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)

An optic chiasm glioma may cause loss of vision, endocrine disturbances, hydrocephalus and cerebral ischemia due to its proximity to the pituitary, hypothalamus, III ventricle and internal carotids. A 3-month-old infant with optic chiasm glioma developed hypopituitarism and inappropriate secretion of antidiuretic hormone with plasma hypo-osmolality. The cerebrospinal fluid (CSF) protein concentration was markedly elevated. The impairment of fluid absorption via arachnoid villi and peritoneum by the high protein content, and reversed osmotic gradient between protein-rich CSF and hypo-osmolar plasma may have contributed to both nonobstructive hydrocephalus and recurrent ascites following ventriculoperitoneal shunting. Cerebral ischemia from carotid compression may have led to cerebral atrophy.
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PMID:Optic chiasm glioma associated with inappropriate secretion of antidiuretic hormone, cerebral ischemia, nonobstructive hydrocephalus and chronic ascites following ventriculoperitoneal shunting. 179 May 31

Eleven patients with combined neurological and endocrine complications after external radiotherapy for nasopharyngeal carcinoma are described. Neurologically, memory disturbance, complex partial seizures and hypodense areas in one or both temporal lobes on CT were typical features. Endocrinologically, hypopituitarism was the prominent manifestation. This constellation of clinical features in a patient with previous radiotherapy to the nasopharynx characterises radiation injury to the inferomedial aspects of the temporal lobes and the hypothalamic-pituitary axis. While the parenchymal brain lesions may mimic metastases or glioma on CT, the associated endocrine disturbance would betray the correct diagnosis. The importance of recognising the hypopituitarism which may be clinically asymptomatic and which is amenable to therapy is emphasised, as is the need for a proper fractionation of the radiation dose to minimise the incidence of these disabling complications.
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PMID:Temporal lobe and hypothalamic-pituitary dysfunctions after radiotherapy for nasopharyngeal carcinoma: a distinct clinical syndrome. 322 87

We report the outcome of growth hormone (GH) therapy in 34 children (17 boys and 17 girls) with brain tumors in whom hypopituitarism developed. The types of tumors included the following: craniopharyngiomas (18); germinomas (four); astrocytomas (three); chromophobe adenomas (three); medulloblastomas (two); glioma (one); dermoid (one); retinoblastoma (one); and metastatic rhabdomyosarcoma from the pelvis (one). Ninety-four percent of the patients were GH deficient post-tumor therapy, which consisted of surgery with and without radiotherapy. Twenty-four of 34 patients received GH. Eight of 24 patients receiving GH had recurrence of tumor; 16 were tumor free eight to 72 months after initial therapy. Eleven patients had 12 recurrences. Patients with tumor recurrence had a considerably lower growth rate during the first year of GH therapy than those without recurrence (mean, 3.5 +/- 1.3 cm/yr v 6.2 +/- 2.5 cm/yr). Three of 11 patients with recurrence had not received GH therapy; however, one was receiving testosterone intramuscularly monthly at the time of a second recurrence. Thus, 24 of 34 patients with brain tumors and hypopituitarism received GH therapy. Eight (33%) of 24 had tumor recurrence, compared with three (30%) of ten who did not receive GH. The data suggest that GH therapy is probably not associated with increased rate of tumor recurrence.
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PMID:Growth hormone therapy and tumor recurrence. Findings in children with brain neoplasms and hypopituitarism. 397 24

Patients with suprasellar lesions develop profound hypothalamic obesity and listlessness with no effective treatment. We added triiodothyronine (T(3)) supplementation in 3 such patients and present their response. All had previous nutritional counseling without benefit. All were treated for diabetes insipidus (DI) and hypopituitarism; serum free thyroxine (T(4)) level was normal. A 24-year-old woman (pineal tumor and astrocytoma) had weight gain (4.7 kg/yr for 3 years), cold intolerance, fatigue, dry skin, and constipation; after T(3), she lost 14 kg over 27 months and reported overall improvement. Her bone mineral density also improved. A 10.6-year-old boy (optic glioma) was gaining 6 kg/yr for 4 years; after T(3) supplement, he lost 4.3 kg over 11 months. A 12-year-old girl (mixed germ cell tumor) had weight gain (8.3 kg/yr for 3 years) and listlessness; after T(3), she lost 8.1 kg over 16 months and had improved alertness. All patients were asymptomatic despite supraphysiologic T(3) levels. We suggest that T(3) may serve as a simple and effective supplement, which can promote weight loss and improve the well being of these patients with hypothalamic obesity.
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PMID:Triiodothyronine supplementation for hypothalamic obesity. 1240 83

We report 2 cases of primary central nervous system (CNS) lymphoma arising in the region of the optic nerve. For both patients, diagnosis of lymphoma was impossible without histological examination because of the rarity of the lymphoma location. The first case involved an 84-year-old woman who developed loss of vision and hypopituitarism. Intraoperative finding was optic glioma; histological diagnosis was diffuse large B cell lymphoma, however. The second case involved a 67-year-old man who developed loss of vision. The pre-surgical diagnosis was optic nerve neuritis; this was then revised to granuloma. The tumor arose in the optic nerve. Methotrexate and rituximab were administered and the patient remained in complete remission for 3 years. However, a sudden intratumoral hemorrhage occurred. Although most of the lymphoma cells obtained from the initial surgery were negative for vascular endothelial growth factor (VEGF) immunoreactivity, high levels of VEGF immunoreactivity in lymphoma cells was detected in the specimen obtained after intratumoral bleeding at recurrence, and correlation between VEGF reactivity and tumor recurrence was suggested. To date, primary CNS lymphomas with intracerebral hemorrhage have been reported in 3 cases only, and a correlation between intratumoral hemorrhage and the degree of VEGF expression has been suggested. VEGF also might have predictive significance for recurrence.
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PMID:Primary CNS lymphoma arising in the region of the optic nerve presenting as loss of vision: 2 case reports, including a patient with a massive intracerebral hemorrhage. 2399 67

Pediatric tectal plate gliomas are indolent slow-growing gliomas that often present with increased intracranial pressure or incidentally on routine brain imaging. We investigated clinical outcomes, endocrinopathies, and neuropsychological sequelae associated with tectal plate gliomas. Twenty-six patients with tectal plate glioma were identified in a 20-year retrospective review. Clinical outcomes, treatments, endocrine function, neuropsychological testing outcomes and radiographic imaging were reviewed for possible signs correlating with tumor progression. Among 26 patients, 19 presented with signs or symptoms of increased intracranial pressure (73 %) versus an incidental finding in 7 (27 %). Median follow-up was 46 months (range 8-143 months). Six of 26 (23 %) experienced progressive disease after diagnosis. Five of 26 (19 %) required more than one surgical procedure due to failure of initial endoscopic third ventriculostomy. Seven of 26 had history of endocrine dysfunction, of which, five presented with endocrine dysfunction (precocious puberty or short stature), 1 developed menstrual irregularities after surgical intervention and 1 had preexisting pan hypopituitarism. Of 12 patients with available neuropsychological testing, eleven had at least one indicator of executive functioning in the low-average to impaired range. While tectal plate gliomas have been considered indolent tumors that are rarely progressive, 23 % of patients in our cohort experienced disease progression and required further therapy. Neurocognitive deficits may occur, while endocrine deficiency is uncommon. Regular multidisciplinary oncology follow-up, routine monitoring with MRI and formal neurocognitive evaluation are imperative to provide early recognition of disease progression or recurrent hydrocephalus and to improve school functioning in this population.
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PMID:Pediatric tectal plate gliomas: a review of clinical outcomes, endocrinopathies, and neuropsychological sequelae. 2558 35