uncommon cause of malnutrition]. 1204 23

Diencephalic syndrome (DES) is an extremely uncommon occurrence, and approximately 100 cases have been reported. It presents as a failure to thrive in infants and children but rarely occurs in adult population. The characteristic clinical features of DES include severely emaciated body, normal linear growth and normal or precocious intellectual development, hyperalertness, hyperkinesis, and euphoria usually associated with intracranial sellar-suprasellar mass lesion, usually optico-chiasmatic glioma or hypothalamic mass. DES as a presentation of craniopharyngioma is extremely uncommon but can also occur with brain stem mass. Detailed PubMed and MEDLINE search for craniopharyngioma associated with DES yielded only six cases in children below 6 years of age. Thus, we reviewed a total of seven cases including previously published six cases and added additional our own case. Overall, the mean age at diagnosis was 4.15 years with male:female ratio of 4:3, the mean time interval between symptom of DES appearance and final diagnosis was 6.6 months. The most commonly observed symptom of DES was weight loss (85%). The clinical feature, imaging, and management of such rare syndrome along with pertinent literature are briefly reviewed.
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PMID:Diencephalic Syndrome as Presentation of Giant Childhood Craniopharyngioma: Management Review. 3093 76